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Sample records for intrahepatic extramedullary hematopoiesis

  1. Transjugular Intrahepatic Porto-Systemic Stent-Shunt for Therapy of Bleeding Esophageal Varices Due to Extramedullary Hematopoiesis in Primary Myelofibrosis: A Case Report

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    Phillip, Veit;Berger, Hermann;Straub, Melanie;Saugel, Bernd;Treiber, Matthias;Einwächter, Henrik;Schmid, Roland M.;Huber, Wolfgang

    2016-01-01

    Background: Primary myelofibrosis belongs to the group of myeloproliferative syndromes. Extramedullary hematopoiesis in the liver can lead to portal hypertension. Patient and Methods: We report a case of a patient with life-threatening, endoscopically not treatable bleeding from esophageal varices due to extramedullary hematopoiesis of the liver that was successfully treated with placement of a transjugular intrahepatic porto-systemic stent-shunt (TIPS). Results: Therapy of variceal bleeding ...

  2. Transjugular intrahepatic porto-systemic stent-shunt for therapy of bleeding esophageal varices due to extramedullary hematopoiesis in primary myelofibrosis: a case report.

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    Phillip, Veit; Berger, Hermann; Straub, Melanie; Saugel, Bernd; Treiber, Matthias; Einwächter, Henrik; Schmid, Roland M; Huber, Wolfgang

    2012-01-01

    Primary myelofibrosis belongs to the group of myeloproliferative syndromes. Extramedullary hematopoiesis in the liver can lead to portal hypertension. We report a case of a patient with life-threatening, endoscopically not treatable bleeding from esophageal varices due to extramedullary hematopoiesis of the liver that was successfully treated with placement of a transjugular intrahepatic porto-systemic stent-shunt (TIPS). Therapy of variceal bleeding by TIPS insertion was successful. During a 29-month follow-up, no hepatic failure, hepatic encephalopathy, or further variceal bleeding episode occurred. TIPS placement is a well-established procedure for the treatment of complications due to portal hypertension mainly due to liver cirrhosis. This report illustrates that TIPS placement can also be a promising treatment option in patients with primary myelofibrosis and portal hypertension due to extramedullary hematopoiesis. Copyright © 2012 S. Karger AG, Basel.

  3. Focal splenic masses of the extramedullary hematopoiesis

    International Nuclear Information System (INIS)

    Incedayi, M.; Sivrioglu, A.

    2012-01-01

    Full text: Extramedullary hematopoiesis arises from pleuripotential stem cells distributed throughout the body. It is most common in patients with congenital hemolytic anemia, such as thalassemia, sickle cell anemia and hereditary spherocytosis as a response to ineffective red blood cell formation. Although microscopic foci of Extramedullary hematopoiesis are commonly seen in the spleen and liver parenchyma, focal mass-like lesion of extramedullary hematopoiesis in the liver and spleen are rare. We report a case of intrasplenic focal extramedullary hematopoiesis lesions and the imaging features of extramedullary hematopoiesis on computed tomography and magnetic resonance imaging. Extramedullary hematopoiesis should always be considered as a diagnosis in a patient with a known hematological disorder

  4. MRI features of epidural extramedullary hematopoiesis

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    Alorainy, Ibrahim A. E-mail: alorainy@ksu.edu.sa; Al-Asmi, Abdullah R.; Carpio, Raquel del

    2000-07-01

    A case of {beta}-thalassemia intermedia with spinal cord compression due to extramedullary hematopoiesis, which was successfully treated by blood transfusion, is presented. Emphasis was made on the MRI appearance of extramedullary hematopoiesis on different pulse sequences. The theories that aimed to explain the involvement of the epidural space by extramedullary hematopoiesis are discussed.

  5. MRI features of epidural extramedullary hematopoiesis

    International Nuclear Information System (INIS)

    Alorainy, Ibrahim A.; Al-Asmi, Abdullah R.; Carpio, Raquel del

    2000-01-01

    A case of β-thalassemia intermedia with spinal cord compression due to extramedullary hematopoiesis, which was successfully treated by blood transfusion, is presented. Emphasis was made on the MRI appearance of extramedullary hematopoiesis on different pulse sequences. The theories that aimed to explain the involvement of the epidural space by extramedullary hematopoiesis are discussed

  6. Mass-like extramedullary hematopoiesis: imaging features

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    Ginzel, Andrew W. [Synergy Radiology Associates, Houston, TX (United States); Kransdorf, Mark J.; Peterson, Jeffrey J.; Garner, Hillary W. [Mayo Clinic, Department of Radiology, Jacksonville, FL (United States); Murphey, Mark D. [American Institute for Radiologic Pathology, Silver Spring, MD (United States)

    2012-08-15

    To report the imaging appearances of mass-like extramedullary hematopoiesis (EMH), to identify those features that are sufficiently characteristic to allow a confident diagnosis, and to recognize the clinical conditions associated with EMH and the relative incidence of mass-like disease. We retrospectively identified 44 patients with EMH; 12 of which (27%) had focal mass-like lesions and formed the study group. The study group consisted of 6 male and 6 female subjects with a mean age of 58 years (range 13-80 years). All 12 patients underwent CT imaging and 3 of the 12 patients had undergone additional MR imaging. The imaging characteristics of the extramedullary hematopoiesis lesions in the study group were analyzed and recorded. The patient's clinical presentation, including any condition associated with extramedullary hematopoiesis, was also recorded. Ten of the 12 (83%) patients had one or more masses located along the axial skeleton. Of the 10 patients with axial masses, 9 (90%) had multiple masses and 7 (70%) demonstrated internal fat. Eight patients (80%) had paraspinal masses and 4 patients (40%) had presacral masses. Seven patients (70%) had splenomegaly. Eleven of the 12 patients had a clinical history available for review. A predisposing condition for extramedullary hematopoiesis was present in 10 patients and included various anemias (5 cases; 45%), myelofibrosis/myelodysplastic syndrome (4 cases; 36%), and marrow proliferative disorder (1 case; 9%). One patient had no known predisposing condition. Mass-like extramedullary hematopoiesis most commonly presents as multiple, fat-containing lesions localized to the axial skeleton. When these imaging features are identified, extramedullary hematopoiesis should be strongly considered, particularly when occurring in the setting of a predisposing medical condition. (orig.)

  7. Renal extramedullary hematopoiesis: interstitial and glomerular pathology.

    Science.gov (United States)

    Alexander, Mariam P; Nasr, Samih H; Kurtin, Paul J; Casey, Edward T; Hernandez, Loren P Herrera; Fidler, Mary E; Sethi, Sanjeev; Cornell, Lynn D

    2015-12-01

    Renal extramedullary hematopoiesis is rarely recognized in the antemortem setting. We identified 14 patients with renal extramedullary hematopoiesis on antemortem specimens from 1994 to 2015. The mean age was 68 years (range 47-87 years); males predominated (M:F=9:5). All presented with renal insufficiency, including five (36%) with acute kidney injury. The mean serum creatinine at biopsy was 2.9 mg/dl (range 1.2-7.3 mg/dl). All had proteinuria (mean 7.9 g/24 h; range 0.5-28; n=13), including 9 with ≥3 g/24 h. Renal extramedullary hematopoiesis appeared histologically as an interstitial infiltrate (n=12) and/or a perirenal infiltrate (n=3) or mass-like lesion (n=1). Five were misdiagnosed as interstitial nephritis. Concurrent glomerular disease was prevalent and included fibrillary-like glomerulonephritis (n=3), chronic thrombotic microangiopathy (n=5), focal segmental glomerulosclerosis (n=6), and diabetic glomerulosclerosis (n=2). All patients had an underlying hematologic malignancy: primary myelofibrosis in 9, myeloproliferative neoplasm not otherwise specified in 1, essential thrombocythemia in 1, polycythemia vera in 1, and plasma cell myeloma in 2. Clinical follow-up was available in 12 patients, mean of 29 months (range 4-120 months). In 10 patients for whom treatment history could be obtained, 9 were treated with chemotherapy, and 1 was treated with steroids. The mean creatinine at last follow-up was 2 mg/dl (range 1.2-3.9 mg/dl) (n=9). Ten patients died in the follow-up period from their underlying hematological disease and had persistent renal disease. The two remaining patients had persistent chronic kidney disease. Renal extramedullary hematopoiesis should be considered in the differential diagnosis of interstitial infiltrates, particularly in the presence of a glomerulopathy and a hematologic malignancy.

  8. Extramedullary hematopoiesis: A report of two cases.

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    Zhang, Huan-Zhu; Li, Ying; Liu, Xin; Chen, Bao-Rong; Yao, Guo-Hua; Peng, Yu-Na

    2016-12-01

    Extramedullary hematopoiesis (EMH) is defined as hematopoiesis occurring in organs outside of the bone marrow. The present report describes two cases of thalassemic patients with paraspinal medullary hematopoiesis and analyzes the clinical manifestations, imaging, pathology, diagnosis and treatment of EMH. In addition, a supplementary review of previously published cases is provided along with a review of the related literature. Computed tomography (CT) of the first case revealed multiple paraspinal masses, and the largest was 6.2×8.0 cm in diameter. Likewise, CT of the second patient revealed multiple paraspinal masses in the bottom of the left thoracic cavity, and the largest was measured 10.1×10.5 cm. The two cases underwent surgical biopsy and the findings were compatible with a diagnosis of EMH. In conclusion, EMH is a compatible and rare disease, and should be distinguished from other neoplasms. EMH must considered when masses with characteristic radiologic appearance are detected in patients with thalassemia intermedia.

  9. Surgical treatment of an unusual case of pelvic extramedullary hematopoiesis.

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    Khen-Dunlop, Naziha; Girot, Robert; Brunelle, Francis; Révillon, Yann; Nihoul-Fékété, Claire; Sarnacki, Sabine

    2006-07-01

    Extramedullary hematopoiesis affects about 15% of the patients treated for thalassemia intermedia. Usually seen in adulthood, the most common location is the paraspinal region. Diagnosis and treatment of extramedullary hematopoiesis located in the pelvis of a young 15-year-old girl is discussed. The young age of the patient and the uncommon site of the mass first lead to the diagnosis of an ovarian dermoid cyst. Because of the clinical history and the typical feature on computed tomography scan, extramedullary hematopoiesis was concluded. A specific treatment based on blood transfusion and hydroxyurea was first proposed but remained inefficient. Surgical excision was thus successfully performed. Whereas surgery is limited to spinal cord compression in paraspinal extramedullary hematopoiesis, this observation argues for surgical treatment in symptomatic intraabdominal extramedullary hematopoiesis when medical treatment fails.

  10. The role of T2*-weighted gradient echo in the diagnosis of tumefactive intrahepatic extramedullary hematopoiesis in myelodysplastic syndrome and diffuse hepatic iron overload: a case report and review of the literature.

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    Belay, Abel A; Bellizzi, Andrew M; Stolpen, Alan H

    2018-01-15

    Extramedullary hematopoiesis is the proliferation of hematopoietic cells outside bone marrow secondary to marrow hematopoiesis failure. Extramedullary hematopoiesis rarely presents as a mass-forming hepatic lesion; in this case, imaging-based differentiation from primary and metastatic hepatic neoplasms is difficult, often leading to biopsy for definitive diagnosis. We report a case of tumefactive hepatic extramedullary hematopoiesis in the setting of myelodysplastic syndrome with concurrent hepatic iron overload, and the role of T2*-weighted gradient-echo magnetic resonance imaging in differentiating extramedullary hematopoiesis from primary and metastatic hepatic lesions. To the best of our knowledge, T2*-weighted gradient-echo evaluation of extramedullary hematopoiesis in the setting of diffuse hepatic hemochromatosis has not been previously described. A 52-year-old white man with myelodysplastic syndrome and marrow fibrosis was found to have a 4 cm hepatic lesion on ultrasound during workup for bone marrow transplantation. Magnetic resonance imaging revealed diffuse hepatic iron overload and non-visualization of the lesion on T2* gradient-echo sequence suggesting the presence of iron deposition within the lesion similar to that in background hepatic parenchyma. Subsequent ultrasound-guided biopsy of the lesion revealed extramedullary hematopoiesis. Six months later, while still being evaluated for bone marrow transplant, our patient was found to have poor pulmonary function tests. Follow-up computed tomography angiogram showed a mass within his right main pulmonary artery. Bronchoscopic biopsy of this mass once again revealed extramedullary hematopoiesis. He received radiation therapy to his chest. However, 2 weeks later, he developed mediastinal hematoma and died shortly afterward, secondary to respiratory arrest. Mass-forming extramedullary hematopoiesis is rare; however, our report emphasizes that it needs to be considered in the initial differential

  11. Intracranial Extramedullary Hematopoiesis in Beta-Thalassemia

    International Nuclear Information System (INIS)

    Karki, Bivek; Xu, Yi Kai; Wu, Yuan Kui; Tamrakar, Karuna

    2012-01-01

    Extramedullary hematopoiesis (EMH) represents tumor-like proliferation of hemopoietic tissue which complicates chronic hemoglobinopathy. Intracranial EMH is an extremely rare occurrence. Magnetic resonance imaging (MRI) offers a precise diagnosis. It is essential to distinguish EMH from other extradural central nervous system tumors, because treatment and prognosis are totally different. Herein, we report the imaging findings of beta-thalassemia in a 13-year-old boy complaining of weakness of left side of the body and gait disturbance; CT and MRI revealed an extradural mass in the right temporoparietal region.

  12. Intracranial extramedullary hematopoiesis in beta-thalassemia.

    Science.gov (United States)

    Karki, Bivek; Xu, Yi-Kai; Tamrakar, Karuna; Wu, Yuan-Kui

    2012-01-01

    Extramedullary hematopoiesis (EMH) represents tumor-like proliferation of hemopoietic tissue which complicates chronic hemoglobinopathy. Intracranial EMH is an extremely rare occurrence. Magnetic resonance imaging (MRI) offers a precise diagnosis. It is essential to distinguish EMH from other extradural central nervous system tumors, because treatment and prognosis are totally different. Herein, we report the imaging findings of beta-thalassemia in a 13-year-old boy complaining of weakness of left side of the body and gait disturbance; CT and MRI revealed an extradural mass in the right temporoparietal region.

  13. Intracranial Extramedullary Hematopoiesis in Beta-Thalassemia

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    Karki, Bivek; Xu, Yi Kai; Wu, Yuan Kui [Nan fang Hospital, Southern Medical University, Guangzhou (China); Tamrakar, Karuna [Zhujiang Hospital, Southern Medical University, Guangzhou (China)

    2012-03-15

    Extramedullary hematopoiesis (EMH) represents tumor-like proliferation of hemopoietic tissue which complicates chronic hemoglobinopathy. Intracranial EMH is an extremely rare occurrence. Magnetic resonance imaging (MRI) offers a precise diagnosis. It is essential to distinguish EMH from other extradural central nervous system tumors, because treatment and prognosis are totally different. Herein, we report the imaging findings of beta-thalassemia in a 13-year-old boy complaining of weakness of left side of the body and gait disturbance; CT and MRI revealed an extradural mass in the right temporoparietal region.

  14. Extramedullary paraspinal hematopoiesis in thalassemia: CT and MRI evaluation

    International Nuclear Information System (INIS)

    Tsitouridis, J.; Stamos, S.; Hassapopoulou, E.; Tsitouridis, K.; Nikolopoulos, P.

    1999-01-01

    We present a comparative CT and MRI study of the paraspinal extramedullary hematopoiesis in 32 thalassemic patients. The patients were classified into four groups according to the MRI and CT imaging findings. Active recent extramedullary paraspinal hematopoietic masses show soft tissue behavior in both CT and MRI. Older inactive masses reveal iron deposition or fatty replacement. Combined imaging findings of paraspinal extramedullary hematopoiesis revealed the phase of its evolution and the correct diagnosis

  15. Extramedullary paraspinal hematopoiesis in thalassemia: CT and MRI evaluation

    Energy Technology Data Exchange (ETDEWEB)

    Tsitouridis, J.; Stamos, S.; Hassapopoulou, E.; Tsitouridis, K.; Nikolopoulos, P

    1999-04-01

    We present a comparative CT and MRI study of the paraspinal extramedullary hematopoiesis in 32 thalassemic patients. The patients were classified into four groups according to the MRI and CT imaging findings. Active recent extramedullary paraspinal hematopoietic masses show soft tissue behavior in both CT and MRI. Older inactive masses reveal iron deposition or fatty replacement. Combined imaging findings of paraspinal extramedullary hematopoiesis revealed the phase of its evolution and the correct diagnosis.

  16. Extramedullary plasmacytoma involving perirenal space accompanied by extramedullary hematopoiesis and amyloid deposition.

    Science.gov (United States)

    Mimura, Rie; Kamishima, Tamotsu; Kubota, Kanako C; Nakano, Fumihito; Yabe, Ichiro; Sasaki, Hidenao; Maruyama, Satoru; Shinohara, Nobuo; Harris, Ardene A; Haga, Hironori; Shirato, Hiroki; Terae, Satoshi

    2010-05-01

    A 62-year-old man was referred to us after unsuccessful treatment of bilateral weakness in his upper and lower extremities with paresthesia in both lower extremities. Computed tomography (CT) revealed soft tissue masses in the left kidney along the capsule and paraaortic region that were of relatively low attenuation with accompanying granular calcifications. Pathological diagnosis of the biopsy specimen was extramedullary plasmacytoma accompanied by extramedullary hematopoiesis and amyloid deposition. Although the CT findings correlated well with the pathological results, the case was extremely atypical for extramedullary plasmacytoma in respect to location and the accompaniment with extramedullary hematopoiesis.

  17. Extramedullary Hematopoiesis: An Unusual Finding in Subdural Hematomas

    Directory of Open Access Journals (Sweden)

    Rong Li

    2011-01-01

    Full Text Available We present a case of a 59-year-old man who was found to have clusters of hyperchromatic, small, round nucleated cells within a subdural hematoma removed after a skull fracture. Immunohistochemistry study confirmed that the cells were hematopoietic components predominantly composed of normoblasts. In this paper, we describe the clinical and pathological findings. A brief review of published information on extramedullary hematopoiesis in subdural hematoma and the mechanisms of pathogenesis are also discussed. While extramedullary hematopoiesis is seen anecdotally by neuropathologists in chronic subdural hematomas, only a few cases are documented in the literature. Furthermore, extramedullary hematopoiesis in subdural hematoma can pose a diagnostic challenge for general pathologists who encounter subdural hematoma evacuations seldom in their surgical pathology practices.

  18. [A case of cutaneous extramedullary hematopoiesis associated with idiopathic myelofibrosis].

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    Corella, F; Barnadas, M A; Bordes, R; Curell, R; Espinosa, I; Vergara, C; Alomar, A

    2008-05-01

    Cutaneous extramedullary hematopoiesis is a rare manifestation of chronic myeloproliferative processes, mainly chronic idiopathic myelofibrosis. In adults, it manifests as macules, papules, nodules, and ulcers on the trunk. The lesions usually appear soon after diagnosis and the possibility of a relationship between splenectomy and the appearance of extramedullary foci of hematopoiesis is still debated. Diagnosis is based on histopathology showing an infiltrate with different combinations of myeloid and erythroid cell precursors and megakaryocytes. Symptomatic treatment is provided alongside treatment of the underlying disease. We report a new case associated with chronic idiopathic myelofibrosis in which foci of cutaneous extramedullary hematopoiesis were observed 9 years after initial diagnosis. The lesions were progressive and the patient went on to develop acute myeloid leukemia.

  19. Thalassemia, extramedullary hematopoiesis, and spinal cord compression: A case report

    OpenAIRE

    Bukhari, Syed Sarmad; Junaid, Muhammad; Rashid, Mamoon Ur

    2016-01-01

    Background: Extramedullary hematopoiesis (EMH) refers to hematopoiesis outside of the medulla of the bone. Chronic anemia states such as thalassemia can cause hematopoietic tissue to expand in certain locations. We report a case of spinal cord compression due to recurrent spinal epidural EMH, which was treated with a combination of surgery and radiotherapy. Pakistan has one of the highest incidence and prevalence of thalassemia in the world. We describe published literature on diagnosis and m...

  20. Intracranial extramedullary hematopoiesis: a rare cause of headaches.

    Science.gov (United States)

    Singer, Adam; Quencer, Robert

    2014-01-01

    Ectopic bone marrow production, known as extramedullary hematopoiesis, may result in symptoms due to compression on normal structures. We present the multimodality imaging findings and subsequent management of a rare case of symptomatic extramedullary hematopoiesis within the calvarium. Case report. A 54-year-old male with a history of myelofibrosis and no previous diagnosis of a headache disorder presented to the emergency department with worsening severe bilateral headaches. A nonenhanced CT of the brain was performed and diffuse extra-axial nodular hyperdensities were visualized. MRI of the brain demonstrated diffuse extra-axial avidly enhancing nodular masses, dural thickening and marked susceptibility. No paravertebral masses, typical for extramedullary hematopoiesis, were present in the chest or abdomen. Although the clinical team considered a biopsy to confirm the diagnosis, we suggested a noninvasive confirmatory test. The subsequent Tc99m sulfur colloid scan corroborated the diagnosis. The patient was then referred to radiation oncology for treatment. In summary, extramedullary hematopoiesis is a hematologic compensatory disorder that rarely occurs within the CNS and may cause neurological compromise due to compression on underlying structures. The diagnosis can be made with noninvasive imaging and treated with low dose radiation therapy. Copyright © 2013 by the American Society of Neuroimaging.

  1. Premature epiphyseal fusion and extramedullary hematopoiesis in thalassemia

    International Nuclear Information System (INIS)

    Colavita, N.; Orazi, C.; Danza, S.M.; Falappa, P.G.; Fabbri, R.

    1987-01-01

    The main skeletal abnormalities in β-thalassemia are widening of medullary spaces, rarefaction of bone trabeculae, thinning of cortical bone, and perpendicular periosteal spiculation. Premature epiphyseal fusion (PEF) and extramedullary hematopoiesis (EH) are found, though more rarely. The incidence of PEF and EH in 64 patients affected by β-thalassemia is reported. The different incidence of such complications in thalassemia major and intermedia is reported, and a possible correlation with transfusion regimen is also considered. (orig.)

  2. Extramedullary hematopoiesis: a rare occurrence in the sinonasal tract.

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    Bizzoni, Andrea; Lombardi, Davide; Maroldi, Roberto; Incardona, Paolo; Nicolai, Piero

    2010-04-01

    Extramedullary hematopoiesis (EMH) is a systemic reaction to inadequate hematopoiesis. We report two exceedingly rare cases of EMH involving the paranasal sinuses. The first patient, a 30-year-old man, presented with a maxillary sinus mass. The lesion was excised by endoscopic surgery: definitive histology identified foci of EMH within an inflammatory fibromyxoid pseudotumor. The second case occurred in a 29-year-old man affected by intermediate beta-thalassemia. He was hospitalized with a diagnosis of sphenoid sinus mucocele secondary to an ethmoid lesion. The patient underwent endoscopic excision of the mass and drainage of the sphenoid mucocele. At definitive histology, a diagnosis of EMH was established. Herein, the presenting modalities, imaging profile, and treatment options of this rare EMH localization are reviewed. Copyright (c) 2009 Elsevier Ireland Ltd. All rights reserved.

  3. Adrenal extramedullary hematopoiesis associated with β-thalassemia major.

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    Keikhaei, Bijan; Shirazi, Ahmad Soltani; Pour, Mahboob Mohammad

    2012-05-10

    The presence of apparently normal hematopoietic tissue outside of bone marrow cavity is defined as extramedullary hematopoiesis (EMH). EMH is a rare complication in thalassemia major (TM) and adrenal gland as well. This report describes a case of adrenal EMH in a 26-year-old man with β-TM. He has been transfused with regular blood transfusion since 9 months. During the routine physical examination he was incidentally found to have a hypoechoic mass at his abdominal ultrasonography. Abdominal computed tomography scan revealed a right well-defined suprarenal mass 7.7×7.3×5.8 cm in size. The diagnosis of EMH was confirmed with ultrasonographic-guided fine needle biopsy. Treatment options which include intensified regular blood transfusion and hydroxyurea have been started.

  4. Spinal cord compression secondary to extramedullary hematopoiesis in a dog.

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    Williams, Lindsay M; Skeen, Todd M

    2013-03-15

    An 11-year-old spayed female Siberian Husky was evaluated because of a 2-week history of progressive paraparesis. Results of neurologic examination were consistent with a T3-L3 myelopathy. There were no abnormalities on CBC, and hypercalcemia was noted on serum biochemical analysis. Several hypoechoic splenic nodules were evident on abdominal ultrasonography, and results of fine-needle aspiration cytology were consistent with splenic extramedullary hematopoiesis (EMH). Two compressive, extradural masses in the dorsal epidural space of the thoracolumbar region of the spinal cord were seen on MRI images. A dorsal laminectomy was performed to remove the extradural spinal masses. Results of histologic examination of tissue samples were consistent with EMH. Following surgery, clinical signs of paraparesis resolved, and there was no recurrence of the masses 24 months after surgery. Extramedullary hematopoesis should be considered as a differential diagnosis in dogs in which results of diagnostic imaging indicate a epidural mass. In human patients, spinal EMH usually occurs secondary to an underlying hematologic disease, but it can also occur spontaneously. Treatment options reported for humans include surgical decompression, radiation therapy, chemotherapy, and blood transfusion. The dog of this report responded favorably to surgical decompression and was clinically normal 2 years after surgery.

  5. Thalassemia, extramedullary hematopoiesis, and spinal cord compression: A case report.

    Science.gov (United States)

    Bukhari, Syed Sarmad; Junaid, Muhammad; Rashid, Mamoon Ur

    2016-01-01

    Extramedullary hematopoiesis (EMH) refers to hematopoiesis outside of the medulla of the bone. Chronic anemia states such as thalassemia can cause hematopoietic tissue to expand in certain locations. We report a case of spinal cord compression due to recurrent spinal epidural EMH, which was treated with a combination of surgery and radiotherapy. Pakistan has one of the highest incidence and prevalence of thalassemia in the world. We describe published literature on diagnosis and management of such cases. An 18-year-old male presented with bilateral lower limb paresis. He was a known case of homozygous beta thalassemia major. He had undergone surgery for spinal cord compression due to EMH 4 months prior to presentation. Symptom resolution was followed by deterioration 5 days later. He was operated again at our hospital with complete resection of the mass. He underwent local radiotherapy to prevent recurrence. At 2 years follow-up, he showed complete resolution of symptoms. Follow-up imaging demonstrated no residual mass. The possibility of EMH should be considered in every patient with ineffective erythropoiesis as a cause of spinal cord compression. Treatment of such cases is usually done with blood transfusions, which can reduce the hematopoietic drive for EMH. Other options include surgery, hydroxyurea, radiotherapy, or a combination of these on a case to case basis.

  6. Pulmonary Extramedullary Hematopoiesis in a Patient with Chronic Asthma Resembling Lung Cancer: A Case Report

    Directory of Open Access Journals (Sweden)

    Massood Hosseinzadeh

    2012-01-01

    Full Text Available Background. Extramedullary hematopoiesis is most often seen in reticuloendothelial organs specially spleen, liver, or lymph nodes, and it is rarely seen in lung parenchyma. Almost all reported cases of pulmonary extramedullary hematopoiesis occurred following myeloproliferative disorders specially myelofibrosis. Other less common underlying causes are thalassemia syndromes and other hemoglobinopathies. There was not any reported case of pulmonary extramedullary hematopoiesis in asthmatic patients in the medical literature. Case. Here we reported a 65-year-old lady who was a known case of bronchial asthma with recent developed right lower lobe lung mass. Chest X-ray and CT studies showed an infiltrating mass resembling malignancy. Fine needle aspiration cytology of mass revealed pulmonary extramedullary hematopoiesis. The patient followed for 10 months with serial physical examination and laboratory evaluations which were unremarkable. Conclusion. Extramedullary hematopoiesis of lung parenchyma can be mistaken for lung cancer radiologically. Although previous reported cases occurred with myelofibrosis or hemoglobinopathies, we are reporting the first case of asthma-associated extramedullary hematopoiesis.

  7. Renal Bleeding Due to Extramedullary Hematopoiesis in a Patient With Chronic Myelogenous Leukemia

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    Stephanie Zettner

    2014-11-01

    Full Text Available Chronic myelogenous leukemia (CML is a myeloproliferative disorder that normally presents in middle-aged adults. Renal infiltration and extramedullary hematopoiesis in renal tissue has been rarely reported. This case report presents a patient with CML and renal insufficiency who developed gross hematuria. Efforts at controlling the hematuria led to a cascade of events propelled by the underlying disorder that ultimately led to a radical nephrectomy, multiorgan failure, and prolonged hospitalization. We suggest that management of gross hematuria in clinically stable patients with CML, suspected of having extramedullary hematopoiesis, should prioritize treatment of the myeloproliferative disorder over efforts to control bleeding.

  8. Renal Bleeding Due to Extramedullary Hematopoiesis in a Patient With Chronic Myelogenous Leukemia.

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    Zettner, Stephanie; Mistry, Sandeep G

    2014-11-01

    Chronic myelogenous leukemia (CML) is a myeloproliferative disorder that normally presents in middle-aged adults. Renal infiltration and extramedullary hematopoiesis in renal tissue has been rarely reported. This case report presents a patient with CML and renal insufficiency who developed gross hematuria. Efforts at controlling the hematuria led to a cascade of events propelled by the underlying disorder that ultimately led to a radical nephrectomy, multiorgan failure, and prolonged hospitalization. We suggest that management of gross hematuria in clinically stable patients with CML, suspected of having extramedullary hematopoiesis, should prioritize treatment of the myeloproliferative disorder over efforts to control bleeding.

  9. Peritoneal carcinomatosis-like implants of extramedullary hematopoiesis. An insolite occurrence during splenectomy for myelofibrosis

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    Marco Casaccia

    Full Text Available Introduction: Primary myelofibrosis (MF is a myeloproliferative neoplasm that results in debilitating constitutional symptoms, splenomegaly, and cytopenias. In patients with symptomatic splenomegaly, splenectomy remains a viable treatment option for MF patients with medically refractory symptomatic splenomegaly that precludes the use of ruxolitinib. Case presentation: We present the clinical case of a patient who was admitted to our Department to perform a splenectomy in MF as a therapeutic step prior to an allogeneic stem cell transplantation (ASCT. A laparotomic splenectomy and excision of whitish wide-spread peritoneal and omental nodulations was performed. There were no operative complications and the surgery was completed with minimal blood loss. The histopathological exam revealed an extramedullary hematopoiesis in both spleen and peritoneal nodules. Conclusion: In primary myelofibrosis it must always be kept in mind the possible presence of peritoneal implants of extramedullary hematopoiesis and ascites of reactive genesis. We report a rare case of peritoneal carcinomatosis-like implants of extramedullary hematopoiesis found at splenectomy for MF. Keywords: Myelofibrosis, Splenomegaly, Splenectomy, Extramedullary hematopoiesis

  10. Adrenal extramedullary hematopoiesis associated with β-thalassemia major

    OpenAIRE

    Bijan Keikhaei; Ahmad Soltani Shirazi; Mahboob Mohammad Pour

    2012-01-01

    The presence of apparently normal hematopoietic tissue outside of bone marrow cavity is defined as extramedullary hema - topoiesis (EMH). EMH is a rare complication in thalassemia major (TM) and adrenal gland as well. This report describes a case of adrenal EMH in a 26-year-old man with β-TM. He has been transfused with regular blood transfusion since 9 months. During the routine physical examination he was incidentally found to have a hypoechoic mass at his abdominal ultrasonography. Abdomin...

  11. Myelopathy due to Spinal Extramedullary Hematopoiesis in a Patient with Polycythemia Vera.

    Science.gov (United States)

    Ito, Shuhei; Fujita, Nobuyuki; Hosogane, Naobumi; Nagoshi, Narihito; Yagi, Mitsuru; Iwanami, Akio; Watanabe, Kota; Tsuji, Takashi; Nakamura, Masaya; Matsumoto, Morio; Ishii, Ken

    2017-01-01

    Extramedullary hematopoiesis (EMH) occasionally occurs in patients exhibiting hematological disorders with decreased hematopoietic efficacy. EMH is rarely observed in the spinal epidural space and patients are usually asymptomatic. In particular, in the patients with polycythemia vera, spinal cord compression due to EMH is extremely rare. We report a case of polycythemia vera, in which operative therapy proved to be an effective treatment for myelopathy caused by spinal EMH.

  12. Cord Compression due to Extramedullary Hematopoiesis in an Adolescent with Known Beta Thalassemia Major

    OpenAIRE

    Soman, Salil; Rosenfeld, David L; Roychowdhury, Sudipta; Drachtman, Richard A; Cohler, Alan

    2009-01-01

    We describe a 16 year-old male with ß thalassemia major and gait disturbances that had not been given blood transfusions due to a severe childhood transfusion reaction. Thoracic spine MRI demonstrated hematopoietic marrow throughout the spine and epidural masses causing cord compression consistent with extramedullary hematopoiesis (EMH). After treatment with steroids, radiotherapy and monitored blood transfusions, the patient demonstrated significant improvement of his paraspinal lesions a...

  13. Myelopathy due to Spinal Extramedullary Hematopoiesis in a Patient with Polycythemia Vera

    OpenAIRE

    Ito, Shuhei; Fujita, Nobuyuki; Hosogane, Naobumi; Nagoshi, Narihito; Yagi, Mitsuru; Iwanami, Akio; Watanabe, Kota; Tsuji, Takashi; Nakamura, Masaya; Matsumoto, Morio; Ishii, Ken

    2017-01-01

    Extramedullary hematopoiesis (EMH) occasionally occurs in patients exhibiting hematological disorders with decreased hematopoietic efficacy. EMH is rarely observed in the spinal epidural space and patients are usually asymptomatic. In particular, in the patients with polycythemia vera, spinal cord compression due to EMH is extremely rare. We report a case of polycythemia vera, in which operative therapy proved to be an effective treatment for myelopathy caused by spinal EMH.

  14. Renal Bleeding Due to Extramedullary Hematopoiesis in a Patient With Chronic Myelogenous Leukemia

    OpenAIRE

    Stephanie Zettner; Sandeep G. Mistry

    2014-01-01

    Chronic myelogenous leukemia (CML) is a myeloproliferative disorder that normally presents in middle-aged adults. Renal infiltration and extramedullary hematopoiesis in renal tissue has been rarely reported. This case report presents a patient with CML and renal insufficiency who developed gross hematuria. Efforts at controlling the hematuria led to a cascade of events propelled by the underlying disorder that ultimately led to a radical nephrectomy, multiorgan failure, and prolonged hospital...

  15. Treatment with hydroxyurea in thalassemia intermedia with paravertebral pseudotumors of extramedullary hematopoiesis.

    Science.gov (United States)

    Cario, H; Wegener, M; Debatin, K-M; Kohne, E

    2002-08-01

    Excessive ineffective erythropoiesis in thalassemia intermedia may cause paravertebral pseudotumors of extramedullary hematopoiesis. Due to the proximity to the spinal canal, these paravertebral masses carry the risk of severe neurological damage. Treatment strategies include hypertransfusion, radiotherapy, and laminectomy. Hydroxyurea, stimulating fetal hemoglobin synthesis, may represent an alternative therapeutic approach. We report on a 26-year-old patient suffering from thalassemia intermedia with progressive anemia symptoms and presenting multiple intrathoracic paravertebral pseudotumors of extramedullary hematopoiesis. Hypertransfusion therapy and splenectomy were followed by regular transfusion (baseline hemoglobin 10 g/dl) and chelation with desferrioxamine. With this treatment, clinical symptoms disappeared, paravertebral hematopoietic masses did not progress, but severe hemosiderosis developed within a few years. Hydroxyurea therapy was initiated to increase the efficacy of erythropoiesis, thereby reducing the required transfusion volume but suppressing concomitantly further expansion of extramedullary hematopoiesis, and finally leading to a reduction of transfusional iron load. Treatment was started with 4 mg/kg per day and stepwise increased to 12.5 mg/kg per day. The fetal hemoglobin concentration increased from 4.5 to 5.5 g/dl after 1 year and to 9.9 g/dl after 2 years of treatment. The yearly transfusion volume was halved during the 1st year of treatment. At present, after 26 months of treatment, the patient has been transfusion-independent for 10 months. Serum ferritin levels decreased from 2844 to 1335 ng/ml. Size and shape of paravertebral hematopoietic pseudotumors remained stable. No side effects of hydroxyurea have been observed. In thalassemia intermedia patients with extramedullary hematopoiesis, hydroxyurea may lead to independence from regular transfusion therapy without further expansion of ectopic hematopoietic tissue.

  16. A Rare Presentation of Extramedullary Hematopoiesis in Post-polycythemic Myelofibrosis

    OpenAIRE

    Konca Degertekin, Ceyla; Özkurt, Zübeyde Nur; Akyürek, Nalan; Yağcı, Münci

    2012-01-01

    Polycythemia vera is a clonal proliferative disorder of the bone marrow that could possibly evolve into myelofibrosis in its natural course. Progression to myelofibrosis is usually a late stage complication and presents clinically with refractory cytopenias and extramedullary hematopoiesis (EMH). EMH can occur in any tissue during the course of post-polycythemic myelofibrosis. However, skin and cardiac involvements seems to be very rare. We present a 56-year-old woman with post-polycythemic m...

  17. Myelopathy due to Spinal Extramedullary Hematopoiesis in a Patient with Polycythemia Vera

    Directory of Open Access Journals (Sweden)

    Shuhei Ito

    2017-01-01

    Full Text Available Extramedullary hematopoiesis (EMH occasionally occurs in patients exhibiting hematological disorders with decreased hematopoietic efficacy. EMH is rarely observed in the spinal epidural space and patients are usually asymptomatic. In particular, in the patients with polycythemia vera, spinal cord compression due to EMH is extremely rare. We report a case of polycythemia vera, in which operative therapy proved to be an effective treatment for myelopathy caused by spinal EMH.

  18. Peritoneal carcinomatosis-like implants of extramedullary hematopoiesis. An insolite occurrence during splenectomy for myelofibrosis.

    Science.gov (United States)

    Casaccia, Marco; Fornaro, Rosario; Frascio, Marco; Palombo, Denise; Stabilini, Cesare; Firpo, Emma; Gianetta, Ezio

    2017-01-01

    Primary myelofibrosis (MF) is a myeloproliferative neoplasm that results in debilitating constitutional symptoms, splenomegaly, and cytopenias. In patients with symptomatic splenomegaly, splenectomy remains a viable treatment option for MF patients with medically refractory symptomatic splenomegaly that precludes the use of ruxolitinib. We present the clinical case of a patient who was admitted to our Department to perform a splenectomy in MF as a therapeutic step prior to an allogeneic stem cell transplantation (ASCT). A laparotomic splenectomy and excision of whitish wide-spread peritoneal and omental nodulations was performed. There were no operative complications and the surgery was completed with minimal blood loss. The histopathological exam revealed an extramedullary hematopoiesis in both spleen and peritoneal nodules. In primary myelofibrosis it must always be kept in mind the possible presence of peritoneal implants of extramedullary hematopoiesis and ascites of reactive genesis. We report a rare case of peritoneal carcinomatosis-like implants of extramedullary hematopoiesis found at splenectomy for MF. Copyright © 2017 The Authors. Published by Elsevier Ltd.. All rights reserved.

  19. Adrenal extramedullary hematopoiesis associated with β-thalassemia major

    Directory of Open Access Journals (Sweden)

    Bijan Keikhaei

    2012-01-01

    Full Text Available The presence of apparently normal hematopoietic tissue outside of bone marrow cavity is defined as extramedullary hema - topoiesis (EMH. EMH is a rare complication in thalassemia major (TM and adrenal gland as well. This report describes a case of adrenal EMH in a 26-year-old man with β-TM. He has been transfused with regular blood transfusion since 9 months. During the routine physical examination he was incidentally found to have a hypoechoic mass at his abdominal ultrasonography. Abdominal computed tomography scan revealed a right well-defined suprarenal mass 7.7¥7.3¥5.8 cm in size. The diagnosis of EMH was confirmed with ultrasonographic-guided fine needle biopsy. Treatment options which include intensified regular blood transfusion and hydroxyurea have been started.

  20. Paraspinal extramedullary hematopoiesis in patients with thalassemia intermedia.

    Science.gov (United States)

    Haidar, Rachid; Mhaidli, Hani; Taher, Ali T

    2010-06-01

    Ineffective erythropoiesis in patients with thalassemia intermedia drives extramedullary hematopoietic tumor formation in several parts of the body. Paraspinal involvement has received increasing attention due to the associated morbidity secondary to spinal cord compression. Although the history and physical examination may help narrow the differential diagnosis, radiographic imaging remains essential to confirm the existence of hematopoietic tissue. Characteristic appearance has been observed mainly on magnetic resonance imaging. Several treatment options have been described, including transfusion therapy, laminectomy, radiotherapy, and the use of fetal hemoglobin inducing agents that decrease the hematopoietic drive. However, the ideal management scheme remains controversial. Until large prospective trials evaluate the efficacy and safety of the available treatment options, both in single and in combination therapy, an individualized approach should be entertained.

  1. Incomplete paraplegia caused by extramedullary hematopoiesis in a patient with thalassemia intermedia.

    Science.gov (United States)

    Hisamud-Din, Nurhasyimah; Mustafah, Nadia Mohd; Fauzi, Aishah Ahmad; Hashim, Natiara Mohamad

    2017-01-01

    Extramedullary hematopoiesis (EMH) is the production of blood cell precursors outside the bone marrow that occur in various hematological diseases. In patients with thalassemia intermedia, ineffective erythropoiesis drives compensatory EMH in the liver, pancreas, pleura, spleen, ribs and spine. We describe a patient with thalassemia intermedia who presented with acute neurological symptoms caused by paraspinal EMH, which responded well to combination therapy of steroid, hypertransfusion, laminectomy and excision of pseudotumor and hydroxyurea therapy to boost the formation of fetal haemoglobin. Prompt recognition of EMH based on clinical presentation and typical radiological findings should be made. Early treatment is recommended to prevent irreversible damage to the spinal cord.

  2. Extramedullary hematopoiesis within the clivus: an unusual cause of lower cranial nerve palsy.

    Science.gov (United States)

    Reames, Davis L; Lindstrom, Katherine; Raghavan, Prashant; Jane, John

    2010-12-01

    We report one year follow up of a case of extramedullary hematopoiesis within the clivus. The imaging findings, brief clinical course, and endoscopic transphenoidal approach are described. A 29-year-old female with thalassemia developed worsening cranial nerve signs. After imaging studies discovered a large clival mass, she underwent endoscopic transphenoidal biopsy of the lesion. Neural compression from exuberant erythrogenesis within tissue normally quiescent of red blood cell production was found to be the etiology of her neural deficit. Treatment for this condition is generally non-operative unless significant neural compression is present. Radiotherapy and anti-neoplastic agents have been used with success.

  3. Extramedullary hematopoiesis of the conjunctiva presenting as active systemic disease in a patient with myelofibrosis.

    Science.gov (United States)

    Cuttler, Nirupa; Heidemann, David; Cendrowski, Christopher; Armin, Ali-Reza; Folberg, Robert

    2014-12-01

    To report the clinicopathological correlation of extramedullary hematopoiesis (EMH) of the conjunctiva in a patient with a history of myelofibrosis. Case report. Elevated pink conjunctival lesions developed bilaterally in a 73-year-old man who had been treated for myelofibrosis for 13 years. EMH was detected in the examination of tissue from the lesion of the inferior fornix of the right eye. The appearance of conjunctival lesions in a patient with myelofibrosis may indicate underlying pathology of EMH that may necessitate a change in systemic treatment of this condition.

  4. Intracranial involvement in extramedullary hematopoiesis: case report and review of the literature

    Energy Technology Data Exchange (ETDEWEB)

    Haidar, Salwa; Ortiz-Neira, Clara; Shroff, Manohar; Gilday, David; Blaser, Susan [Hospital for Sick Children, Department of Diagnostic Imaging, Toronto, ON (Canada)

    2005-06-01

    Intracranial involvement in extramedullary hematopoiesis (EMH) is rare, but it should be suspected in patients with myelofibrosis presenting with chronic severe headache. We present a 9-year-old girl with known myelofibrosis whose headaches were unresponsive to routine treatment. CT and MRI studies of the brain showed diffuse pachymeningeal thickening. CT examinations of the chest and abdomen had demonstrated bilateral thoracic paraspinal masses caused by EMH, suggesting the possibility that the intracranial involvement might also be related to EMH. The diagnosis was confirmed by sulfur colloid isotope scan. (orig.)

  5. Splenomegaly unresponsive to standard and salvage chemotherapy in Langerhans cell histiocytosis: a case of extramedullary hematopoiesis.

    Science.gov (United States)

    Christiansen, Ellen C; Ellwein, Marcine; Neglia, Joseph P

    2012-06-01

    Langerhans cell histiocytosis (LCH) is a proliferative disorder of dendritic cells which has evaded efforts to clearly define pathogenesis, diagnostic criteria, and therapeutic response markers. Strides have been made at classification with the recent development of a quantified score of disease severity. Splenic involvement is an indicator of poor prognosis, with spleen size its surrogate marker in evaluation and scoring. We describe a case of pediatric LCH with progressive splenomegaly despite treatment, which when examined at splenectomy revealed no LCH involvement but extramedullary hematopoiesis. These findings thus challenge our understanding of splenomegaly as a marker of disease. Copyright © 2011 Wiley Periodicals, Inc.

  6. Intracranial involvement in extramedullary hematopoiesis: case report and review of the literature

    International Nuclear Information System (INIS)

    Haidar, Salwa; Ortiz-Neira, Clara; Shroff, Manohar; Gilday, David; Blaser, Susan

    2005-01-01

    Intracranial involvement in extramedullary hematopoiesis (EMH) is rare, but it should be suspected in patients with myelofibrosis presenting with chronic severe headache. We present a 9-year-old girl with known myelofibrosis whose headaches were unresponsive to routine treatment. CT and MRI studies of the brain showed diffuse pachymeningeal thickening. CT examinations of the chest and abdomen had demonstrated bilateral thoracic paraspinal masses caused by EMH, suggesting the possibility that the intracranial involvement might also be related to EMH. The diagnosis was confirmed by sulfur colloid isotope scan. (orig.)

  7. Paraplegia due to extramedullary hematopoiesis in thalassemia treated successfully with radiation therapy.

    Science.gov (United States)

    Malik, Monica; Pillai, Lakshmi S; Gogia, Nidhi; Puri, Tarun; Mahapatra, M; Sharma, Daya Nand; Kumar, Rajat

    2007-03-01

    Spinal cord compression due to extramedullary hematopoiesis (EMH) is a rare complication of thalassemia and generally presents as paraparesis with sensory impairment. Complete paraplegia is extremely rare in EMH due to thalassemia although it is known to occur in polycythemia vera and sickle cell anemia. Treatment options mostly include surgery and/or radiotherapy. Whereas cases presenting with paraparesis have been treated with either surgery or radiotherapy with equal frequency and efficacy, almost all reported cases with paraplegia have been treated with surgery with or without radiation therapy. We hereby report a case of thalassemia intermedia with paraplegia treated successfully with radiotherapy.

  8. Extramedullary Hematopoiesis in a Man With β-Thalassemia: An Uncommon Cause of an Adrenal Mass

    Directory of Open Access Journals (Sweden)

    Zeighami

    2015-06-01

    Full Text Available Introduction Extramedullary hematopoiesis (EMH commonly occurs in the spleen, liver and lymph nodes. Rare cases of EMH in the adrenal gland have been reported. Case Presentation We report the case of a 33-year-old man from the South of Iran suffering from major β-thalassemia, who underwent open left adrenalectomy and the histopathology revealed EMH. Conclusions In patients in which a history of hematologic disorders exists, careful imaging and hormonal assay should be done to certify a diagnosis of EMH. However, the surgical management becomes inevitable in certain cases.

  9. Temporal bone extramedullary hematopoiesis as a causeof pediatric bilateral conductive hearing loss:Case report and review of the literature.

    Science.gov (United States)

    Lanigan, Alexander; Fordham, M Taylor

    2017-06-01

    Extramedullary hematopoiesis occurs in children with hemoglobinopathy and chronic anemia. The liver and spleen are often affected first, but other foci can develop to support erythrocyte demand. We report a case of a nine-year-old with beta thalassemia and temporal bone extramedullary hematopoiesis causing ossicular fixation and bilateral conductive hearing loss. There is only one case in the literature describing this phenomenon in pediatric patients, and this is the first case report of bilateral hearing loss from this physiologic phenomenon. Otolaryngologists should consider this etiology in patients with chronic anemia and conductive hearing loss in the absence of otitis media. Published by Elsevier B.V.

  10. Extramedullary hematopoiesis (EMH) in laboratory animals: offering an insight into stem cell research.

    Science.gov (United States)

    Chiu, Shao-Chih; Liu, Hua-Hsing; Chen, Chia-Ling; Chen, Pin-Ru; Liu, Ming-Chao; Lin, Shinn-Zong; Chang, Ko-Tung

    2015-01-01

    Extramedullary hematopoiesis (EMH) is a pathological process secondary to underlying bone marrow (BM) insufficiency in adults. It is characterized by the emergence of multipotent hematopoietic progenitors scattered around the affected tissue, most likely in the spleen, liver, and lymph node, etc. EMH in patients frequently receives less medical attention and is neglected unless a compressive or obstructive hematopoietic mass appears to endanger the patient's life. However, on a biological basis, EMH reflects the alteration of relationships among hematopoietic stem and progenitor cells (HSPCs) and their original and new microenvironments. The ability of hematopoietic stem cells (HSCs) to mobilize from the bone marrow and to accommodate and function in extramedullary tissues is rather complicated and far from our current understanding. Fortunately, many reports from the studies of drugs and genetics using animals have incidentally found EMH to be involved. Thereby, the molecular basis of EMH could further be elucidated from those animals after cross-comparison. A deeper understanding of the extramedullary hematopoietic niche could help expand stem cells in vitro and establish a better treatment in patients for stem cell transplantation.

  11. Serial CT Findings of Resolving Extramedullary Hematopoiesis as Unilateral Posterior Mediastinal Mass after Splenectomy in Hereditary Spherocytosis: A Case Report

    International Nuclear Information System (INIS)

    Nam, Mi Yeon; Lee, Ju Won; Kim, Yeo Ju; Kim, Youn Jeong; Kang, Young Hye; Lee, Kyung Hee

    2012-01-01

    Intrathoracic extramedullary hematopoiesis (EMH) is a rare condition of the hereditary spherocytosis. EMH usually regresses or disappears after treatment; such as splenectomy in the case of spherocytosis. We report a case of hereditary spherocytosis. It is presented with an unilateral paravertebral posterior mediastinal mass. After splenectomy, it revealed shrinkage and fatty replacement on serial CT scans.

  12. Serial CT Findings of Resolving Extramedullary Hematopoiesis as Unilateral Posterior Mediastinal Mass after Splenectomy in Hereditary Spherocytosis: A Case Report

    Energy Technology Data Exchange (ETDEWEB)

    Nam, Mi Yeon; Lee, Ju Won; Kim, Yeo Ju; Kim, Youn Jeong; Kang, Young Hye; Lee, Kyung Hee [Dept. of Radiology, Inha University Hospital, Incheon (Korea, Republic of)

    2012-03-15

    Intrathoracic extramedullary hematopoiesis (EMH) is a rare condition of the hereditary spherocytosis. EMH usually regresses or disappears after treatment; such as splenectomy in the case of spherocytosis. We report a case of hereditary spherocytosis. It is presented with an unilateral paravertebral posterior mediastinal mass. After splenectomy, it revealed shrinkage and fatty replacement on serial CT scans.

  13. Ectopic extramedullary hematopoiesis: evaluation and treatment of a rare and benign paraspinal/epidural tumor.

    Science.gov (United States)

    Mattei, Tobias A; Higgins, Michael; Joseph, Flynn; Mendel, Ehud

    2013-03-01

    Ectopic extramedullary hematopoiesis (EMH), defined as the formation of blood cells outside the bone marrow, usually occurs in a scenario of chronic anemia when, even after conversion of the bony yellow marrow to red marrow, the body is still unable to meet the demand for red blood cells. Ectopic extramedullary hematopoiesis most commonly occurs in the liver and spleen but may, in fact, occur almost anywhere in the body. Although previous reports have documented EMH presenting as paraspinal masses, such lesions have almost always been associated with a predisposing hematological disorder such as hemolytic anemia, myelofibrosis or myelodysplastic syndromes, thalassemia, polycythemia vera, leukemia, or lymphoma. The authors of this report describe the first reported instance of EMH in a patient presenting with a symptomatic epidural and paraspinal cervical lesion arising from the posterior spinal elements and no known predisposing hematological disease. Initial radiographs revealed a bony lesion arising posteriorly from the C2-3 laminae and spinous processes. Subsequent imaging suggested the diagnosis, which was confirmed by CT-guided biopsy, peripheral blood smears, and bone marrow aspirate. Despite epidural compression and slight displacement of the cervical cord and thecal sac, the patient's symptoms were limited to pain and diminished cervical range of motion. Therefore, surgery was deferred in favor of nonsurgical therapy. Several alternative modalities for the treatment of EMH have been suggested in the literature, including cytotoxic agents and radiotherapy. The authors opted for an approach utilizing directed low-dose radiotherapy of a total of 25 Gy divided in 2.5-Gy fractions. At the 3-month follow-up, the patient continued to be asymptomatic, and MRI demonstrated a significant reduction in the dimensions of the lesion. Extramedullary hematopoiesis with spinal cord compression in the absence of a preexisting hematological disorder has not been described in

  14. Intrathoracic extramedullary hematopoiesis: appearance on /sup 99m/Tc sulfur colloid marrow scan

    International Nuclear Information System (INIS)

    Bronn, L.J.; Paquelet, J.R.; Tetalman, M.R.

    1980-01-01

    Imaging of the bone marrow by radionuclide scanning was performed using colloids, which are phagocytized by the reticuloendothelial cells of the marrow, or radioiron, which is incorporated into reticulocytes. The use of the former radiopharmaceutical is based on the assumption, generally valid except in aplastic states or after irradiation, that the distribution of hematopoietic and reticuloendothelial tissue in the marrow is similar. Regardless of the method used, active adult marrow is normally distributed only in the axial skeleton and proximal humeri and femurs. Marrow imaging has been used in the evaluation of myeloproliferative disorders, leukemia, lymphoma, aplastic states, malignancy metastatic to marrow, and hemolytic anemia. We report a case of thalassemia major in which the diagnosis of intrathoracic extramedullary hematopoiesis was confirmed with the /sup 99m/Tc sulfur colloid bone marrow scan

  15. Extramedullary hematopoiesis with spinal cord compression in a child with thalassemia intermedia.

    Science.gov (United States)

    Ileri, Talia; Azik, Fatih; Ertem, Mehmet; Uysal, Zumrut; Gozdasoglu, Sevgi

    2009-09-01

    Spinal cord compression due to extramedullary hematopoiesis is an extremely rare complication of thalassemia intermedia. No cases with this complication have been reported in the first decade of life, because masses of heterotropic marrow developed in patients as a result of continuous erythropoiesis. We report the 9-year-old patient suffering from thalassemia intermedia and presenting spinal cord compression. We also review the literature about treatment options, because there is no consensus about the optimal treatment of these patients. Our patient was successfully treated with radiation therapy followed by hydroxyurea. With this combination therapy, he had no recurrence during the 4-year follow-up period. Clinical awareness of this phenomenon with the early treatment is essential for optimizing the successful outcome.

  16. Maxillofacial extramedullary hematopoiesis in a child with sickle cell presenting as bilateral periorbital cellulitis.

    Science.gov (United States)

    Reiersen, David A; Mandava, Mamatha; Jeroudi, Majed; Gungor, Anil

    2014-07-01

    Review of a case of paraosseous extramedullary hematopoiesis (EMH) affecting the maxillary sinuses and retro-orbital spaces imitating bilateral orbital cellulitis. Maxillofacial EMH causes diagnostic/therapeutic challenges. This case report describes a 4-year-old African American male with sickle cell disease (HbSS) who presented with bilateral orbitofacial swelling. Diagnosis was made with imaging and confirmed with tissue sampling. Partial exchange transfusion was utilized to stop the progression of maxillofacial EMH and to treat the patient's chronic anemia. Follow-up MRI showed regression of orbital and retro-orbital involvement. Early treatment with conservative modalities and close observation may prevent need for more invasive treatments. Copyright © 2014 Elsevier Ireland Ltd. All rights reserved.

  17. A Rare Presentation of Extramedullary Hematopoiesis in Post-polycythemic Myelofibrosis.

    Science.gov (United States)

    Konca Degertekin, Ceyla; Ozkurt, Zübeyde Nur; Akyürek, Nalan; Yağcı, Münci

    2014-09-01

    Polycythemia vera is a clonal proliferative disorder of the bone marrow that could possibly evolve into myelofibrosis in its natural course. Progression to myelofibrosis is usually a late stage complication and presents clinically with refractory cytopenias and extramedullary hematopoiesis (EMH). EMH can occur in any tissue during the course of post-polycythemic myelofibrosis. However, skin and cardiac involvements seems to be very rare. We present a 56-year-old woman with post-polycythemic myelofibrosis refractory to treatment, developing EMH after splenectomy in various organs, exceptionally the skin and the heart. Along with the case, the clinical presentations, treatment options, prognostic significance of EMH and the role of cytogenetics is discussed in the light of the literature.

  18. [Spinal cord compression due to extramedullary hematopoiesis in a patient with myelofibrosis].

    Science.gov (United States)

    Hijikata, Yasuhiro; Ando, Tetsuo; Inagaki, Tomonori; Watanabe, Hirohisa; Ito, Mizuki; Sobue, Gen

    2014-01-01

    Development and growth of hematopoietic tissue outside of the bone marrow is termed extramedullary hematopoiesis (EMH). It occurs in patients with hematological diseases such as myelofibrosis and thalassemia. Liver and spleen are the usual sites of EMH. However, spinal cord compression caused by EMH is a rare complication. A 65-year-old man with myelofibrosis was admitted to our hospital with progressive paraparesis. Thoracic spine MRI revealed epidural masses causing cord compression. Histological examination of the epidural mass showed evidence of EMH consisting of megakaryocytic and erythroid hyperplasia. After surgical decompression and radiotherapy, lower limb weakness and sensory disturbance were significantly improved. MRI showed disappearance of the spinal cord compression. With this therapy, he had no recurrence until he died of myelofibrosis. Spinal EMH should be considered as a differential diagnosis in patients with hematological diseases presenting with paraparesis. Surgical decompression and radiotherapy are effective approaches for the treatment of paraparesis due to EMH.

  19. Extramedullary hematopoiesis and paraplegia in a patient with hemoglobin e-Beta thalassemia.

    Science.gov (United States)

    Alam, M R; Habib, M S; Dhakal, G P; Khan, M R; Rahim, M A; Chowdhury, A J; Mahmud, T K

    2010-07-01

    Extramedullary hematopoiesis (EMH) occurs in patients with various hematologic disorders involving a chronic increase in the production of red blood cells, and is often associated polycythemia vera and sickle cell anaemia, but is less common with thalassemia especially with hemoglobin E-beta thalassemia. Spinal cord compression due to EMH is a extremely rare complication of thalassemia and may present with paraparesis or paraplegia with or without sensory impairment. Treatment options mostly include surgery and/or radiotherapy. Whereas cases presenting with paraplegia have been treated with either surgery or radiotherapy with equal frequency and efficacy, almost all reported cases with paraplegia have been treated with surgery with or without radiation therapy. We hereby report a case of hemoglobin E-beta thalassemia with paraplegia treated successfully with radiotherapy.

  20. Cord Compression due to Extramedullary Hematopoiesis in an Adolescent with Known Beta Thalassemia Major

    Science.gov (United States)

    Soman, Salil; Rosenfeld, David L; Roychowdhury, Sudipta; Drachtman, Richard A; Cohler, Alan

    2009-01-01

    We describe a 16 year-old male with β thalassemia major and gait disturbances that had not been given blood transfusions due to a severe childhood transfusion reaction. Thoracic spine MRI demonstrated hematopoietic marrow throughout the spine and epidural masses causing cord compression consistent with extramedullary hematopoiesis (EMH). After treatment with steroids, radiotherapy and monitored blood transfusions, the patient demonstrated significant improvement of his paraspinal lesions and near complete resolution of his neurological symptoms. While EMH causing cord compression in adolescents is rare in the current era of bone marrow transplantation or chronic transfusions, it should be considered when thalassemia major patients present with neurological deficits. The well defined imaging features of EMH can play a central role in its diagnosis and management, especially because surgical and / or radiotherapeutic intervention are often considered in cases of failed medical treatment. PMID:22470615

  1. Cord Compression due to Extramedullary Hematopoiesis in an Adolescent with Known Beta Thalassemia Major

    Directory of Open Access Journals (Sweden)

    Alan COHLER

    2009-01-01

    Full Text Available We describe a 16 year-old male with ß thalassemia major and gait disturbances that had not been given blood transfusions due to a severe childhood transfusion reaction. Thoracic spine MRI demonstrated hematopoietic marrow throughout the spine and epidural masses causing cord compression consistent with extramedullary hematopoiesis (EMH. After treatment with steroids, radiotherapy and monitored blood transfusions, the patient demonstrated significant improvement of his paraspinal lesions and near complete resolution of his neurological symptoms. While EMH causing cord compression in adolescents is rare in the current era of bone marrow transplantation or chronic transfusions, it should be considered when thalassemia major patients present with neurological deficits. The well defined imaging features of EMH can play a central role in its diagnosis and management, especially because surgical and / or radiotherapeutic intervention are often considered in cases of failed medical treatment.

  2. Idiopathic myelofibrosis accompanied by peritoneal extramedullary hematopoiesis presenting as refractory ascites in a dog.

    Science.gov (United States)

    Rautenbach, Yolandi; Goddard, Amelia; Clift, Sarah J

    2017-03-01

    A 2.5-year-old spayed female American Pit Bull Terrier dog presented with a primary complaint of chronic refractory ascites. The dog's CBC displayed a moderate to severe macrocytic, hypochromic, nonregenerative anemia, and a moderate leukopenia as result of a moderate neutropenia and monocytopenia. Microscopic examination of the blood smear showed marked anisocytosis, mild polychromasia, mild acanthocytosis and ovalocytosis, moderate schistocytosis and poikilocytosis, and 4 metarubricytes/100 WBC. Abdominal ultrasonography revealed a homogenous, mild to moderately hyperechoic appearing liver as well as marked amounts of speckled anechoic to slightly hypoechoic peritoneal fluid. Cytology of the ascitic fluid demonstrated a sterile transudate, with evidence of a chronic inflammatory reaction as well as erythroid and myeloid precursor cells, and a few megakaryocytes with occasional micromegakaryocytes. Histologic sections of bone marrow, spleen, and liver were examined, using routine H&E stains, as well as a variety of immunohistochemistry and other special stains. Histopathology of the bone marrow and spleen revealed varying degrees of fibrosis, erythroid, and myeloid hyperplasia, as well as multiple small hyperplastic clusters of megakaryocytes. The megakaryocytes displayed many features of atypia such as increased cytoplasmic basophilia and occasional abnormal chromatin clumping with mitoses. Histopathologic examination of the liver disclosed evidence of mild extramedullary hematopoiesis. This case represents the first report of canine idiopathic myelofibrosis associated with peritoneal extramedullary hematopoiesis, resulting in refractory ascites. Although idiopathic myelofibrosis is a relatively rare condition in dogs, this case demonstrates that ascites caused by peritoneal implants of hematopoietic tissue may be the initial manifestation of myelofibrosis. © 2016 American Society for Veterinary Clinical Pathology.

  3. Intracranial extramedullary hematopoiesis in patients with thalassemia: a case report and review of the literature.

    Science.gov (United States)

    Eskazan, Ahmet Emre; Ar, Muhlis Cem; Baslar, Zafer

    2012-08-01

    Extramedullary hematopoiesis (EH) is a compensatory phenomenon that results in the production of blood cell precursors outside the marrow in patients with chronic hemolytic anemia and ineffective erythropoiesis. EH usually involves the liver, spleen, and lymph nodes. It can also be found at paravertebral, intrathoracic, or pelvic locations. Intracranial EH is a rare entity and often asymptomatic but can sometimes lead to symptomatic tumor-like masses. Treatment options are controversial and include hypertransfusion, surgical excision, radiotherapy, and hydroxyurea (HU). Successful treatment of an intracranial EH mass with HU and blood transfusions in a beta-thalassemia major patient was discussed along with a review of the published literature on intracranial EH in thalassemia. In our patient, the extramedullary hematopoietic mass in the interhemispheric fissure showed a marked improvement after 6 months of HU and hypertransfusion therapy. In the English literature, there are a few cases with intracranial EH and thalassemia, which were treated with different treatment modalities, with different outcomes. There is no standard treatment approach in patients with symptomatic EH. HU with hypertransfusion regimen is a reasonable first-choice modality in treating intracranial EH masses. © 2012 American Association of Blood Banks.

  4. Helical tomotherapy for extramedullary hematopoiesis involving the pericardium in a patient with chronic myeloid leukemia.

    Science.gov (United States)

    Toms, Daniel R; Cannick, Leander; Stuart, Robert K; Jenrette, Joseph M; Terwiliger, Lacy

    2010-07-01

    Extramedullary hematopoiesis (EMH) refers to the development of foci of hematopoiesis outside its normal location in the bone marrow. This occurs normally during fetal development but is abnormal postpartum. The most common sites of EMH are the spleen and liver. The phenomenon occurs in a number of disease states, notably in myelofibrosis, thalassemia, immune thrombocytopenic purpura, sickle cell anemia, polycythemia vera, and myelodysplastic syndrome. Affected patients often develop symptoms related to the location of the EMH. Reported treatments include red blood cell transfusions, surgical excision, decompressive laminectomy in cases of cord compression, chemotherapy, and irradiation. Radiation therapy is highly effective for treating hematopoietic tissue because such tissues are extremely radiosensitive. Megavoltage helical tomotherapy is a technical advance in the delivery of radiation therapy, allowing more conformal and precise treatments. The present case report describes a patient with the diagnosis of atypical chronic myeloid leukemia and myelofibrosis who subsequently developed EMH of the pericardium with effusion and tamponade. By utilizing tomotherapy we were able to treat the pericardium while sparing much of the myocardium. The patient tolerated treatment well without acute adverse effects. His symptoms were alleviated, but he died approximately 1 year later.

  5. 27-Hydroxycholesterol induces hematopoietic stem cell mobilization and extramedullary hematopoiesis during pregnancy.

    Science.gov (United States)

    Oguro, Hideyuki; McDonald, Jeffrey G; Zhao, Zhiyu; Umetani, Michihisa; Shaul, Philip W; Morrison, Sean J

    2017-09-01

    Extramedullary hematopoiesis (EMH) is induced during pregnancy to support rapid expansion of maternal blood volume. EMH activation requires hematopoietic stem cell (HSC) proliferation and mobilization, processes that depend upon estrogen receptor α (ERα) in HSCs. Here we show that treating mice with estradiol to model estradiol increases during pregnancy induced HSC proliferation in the bone marrow but not HSC mobilization. Treatment with the alternative ERα ligand 27-hydroxycholesterol (27HC) induced ERα-dependent HSC mobilization and EMH but not HSC division in the bone marrow. During pregnancy, 27HC levels increased in hematopoietic stem/progenitor cells as a result of CYP27A1, a cholesterol hydroxylase. Cyp27a1-deficient mice had significantly reduced 27HC levels, HSC mobilization, and EMH during pregnancy but normal bone marrow hematopoiesis and EMH in response to bleeding or G-CSF treatment. Distinct hematopoietic stresses thus induce EMH through different mechanisms. Two different ERα ligands, estradiol and 27HC, work together to promote EMH during pregnancy, revealing a collaboration of hormonal and metabolic mechanisms as well as a physiological function for 27HC in normal mice.

  6. Extramedullary hematopoiesis presented as cytopenia and massive paraspinal masses leading to cord compression in a patient with hereditary persistence of fetal hemoglobin

    OpenAIRE

    Katchi, Tasleem; Kolandaivel, Krishna; Khattar, Pallavi; Farooq, Taliya; Islam, Humayun; Liu, Delong

    2016-01-01

    Background Extramedullary hematopoeisis (EMH) can occur in various physiological and pathologic states. The spleen is the most common site of EMH. Case presentation We report a case with hereditary persistence of fetal hemoglobin with extramedullary hematopoiesis presented as cord compression and cytopenia secondary to multi-paraspinal masses. Conclusion Treatment can be a challenge. Relapse is a possibility.

  7. Extramedullary hematopoiesis presented as cytopenia and massive paraspinal masses leading to cord compression in a patient with hereditary persistence of fetal hemoglobin.

    Science.gov (United States)

    Katchi, Tasleem; Kolandaivel, Krishna; Khattar, Pallavi; Farooq, Taliya; Islam, Humayun; Liu, Delong

    2016-01-01

    Extramedullary hematopoeisis (EMH) can occur in various physiological and pathologic states. The spleen is the most common site of EMH. We report a case with hereditary persistence of fetal hemoglobin with extramedullary hematopoiesis presented as cord compression and cytopenia secondary to multi-paraspinal masses. Treatment can be a challenge. Relapse is a possibility.

  8. Intrathoracic extramedullary hematopoiesis presenting as tumor-simulating lesions of the mediastinum in α-thalassemia: A case report.

    Science.gov (United States)

    An, Jun; Weng, Yimin; He, Jinyuan; Li, Yun; Huang, Shaohong; Cai, Songwang; Zhang, Junhang

    2015-10-01

    Extramedullary hematopoiesis (EMH) is a rare disease, where hematological disorder drives extramedullary hematopoietic tumor formation in multiple regions of the body. The present study reports a case of EMH presenting as multiple tumor-like lesions of mediastinum in a 61-year-old male with α-thalassemia, which was subjected to a video-assisted thoracoscopic surgery tissue biopsy to differentiate it from other mediastinal tumors. To date, only three cases of EMH in patients with α-thalassemia have been described in the literature. Patients with EMH typically exhibit no hematological disorder preoperatively and therefore EMH is frequently misdiagnosed. In the present study, along with a literature review of the clinicopathological features of EMH, the diagnosis and treatment of this rare case was discussed, in order to differentiate diagnosis, and particularly to distinguish EHM from extramedullary myeloid sarcoma.

  9. Role of the spleen in cyclophosphamide-induced hematosuppression and extramedullary hematopoiesis in mice.

    Science.gov (United States)

    Wang, Yuli; Meng, Qinggang; Qiao, Haiquan; Jiang, Hongchi; Sun, Xueying

    2009-05-01

    Extramedullary hematopoiesis (EMH) is induced in spleens due to various diseases. The aim of this study is to investigate the role of spleen in cyclophosphamide (CTX)-induced hematosuppression and EMH in mice. Balb/c mice were IP injected with 300 mg/kg CTX 2 weeks after splenectomy or sham operation and randomly sacrificed 1, 3, 7, 14, and 21 days after injection. Blood samples were collected, and spleens were weighed, histologically analyzed, and then used for flow cytometry. There were significant differences in white blood count, red blood count, platelet numbers and hemoglobin concentration between the splenectomized and sham-operated mice after CTX injection. The cellularity of the spleen was reduced 3 days following CTX treatment but then rose 7 days after CTX treatment. The numbers of colony-forming units in the spleen reached a peak 7 days after CTX injection, then declined. Flow cytometry demonstrated the percentage of CD34(+) and CD117(+) cells in the spleen increased 7 days after CTX injection, indicating the hematopoietic stem and progenitor cells in the spleen. The study indicates that EMH occurs as a compensatory reaction to CTX-induced hematosuppression in the murine spleen, implying that conservation of the spleen may promote the recovery of cancer patients from chemotherapy-induced hematosuppression.

  10. Extramedullary hematopoiesis of the liver in a child with sickle cell disease: A rare complication.

    Science.gov (United States)

    Barrier, Angela; Willy, Simo; Slone, Jeremy S

    2015-08-01

    We present the case of a 7-year-old Cameroonian girl with sickle cell disease (SCD) who presented with progressive abdominal distension, fever, severe anemia, respiratory distress, and fatigue. Abdominal ultrasound showed a 15.3 cm × 11.5 cm × 15.5 cm solid echogenic mass within the left lobe of the liver. Fine-needle aspiration showed features of extramedullary hematopoiesis (EMH). Despite transfusions, antibiotics, and initiation of hydroxyurea the patient died of respiratory failure during the hospital stay. There is a paucity of information on EMH in the pediatric sickle cell population, especially from resource-limited settings such as western Africa. EMH, however, is a known complication of SCD and should be considered in patients presenting with mass lesions in the setting of chronic anemia. With limited therapeutic interventions for EMH, including radiation and hydroxyurea, the emphasis should be on improving overall treatment of patients with chronic and untreated hemolytic anemia, especially in low-income countries. © 2015 Japan Pediatric Society.

  11. Chronic Idiopathic Myelofibrosis Presenting as Cauda Equina Compression due to Extramedullary Hematopoiesis: A Case Report

    Science.gov (United States)

    Goh, Duck-Ho; Cho, Dae-Chul; Park, Seong-Hyun; Hwang, Jeong-Hyun; Sung, Joo-Kyung

    2007-01-01

    Extramedullary hematopoiesis (EMH) is occasionally reported in idiopathic myelofibrosis and is generally found in the liver, spleen, and lymph nodes several years after diagnosis. Myelofibrosis presenting as spinal cord compression, resulting from EMH tissue is very rare. A 39-yr-old man presented with back pain, subjective weakness and numbness in both legs. Sagittal magnetic resonance imaging showed multiple anterior epidural mass extending from L4 to S1 with compression of cauda equina and nerve root. The patient underwent gross total removal of the mass via L4, 5, and S1 laminectomy. Histological analysis showed islands of myelopoietic cells surrounded by fatty tissue, consistent with EMH, and bone marrow biopsy performed after surgery revealed hypercellular marrow and megakaryocytic hyperplasia and focal fibrosis. The final diagnosis was chronic idiopathic myelofibrosis leading to EMH in the lumbar spinal canal. Since there were no abnormal hematological findings except mild myelofibrosis, additional treatment such as radiothepary was not administered postoperatively for fear of radiotoxicity. On 6 month follow-up examination, the patient remained clinically stable without recurrence. This is the first case of chronic idiopathic myelofibrosis due to EMH tissue in the lumbar spinal canal in Korea. PMID:18162730

  12. The protumorigenic potential of FTY720 by promoting extramedullary hematopoiesis and MDSC accumulation.

    Science.gov (United States)

    Li, Y; Zhou, T; Wang, Y; Ning, C; Lv, Z; Han, G; Morris, J C; Taylor, E N; Wang, R; Xiao, H; Hou, C; Ma, Y; Shen, B; Feng, J; Guo, R; Li, Y; Chen, G

    2017-06-29

    FTY720 (also called fingolimod) is recognized as an immunosuppressant and has been approved by the Food and Drug Administration to treat refractory multiple sclerosis. However, long-term administration of FTY720 potentially increases the risk for cancer in recipients. The underlying mechanisms remain poorly understood. Herein, we provided evidence that FTY720 administration potentiated tumor growth. Mechanistically, FTY720 enhanced extramedullary hematopoiesis and massive accumulation of myeloid-derived suppressor cells (MDSCs), which actively suppressed antitumor immune responses. Granulocyte-macrophage colony-stimulating factor (GM-CSF), mainly produced by MDSCs, was identified as a key factor to mediate these effects of FTY720 in tumor microenvironment. Furthermore, we showed that FTY720 triggers MDSCs to release GM-CSF via S1P receptor 3 (S1pr3) through Rho kinase and extracellular signal-regulated kinase-dependent pathway. Thus, our findings provide mechanistic explanation for the protumorigenic potentials of FTY720 and suggest that targeting S1pr3 simultaneously may be beneficial for the patients receiving FTY720 treatment.

  13. Fluorine-18 fluorodeoxyglucose splenic uptake from extramedullary hematopoiesis after granulocyte colony-stimulating factor stimulation.

    Science.gov (United States)

    Abdel-Dayem, H M; Rosen, G; El-Zeftawy, H; Naddaf, S; Kumar, M; Atay, S; Cacavio, A

    1999-05-01

    Two patients with sarcoma, one with recurrent osteosarcoma of the spine and the other with metastatic synovial cell sarcoma, were treated with high-dose chemotherapy that produced severe leukopenia. The patients received granulocyte colony-stimulating factor (G-CSF) to stimulate the bone marrow (480 mg given subcutaneously twice daily for 5 to 7 days); their responses were seen as a marked increase in peripheral leukocyte count with no change in the erythrocyte or platelet counts. The patients had fluorine-18 fluorodeoxyglucose (F-18 FDG) imaging 24 hours after the end of G-CSF treatment. Diffusely increased uptake of F-18 FDG was seen in the bone marrow in both patients. In addition, markedly increased uptake in the spleen was noted in both, indicating that the spleen was the site of extramedullary hematopoiesis. The patients had no evidence of splenic metastases. The first patient had a history of irradiation to the dorsal spine, which was less responsive to G-CSF administration than was the nonirradiated lumbar spine.

  14. Spinal cord compression due to extramedullary hematopoiesis in beta-thalassemia intermedia

    International Nuclear Information System (INIS)

    Munn, Rita K.; Kramer, Carol A.; Arnold, Susanne M.

    1998-01-01

    Background: Extramedullary hematopoiesis (EMH) occurs in many disorders, including thalassemias and other hemoglobinopathies, and commonly presents in the spleen and liver. We present a case of spinal cord compression in a patient with beta-thalassemia intermedia, and review the literature and available treatment options. Patient and Methods: A 35-year-old black female with beta-thalassemia intermedia presented with a 3-week history of back pain and lower extremity weakness. Neurologic examination was consistent with spinal cord compression, and gadolinium enhanced magnetic resonance imaging (MRI) confirmed this diagnosis. She was given intravenous steroids and radiotherapy was begun in 200 cGy fractions to a total dose of 2000 cGy. Results: At the completion of radiotherapy the patient was ambulatory with mild residual weakness. MRI scans 16 months later showed smaller, but persistent masses, and she remains asymptomatic 5 years from her diagnosis. Conclusion: Recognition of spinal cord EMH requires prompt physical examination and MRI for accurate diagnosis. EMH can be managed with radiation, surgery, transfusions, or a combination of these therapies. Radiation in conservative doses of (750-3500 cGy) is non-invasive, avoids the surgical risks of potentially severe hemorrhage and incomplete resection, and has a high complete remission rate in the majority of patients. Relapse rates are moderate (37.5%), but retreatment provides excellent chance for second remission

  15. Nephrotic syndrome in primary myelofibrosis with renal extramedullary hematopoiesis and glomerulopathy in the JAK inhibitor era.

    Science.gov (United States)

    Del Sordo, Rachele; Brugnano, Rachele; Covarelli, Carla; Fiorucci, Gioia; Falzetti, Franca; Barbatelli, Giorgio; Nunzi, Emidio; Sidoni, Angelo

    2017-01-01

    Primary myelofibrosis (PMF) is an uncommon form of myeloproliferative neoplasm (MPN) characterized by a proliferation of predominantly megakaryocytes and granulocytes in the bone marrow that, in fully-developed disease, is associated with reactive deposition of fibrous connective tissue, extramedullary hematopoiesis (EMH), and splenomegaly. Kidney involvement is rare and clinically presents with proteinuria, nephrotic syndrome, and renal insufficiency. Renal damage can be due to EMH and glomerulopathy. Renal EMH presents three patterns: infiltration of the interstitium with possible renal failure caused by functional damage of parenchyma and vessels, infiltration of capsule and pericapsular adipose tissue, and sclerosing mass-like lesions that can cause hydronephrosis and hydroureter with obstructive uropathy and renal failure. Glomerulopathy associated with PMF is rarely described, ranging from 1 month to 18 years from diagnosis of the neoplasm to renal biopsy. It is characterized by expansion and hypercellularity mesangial, segmental sclerosis, features of chronic thrombotic microangiopathy (TMA), and intracapillary hematopoietic cells infiltrating in absence of immune-mediated glomerulonephritis. We present a nephrotic syndrome in PMF-related glomerulopathy, associated with EMH, without renal failure, in a patient under treatment for 2 years with JAK2 inhibitor ruxolitinib. Despite treatment, the patient died 7 months after renal biopsy. Nephrologists still know very little about this topic and there is no homogeneous data about incidence, pathogenesis, and optimal treatment of this poor prognostic PMF-associated nephrotic syndrome. We focus on data in the literature in the hope of stimulating hematologists, nephrologists, pathologists to future studies about the natural history of renal involvement, useful for optimal management of this rare pathology.

  16. Hydroxyurea as a first-line treatment of extramedullary hematopoiesis in patients with beta thalassemia: Four case reports.

    Science.gov (United States)

    Karimi, Mehran; Cohan, Nader; Pishdad, Parisa

    2015-01-01

    Extramedullary hematopoiesis (EMH) is evidenced by erythropoietic masses, which occurs as a compensatory mechanism to overcome hypoxia during chronic anemia. EMH masses in spinal cord could lead to cord compression and neurological symptoms. Besides transfusion, radiotherapy, and surgery, hydroxyurea (HU) is also a treatment strategy in EMH. We described four cases of beta thalassemia with EMH who were treated with HU as a monotherapy. INTERVENTION (AND TECHNIQUE): HU therapy was done in all patients without any transfusion during therapy. HU is a good treatment option for patients with EMH and it could be a substitute for radiotherapy and invasive surgery or regular blood transfusion.

  17. A persistent cough as atypical clinical presentation of intrathoracic extramedullary hematopoiesis (EMH) in a female with thalassemia intermedia.

    Science.gov (United States)

    Abdulla, Mohammad Aj; Yassin, Mohamed A; Abdelrazek, Mohamed; Mudawi, Deena; Ibrahim, Firyal; Soliman, Dina S; ElOmri, Halima; Nashwan, Abdulqadir J; Fernihough, Liam; De Sanctis, Vincenzo; Soliman, Ashraf T

    2018-02-16

    Extramedullary hematopoiesis (EMH) is a rare disorder, defined as the appearance of hematopoietic elements outside the bone marrow or peripheral blood. The most common sites of EMH are liver and spleen, but it has been documented in other organs such as the mediastinum, lymph nodes, breast, and central nervous system. EMH occurs as a compensatory mechanism for bone marrow dysfunction in severe thalassemia. We report a case of EMH presenting as a posterior mediastinal mass in a 34-year-old woman with thalassemia intermedia with chronic cough and shortness of breath on exertion. The diagnosis of EMH was confirmed by a CT-guided fine needle biopsy. All symptoms disappeared after surgical removal of the mass.

  18. C-X-C motif chemokine 12 influences the development of extramedullary hematopoiesis in the spleens of myelofibrosis patients.

    Science.gov (United States)

    Wang, Xiaoli; Cho, Sool Yeon; Hu, Cing Siang; Chen, Daniel; Roboz, John; Hoffman, Ronald

    2015-02-01

    Myelofibrosis (MF) is characterized by the constitutive mobilization of hematopoietic stem cells (HSC) and hematopoietic progenitor cells (HPC) and the establishment of extramedullary hematopoiesis. The mechanisms underlying this abnormal HSC/HPC trafficking pattern remain poorly understood. We demonstrated that both splenic and peripheral blood (PB) MF CD34(+) cells equally share a defective ability to home to the marrow, but not to the spleens, of NOD/LtSz-Prkdc(scid) mice. This trafficking pattern could not be attributed to discordant expression of integrins or chemokine receptors other than the downregulation of C-X-C chemokine receptor type 4 by both PB and splenic MF CD34(+) cells. The number of both splenic MF CD34(+) cells and HPCs that migrated toward splenic MF plasma was, however, significantly greater than the number that migrated toward PB MF plasma. The concentration of the intact HSC/HPC chemoattractant C-X-C motif chemokine 12 (CXCL12) was greater in splenic MF plasma than PB MF plasma, as quantified using mass spectrometry. Functionally inactive truncated products of CXCL12, which are the product of proteolytic degradation by serine proteases, were detected at similar levels in both splenic and PB MF plasma. Treatment with an anti-CXCL12 neutralizing antibody resulted in a reduction in the degree of migration of splenic MF CD34(+) cells toward both PB and splenic MF plasma, validating the role of CXCL12 as a functional chemoattractant. Our data indicate that the MF splenic microenvironment is characterized by increased levels of intact, functional CXCL12, which contributes to the localization of MF CD34(+) cells to the spleen and the establishment of extramedullary hematopoiesis. Copyright © 2015 ISEH - International Society for Experimental Hematology. Published by Elsevier Inc. All rights reserved.

  19. Extramedullary hematopoiesis in a case of benign mixed mammary tumor in a female dog: cytological and histopathological assessment

    Directory of Open Access Journals (Sweden)

    Leão João

    2010-09-01

    Full Text Available Abstract Backgroud Extramedullary hematopoiesis (EMH is defined as the presence of hematopoietic stem cells such as erythroid and myeloid lineage plus megakaryocytes in extramedullary sites like liver, spleen and lymph nodes and is usually associated with either bone marrow or hematological disorders. Mammary EMH is a rare condition either in human and veterinary medicine and can be associated with benign mixed mammary tumors, similarly to that described in this case. Case presentation Hematopoietic stem cells were found in a benign mixed mammary tumor of a 7-year-old female mongrel dog that presents a nodule in the left inguinal mammary gland. The patient did not have any hematological abnormalities. Cytological evaluation demonstrated two distinct cell populations, composed of either epithelial or mesenchymal cells, sometimes associated with a fibrillar acidophilic matrix, apart from megakaryocytes, osteoclasts, metarubricytes, prorubricytes, rubricytes, rubriblasts, promyelocytes, myeloblasts. Histological examination confirmed the presence of an active hematopoietic bone marrow within the bone tissue of a benign mammary mixed tumor. Conclusions EMH is a rare condition described in veterinary medicine that can be associated with mammary mixed tumors. It's detection can be associated with several neoplastic and non-neoplastic mammary lesions, i.e. osteosarcomas, mixed tumors and bone metaplasia.

  20. Spinal cord compression secondary to extramedullary hematopoiesis: A rareness in a young adult with thalassemia major.

    Science.gov (United States)

    Fareed, Shehab; Soliman, Ashraf T; De Sanctis, Vincenzo; Kohla, Samah; Soliman, Dina; Khirfan, Diala; Tambuerello, Adriana; Talaat, Mohamed; Nashwan, Abdulqadir; Caparrotti, Palmira; Yassin, Mohamed A

    2017-08-23

    We report a case of a thalassemia major male patient with back pain associated to severe weakness in lower extremities resulting in the ability to ambulate only with assistance. An urgent magnetic resonance imaging (MRI) of  thoracic and lumbosacral spine was requested. A posterior intraspinal extradural mass lesion compressing the spinal cord at the level of thoracic T5-8 was present, suggesting an extramedullary hematopoietic centre, compressing the spinal cord. He was treated successfully with thalassemia major alone. The patient was treated with blood transfusion, dexamethasone, morphine and paracetamol, followed by radiotherapy in 10 fractions to the spine (daily fraction of 2Gy from T3 to T9, total dose 20 Gy). His pain and neurologic examination quickly improved. A new MRI of the spine, one week after radiotherapy, showed an improvement of the extramedullary hematopoietic mass compression. In conclusion, EMH should be considered in every patient with ineffective erythropoiesis and spinal cord symptoms. MRI is the most effective method of demonstrating EMH. The rapid recognition and treatment can dramatically alleviate symptoms. There is still considerable controversy regarding indications, benefits, and risks of each of modality of treatment due to the infrequency of this disorder.

  1. The effects of hematopoietic stem cell transplant on splenic extramedullary hematopoiesis in patients with myeloproliferative neoplasm-associated myelofibrosis.

    Science.gov (United States)

    Pizzi, Marco; Gergis, Usama; Chaviano, Felicia; Orazi, Attilio

    2016-09-01

    Hematopoietic stem cell transplant (HSCT) is the only curative treatment for myeloproliferative neoplasm-associated myelofibrosis (MPN-MF). The main clinical manifestation of MPN-MF is splenomegaly secondary to extramedullary hematopoiesis (EMH). The effects of HSCT on splenic EMH and associated vascular and stromal changes are unknown. This study compares the findings seen in spleens following HSCT with those of nontransplanted patients, normal controls, and matched bone marrow (BM) samples. This study included three transplanted MPN-MF spleens, three nontransplanted MPN-MF spleens, and three normal controls. Spleens were assessed for: (a) presence/extent of EMH; (b) presence of Gamna-Gandy bodies; (c) splenic fibrosis; (d) CD34-positive microvessel density; (e) CD8-positive sinusoids; (f) frequency of smooth muscle actin-positive myoid cells; and (g) nerve growth factor receptor-positive adventitial reticulum cells. In two cases, matched BM samples were assessed for cellularity, presence of atypical megakaryocytes, and fibrosis. Compared with normal controls, all MPN-MF spleens were larger in size, had EMH, red pulp fibrosis, higher CD34-positive microvessel density, and decreased CD8-positive sinusoids. Compared with nontransplanted cases, post-HSCT spleens showed disappearance or reduction of EMH. Gamna-Gandy bodies were increased; no differences in the remaining parameters were found. A reduction of splenic EMH was associated with normalization of BM cellularity and megakaryopoiesis. HSCT reduces/abrogates splenic EMH and is associated with an increased number of Gamna-Gandy bodies, which may suggest vascular damage. The lack of stromal changes in spleens removed shortly after transplant is in line with similar observations in the BM, where a longer interval is often necessary for resolution of fibrosis. Copyright © 2016 King Faisal Specialist Hospital & Research Centre. Published by Elsevier Ltd. All rights reserved.

  2. Hematopoiesis

    International Nuclear Information System (INIS)

    Testa, N.G.; Gale, R.P.

    1988-01-01

    Hematopoiesis critically assesses the implications of therapeutic or accidental exposure to cytotoxic drugs or radiation by analyzing their mechanisms of myelosuppression, - possible mutagenic action...capacity to induce chromosome abnormalities, - and effects on the immune system and other tissues. In addition, this reference: considers clinical and experimental observations on hemopoietic damage in the context of modern cell biology; evaluates the effects of treatment, natural history of the malignancy, and induced proliferative stress on the hemopoietic system; examines hemopoiesis within a framework relating to immunology, cytogenetics, and leukemogenesis; presents the subject in a logically developed sequence-building on fundamental biology and continuing through experimental data, clinical data, and future research trends; focuses on new research approaches to the therapy of malignancies; indicating possible approaches to correct long-term damage by control of cell proliferation and differentiation

  3. Extramedullary pulmonary hematopoiesis causing pulmonary hypertension and severe tricuspid regurgitation detected by {sup 99m} technetium sulfur colloid bone marrow scan and single-photon emission computed tomography/CT

    Energy Technology Data Exchange (ETDEWEB)

    Ali, Syed Zama; Clarke, Michael John; Kannivelu, Anbalagan; Chinchure, Dinesh; Srinivasan, Sivasubramanian [Dept. of Diagnostic Radiology, Khoo Teck Puat Hospital, Singapore (Singapore)

    2014-06-15

    Extramedullary pulmonary hematopoiesis is a rare entity with a limited number of case reports in the available literature only. We report the case of a 66-year-old man with known primary myelofibrosis, in whom a {sup 99m}technetium sulfur colloid bone marrow scan with single-photon emission computed tomography (SPECT)/CT revealed a pulmonary hematopoiesis as the cause of pulmonary hypertension and severe tricuspid regurgitation. To the best of our knowledge, this is the first description of {sup 99m} technetium sulfur colloid SPECT/CT imaging in this rare condition.

  4. Extramedullary pulmonary hematopoiesis causing pulmonary hypertension and severe tricuspid regurgitation detected by 99m technetium sulfur colloid bone marrow scan and single-photon emission computed tomography/CT

    International Nuclear Information System (INIS)

    Ali, Syed Zama; Clarke, Michael John; Kannivelu, Anbalagan; Chinchure, Dinesh; Srinivasan, Sivasubramanian

    2014-01-01

    Extramedullary pulmonary hematopoiesis is a rare entity with a limited number of case reports in the available literature only. We report the case of a 66-year-old man with known primary myelofibrosis, in whom a 99m technetium sulfur colloid bone marrow scan with single-photon emission computed tomography (SPECT)/CT revealed a pulmonary hematopoiesis as the cause of pulmonary hypertension and severe tricuspid regurgitation. To the best of our knowledge, this is the first description of 99m technetium sulfur colloid SPECT/CT imaging in this rare condition.

  5. EXEL-8232, a small-molecule JAK2 inhibitor, effectively treats thrombocytosis and extramedullary hematopoiesis in a murine model of myeloproliferative neoplasm induced by MPLW515L.

    Science.gov (United States)

    Wernig, G; Kharas, M G; Mullally, A; Leeman, D S; Okabe, R; George, T; Clary, D O; Gilliland, D G

    2012-04-01

    About 10% of patients with essential thrombocythemia (ET) or myelofibrosis (MF) that lack mutations in JAK2 harbor an activating mutation in the thrombopoietin receptor, MPLW515L. Distinct from the JAK2V617F retroviral transplant model, the MPLW515L model recapitulates many features of ET and MF, including severe fibrosis and thrombocytosis. We have tested EXEL-8232, an experimental potent JAK2 inhibitor, for efficacy in suppression of thrombocytosis in vivo and for its ability to attenuate MPLW515L myeloproliferative disease. EXEL-8232 was administered for 28 days q12 h by oral gavage at doses of 30 or 100 mg/kg, prospectively. Animals treated with EXEL-8232 at 100 mg/kg had normalized high platelet counts, eliminated extramedullary hematopoiesis in the spleen and eliminated bone marrow fibrosis, whereas the wild-type controls did not develop thrombocytopenia. Consistent with a clinical response in this model, we validated surrogate end points for response to treatment, including a reduction of endogenous colony growth and signaling inhibition in immature erythroid and myeloid primary cells both in vitro and upon treatment in vivo. We conclude that EXEL-8232 has efficacy in treatment of thrombocytosis in vivo in a murine model of ET and MF, and may be of therapeutic benefit for patients with MPL-mutant MPN.

  6. Regression of extramedullary hematopoiesis with hydroxyurea therapy in ß-thalassemia intermedia Regressão da hematopoese extramedular na talassemia intermédia após terapia com hidroxiuréia

    Directory of Open Access Journals (Sweden)

    Perla Vicari

    2006-03-01

    Full Text Available Excessive ineffective erythropoiesis in thalassemia intermedia may cause extramedullary hematopoiesis (EMH, resulting in spleen and liver enlargement or masses in several tissues, mainly paravertebrally. Other less frequent locations of diffuse compensatory EMH are kidneys, adrenal glands, breasts, spinal cord, pleura, pericardium, duramater, adipose tissue and skin, although intrathoracic extramedullary hematopoiesis is a rare condition. Management strategies have included radiation and transfusion therapy. Hydroxyurea with transfusion therapy has been associated with clinical regression of EMH in thalassemia. We report an uncommon case of intrathoracic EMH in a patient with beta-thalassemia intermedia, that showed significant recovery with HU therapy.A excessiva eritropoese ineficaz na talassemia pode causar hemato-poese extramedular (HEM, resultando em hepatomegalia, esplenomegalia e massas de tecido hematopoético em diversos tecidos. Localizações de HEM compensatória menos freqüentes são rins, glândulas adrenais, canal medular, pleura, pericárdio, duramáter, tecido adiposo e pele. Entretanto, HEM intratorácica é condição rara. Estratégias terapêuticas incluem radiação e transfusões sanguíneas. O uso de hidroxiuréia concomitante a terapêutica transfusional foi associado à regressão clínica da HEM na talassemia. Nós descrevemos um caso de HEM intratorácica em paciente portadora de talassemia intermédia, com significante regressão do quadro após terapêutica isolada com hidroxiuréia.

  7. Excellent and durable response to radiotherapy in a rare case of spinal cord compression due to extra-medullary hematopoiesis in β-thalassemia intermedia: case report and clinicoradiological correlation.

    Science.gov (United States)

    Yathiraj, Prahlad H; Singh, Anshul; Vidyasagar, Sudha; Varma, Muralidhar; Mamidipudi, Vidyasagar

    2017-04-01

    Spinal cord compression (SCC) is an unusual sequale of extra-medullary hematopoiesis (EMH). We report a patient diagnosed with β-thalassemia intermedia at the age of 7 years presenting as a 24-year-old with symptoms suggestive of paraparesis. MR imaging revealed long masses of EMH opposite T5-T11 and L5-S2 vertebrae with cord compression at T6 vertebrae. Patient was treated with external beam radiotherapy (EBRT) to a low dose of 20 Gy in 10 fractions over 2 weeks. The patient had symptomatic relief of paraparesis by the 5th fraction and nearly regained full power in bilateral lower limbs by EBRT conclusion. Patient was begun on hydroxyurea post EBRT and was symptom free at 2-month follow up. With a follow-up of 18 months so far, he remains asymptomatic and free of recurrence. MRI correlation of pre-EBRT, post-EBRT and at first follow-up showed a significant reduction in the size of EMH, increase in diameter of spinal canal post EBRT but a persistent edema which had no clinical manifestation. Though there was a 58% drop in leukocyte count by the end of EBRT, there was no leukocytopenia. We suggest that EBRT should be treatment of choice for SCC due to EMH as it produces as rapid and durable response with minimal acute hematological side-effects.

  8. Atypical location of extramedullary hematopoietic masses in thalassemia

    International Nuclear Information System (INIS)

    Gemenis, T.; Philippou, A.; Gouliamos, A.; Kalovidouris, A.; Papavasiliou, C.; Papacharalambus, X.; Panani, A.; Chalevelakis, G.; Raptis, S.

    1989-01-01

    A case of β-thalassemia with multiple foci of extramedullary hematopoiesis (EH) is reported. EH masses were demonstrated in the presacral and the costovertebral space. EH foci were also encountered in the spleen. (orig.) [de

  9. Extramedullary plasmocytomas

    International Nuclear Information System (INIS)

    Ben Salah, H.; Daoud, J.; Hdiji, S.; Elloumi, M.; Makni, S.; Boudawara, T.; Ghorbel, A.M.

    2012-01-01

    Purpose. - To study the localization, treatment and prognosis of extramedullary plasmocytoma through a series of eight patients and a literature review. Patients and methods. - Eight patients with extramedullary plasmocytoma were treated in the university's hospital of Sfax in Tunisia. The average age was 57.3 years. Female represented 75% of patients. The diagnosis of plasmocytoma was based on anatomo-pathology and immunohistochemistry of a biopsy or resected tumour. Extramedullary location was confirmed if biological and radiological exams and medullary biopsy were normal. The therapeutic decision was made after multidisciplinary meetings regarding tumour location and anterior treatment. Results. - Solitary extramedullary plasmocytoma was located in nasal cavity, cervical node, testis, ovary, bladder and the tongue. One patient was treated for three simultaneous locations of extramedullary plasmocytoma (node, bowel, pleura) without evidence of myeloma. Radiotherapy was proposed in six cases but refused in one case (plasmocytoma of the bladder is currently receiving radiotherapy). Treatment consisted in chemotherapy in two cases. Evaluation after treatment revealed complete remission in 86% of the cases. Nodal recurrence was noted in two cases. These two patients were lost to follow up. The five other patients were in complete remission after a mean follow up of 5.7 years. No local recurrence or myeloma was noted. Conclusion. - Extramedullary plasmocytoma is a rare affection. It can occur in any region of the body. Head and neck is most frequent localization. The treatment is irradiation or surgery in some localization. Progression to myeloma is the most important factor that influences the prognosis of the disease. (authors)

  10. Bilateral Pleural Effusion in a Patient with an Extensive Extramedullary Hematopoietic Mass

    Directory of Open Access Journals (Sweden)

    Yun Luo

    2013-01-01

    Full Text Available We present a 56-year-old woman with bilateral pleural effusions, widespread enlarged lymph nodes, and soft tissue masses located within the renal pelvis. The initially working diagnosis was tuberculosis and lymphoma. Further pathological examination of the lymph node biopsy confirmed a diagnosis of extramedullary hematopoiesis, and a bone marrow biopsy revealed myelofibrosis. Unlike common treatment options such as radiotherapy and/or surgery, intrathoracic cisplatin and dexamethasone for the treatment of pleural effusions secondary to extramedullary hematopoiesis demonstrated an improvement in feasibility and efficacy in the present case.

  11. Soft Tissue Extramedullary Plasmacytoma

    Directory of Open Access Journals (Sweden)

    Fernando Ruiz Santiago

    2010-01-01

    Full Text Available We present the uncommon case of a subcutaneous fascia-based extramedullary plasmacytoma in the leg, which was confirmed by the pathology report and followed up until its remission. We report the differential diagnosis with other more common soft tissue masses. Imaging findings are nonspecific but are important to determine the tumour extension and to plan the biopsy.

  12. Multiple myeloma with extramedullary disease.

    Science.gov (United States)

    Oriol, Albert

    2011-11-01

    Plasmacytoma is a tumor mass consisting of atypical plasma cells. Incidence of plasmacytomas associated with multiple myeloma range from 7% to 17% at diagnosis and from 6% to 20% during the course of the disease. In both situations, occurrence of extramedullary disease has been consistently associated with a poorer prognosis of myeloma. Extramedullary relapse or progression occurs in a variety of clinical circumstances and settings, and therefore requires individualization of treatment. Alkylating agents, bortezomib, and immunomodulatory drugs, along with corticoids, have been used to treat extramedullary relapse but, because of the relatively low frequency or detection rate of extramedullary relapse, no efficacy data are available from controlled studies in this setting.

  13. Treatment of extramedullary plasmacytoma

    International Nuclear Information System (INIS)

    Wang Weihu; Li Suyan; Gao Li; Qu Yuan; Xu Guozhen; Li Yexiong

    2004-01-01

    Objective: To analyze the clinical feature of extramedullary plasmacytoma (EMP) and treatment results. Methods: From Jan. 1960 to Aug. 2000, 28 EMP patients accumulated in a period of 40 odd years were evaluated retrospectively. Sixteen of them were treated with radiotherapy alone (R group, 57.1%) with a median dose of 50 Gy, 11 with combined modality therapy (CMT group, 39.3%) and 1 with surgery alone (S group, 3.6%). Results: During a median follow-up of 90 months (4-280 months), 2 developed local recurrence and 2 were converted to multiple myeloma. The overall 3-, 5-, and 10-year survival rates were 92.6%, 75.6% and 75.6%, respectively. In the R group, the overall 3-, 5-, and 10-year survival rates were 93.8%, 77.3% and 77.3%, as compared with 90.9%, 72.7% and 72.7% in the CMT group (P>0.05). Conclusions: Extramedullary plasmacytoma may well be cured with small field and limited dose of radiation therapy alone. The diagnosis of extramedullary plasmacytoma requires a complete work-up (including MRI) and strict criteria to exclude multiple myeloma. Close follow-up is necessary in those converted to multiple myeloma. (authors)

  14. Gut microbiota sustains hematopoiesis

    DEFF Research Database (Denmark)

    Theilgaard-Mönch, Kim

    2017-01-01

    In this issue of Blood, Josefsdottir et al provide substantial evidence that commensal gut microbes regulate and sustain normal steady-state hematopoiesis.1......In this issue of Blood, Josefsdottir et al provide substantial evidence that commensal gut microbes regulate and sustain normal steady-state hematopoiesis.1...

  15. Bilateral Pleural Effusion in a Patient with an Extensive Extramedullary Hematopoietic Mass

    OpenAIRE

    Yun Luo; Ying Zhang; Shi-feng Lou

    2013-01-01

    We present a 56-year-old woman with bilateral pleural effusions, widespread enlarged lymph nodes, and soft tissue masses located within the renal pelvis. The initially working diagnosis was tuberculosis and lymphoma. Further pathological examination of the lymph node biopsy confirmed a diagnosis of extramedullary hematopoiesis, and a bone marrow biopsy revealed myelofibrosis. Unlike common treatment options such as radiotherapy and/or surgery, intrathoracic cisplatin and dexamethasone for the...

  16. Extramedullary plasmacytoma of the larynx

    Directory of Open Access Journals (Sweden)

    Pinto, José Antônio

    2012-01-01

    Full Text Available Introduction: The extramedullary plasmocytoma is one of the localized forms of malignancy of the plasma cells, which has multiple myeloma main diagnosis. Its main site to the head and neck, but with a rare presentation in the larynx. Objective: To describe a case of extramedullary plasmocytoma of the larynx, with literature review. Case Report: Patient female, 49, referring to intermittent dysphonia for 01 years with progressive worsening associated with vocal fatigue and vocal effort, with reddish lesion, smooth edges fold left ventricular endoscopy. Being subjected to excisional biopsy diagnosed with extramedullary histopathological plasmocytoma. Conclusion: Extramedullary Plasmocytoma must be considered in the differential diagnosis of rare tumors of the larynx. It is essential after the diagnosis of multiple myeloma research and a "follow up" appropriate.

  17. Extramedullary plasmacytoma of the larynx.

    Science.gov (United States)

    Pinto, José Antônio; Sônego, Thiago Branco; Artico, Marina Spadari; Leal, Carolina de Farias Aires; Bellotto, Silvana

    2012-07-01

     The extramedullary plasmocytoma is one of the localized forms of malignancy of the plasma cells, which has multiple myeloma main diagnosis. Its main site to the head and neck, but with a rare presentation in the larynx.  To describe a case of extramedullary plasmocytoma of the larynx, with literature review.  Patient female, 49, referring to intermittent dysphonia for 01 years with progressive worsening associated with vocal fatigue and vocal effort, with reddish lesion, smooth edges fold left ventricular endoscopy. Being subjected to excisional biopsy diagnosed with extramedullary histopathological plasmocytoma.  Extramedullary Plasmocytoma must be considered in the differential diagnosis of rare tumors of the larynx. It is essential after the diagnosis of multiple myeloma research and a "follow up" appropriate.

  18. Nasal septum extramedullary plasmacytoma

    Directory of Open Access Journals (Sweden)

    Belić Branislav

    2013-01-01

    Full Text Available Introduction. Plasmacytomas are malignant tumors characterized by abnormal monoclonal proliferation of plasma cells. They originate in either bone - solitary osseous plasmacytoma, or in soft tissue - extramedullary plasmacytoma (EMP. EMP represents less than 1% of all head and neck malignancies. Case report. We presented a case of EMP of the nasal septum in a 44-year-old male who had progressive difficulty in breathing through the nose and frequent heavy epistaxis on the right side. Nasal endoscopy showed dark red, soft, polypoid tumor in the last third of the right nasal cavity arising from the nasal septum. The biopsy showed that it was plasmacytoma. Bence Jones protein in the urine, serum electrophoresis, bone marrow biopsy, skeletal survey and other screening tests failed to detect multiple myeloma. This confirmed the diagnosis of EMP. The mass was completely removed via an endoscopic approach, and then, 4 week later, radiotherapy was conducted with a radiation dose of 50 Gray. No recurrence was noted in a 3-year follow- up period. Conclusion. EMP of the nasal cavity, being rare and having long natural history, represents a diagnostic and therapeutic challenge for any ear, nose and throat surgeon. Depending on the resectability of the lesion, a combined therapy is the accepted treatment.

  19. Intrahepatic ovulation

    Directory of Open Access Journals (Sweden)

    Artur L. Wozniak, HBSc

    2014-01-01

    Full Text Available Ectopic ovaries are a rare finding in the literature, with fewer than 50 published cases to date. This phenomenon has been found in the omentum, bladder, mesentery, and uterus; attached to the colon; inside the left labia majora; and in the kidney. Various etiologies have been proposed, including postsurgical or postinflammatory transplantation, malignant origins, and abnormal embryologic development. We report the ultrasonographic, computed tomographic (CT, and magnetic resonance (MR imaging of, what is to the best of our knowledge, the first case of an intrahepatic ectopic ovary.

  20. Epidural extramedullary haemopoiesis in thalassaemia

    International Nuclear Information System (INIS)

    Boyacigil, S.; Ali, A.; Ardic, S.; Yuksel, E.

    2002-01-01

    lntrathoracic extramedullary haematopoiesis is a rare condition. Involvement of the spinal epidural space with haematopoietic tissue is rather unusual. A 31-year-old-man with a known diagnosis of β-thalassaemia was referred with focal back pain. Magnetic resonance imaging revealed diffuse bone-marrow changes, thoracic paraspinal masses and lobulated epidural masses, suggesting extramedullary haemopoiesis. The patient was treated with radiotherapy and blood transfusions. Follow-up MRI was performed for evaluation efficacy of the treatment. Copyright (2002) Blackwell Science Pty Ltd

  1. Congenital Intrahepatic Portosystemic Shunts

    Energy Technology Data Exchange (ETDEWEB)

    Lee, Woong Hee; Kim, Young Tong; Jou, Sung Shick; Shin, Hyeong Cheol [Soonchunhyang University, Asan (Korea, Republic of)

    2008-12-15

    Intrahepatic portosystemic shunts are an anomalous connection between the portal vein and hepatic vein/IVC, which may be either congenital or acquired secondary to liver cirrhosis or portal hypertension. Cases of congenital intrahepatic shunts are usually encountered in children and may spontaneously resolve. We report 5 cases of congenital intrahepatic portosystemic shunts in neonates and an adult

  2. MRI findings of extramedullary haemopoiesis

    International Nuclear Information System (INIS)

    Chourmouzi, D.; Pistevou-Gompaki, K.; Plataniotis, G.; Skaragas, G.; Papadopoulos, L.; Drevelegas, A.

    2001-01-01

    Extramedullary haemopoiesis (EH) is a compensatory process associated with chronic haemolytic anaemia. It is rare, however, for such an abnormality to cause spinal cord compression. We present two patients with known beta-thalassaemia intermedia who developed spinal cord compression due to masses of extramedullary haematopoietic tissue in the epidural space of the thoracic spine. The EH masses were diagnosed by MRI as an isointense epidural lesion on both T1- and T2-weighted images, compressing severely the spinal cord. After administration of a paramagnetic agent, an intermediate enhancement of the masses was evident. All the vertebral bodies had low to intermediate signal intensity as a result of displacement of fatty marrow by haematopoietic marrow. Expansion of thoracic ribs with bilateral paravertebral masses were characteristic. A small dose of radiotherapy was given and marked improvement in neurological symptoms was evident. An MRI examination established shrinkage of the mass and decompression of spinal cord. The role of MRI in diagnosis of EH masses is essential and radiation therapy is a very effective treatment for this rare complication. (orig.)

  3. Insect immunology and hematopoiesis.

    Science.gov (United States)

    Hillyer, Julián F

    2016-05-01

    Insects combat infection by mounting powerful immune responses that are mediated by hemocytes, the fat body, the midgut, the salivary glands and other tissues. Foreign organisms that have entered the body of an insect are recognized by the immune system when pathogen-associated molecular patterns bind host-derived pattern recognition receptors. This, in turn, activates immune signaling pathways that amplify the immune response, induce the production of factors with antimicrobial activity, and activate effector pathways. Among the immune signaling pathways are the Toll, Imd, Jak/Stat, JNK, and insulin pathways. Activation of these and other pathways leads to pathogen killing via phagocytosis, melanization, cellular encapsulation, nodulation, lysis, RNAi-mediated virus destruction, autophagy and apoptosis. This review details these and other aspects of immunity in insects, and discusses how the immune and circulatory systems have co-adapted to combat infection, how hemocyte replication and differentiation takes place (hematopoiesis), how an infection prepares an insect for a subsequent infection (immune priming), how environmental factors such as temperature and the age of the insect impact the immune response, and how social immunity protects entire groups. Finally, this review highlights some underexplored areas in the field of insect immunobiology. Copyright © 2015 Elsevier Ltd. All rights reserved.

  4. Extramedullary plasmacytoma of the testicle.

    Science.gov (United States)

    Carrion, Diego M; Álvarez-Maestro, Mario; Gómez Rivas, Juan; González-Peramato, Pilar; Cisneros Ledo, Jesús

    2017-12-01

    We present the case of a patient diagnosed with a testicular extramedullary plasmacytoma (EMP), and perform a brief review of the literature of this pathology. A 64 year-old male patient, with history of multiple myeloma successfully treated three years before, presented with left testicular swelling. Initial work-up was compatible with a testicular tumor and radical inguinal orchiectomy was performed. Histologic examination of the testis revealed extensive intertubular infiltration by CD138 and CD56 atypical plasma cells, with diffuse staining for IgA, compatible with EMP. Invasion of the testis in multiple myeloma patients as a recurrence of the disease is an extremely rare condition, as EMPs are more common in other organ systems. Initial treatment should be the same as a primary testicular tumor with radical inguinal orchiectomy, and definitive diagnosis is established in histologic analysis.

  5. microRNAs in hematopoiesis

    NARCIS (Netherlands)

    Lazare, Seka S.; Wojtowicz, Edyta E.; Bystrykh, Leonid V.; de Haan, Gerald

    2014-01-01

    miRNAs have been implicated in all stages of hematopoiesis including maintenance of self-renewal of hematopoietic stem cells (HSCs) and differentiation into mature blood cells. Regulation by miRNAs is markedly intertwined with transcription factors. In this review, we highlight miRNAs shown to be

  6. Solitary extramedullary plasmacytoma of the sinonasal region.

    Science.gov (United States)

    Hazarika, Produl; Balakrishnan, R; Singh, Rohit; Pujary, Kailesh; Aziz, Benazim

    2011-07-01

    Less than 10% of the patients with plasma cell neoplasms present with a solitary plasmacytoma. Though the nasal cavity is a common extramedullary site, the occurrence is extremely rare. Two cases of solitary extramedullary plasmacytoma of the sinonasal region are reported. The first of which is sinonasal plasmacytoma with concomitant HIV, an association that has been reported rarely in literature to date and is matter of much debate. In the second case report, we present an instance of surgical excision of the tumor using KTP 532 laser. The diagnosis was established using immunohistochemical techniques and multiple myeloma workups were negative in all cases.

  7. Right side splenorenal fusion with marked extramedullary hematopoiesis, a case report

    Directory of Open Access Journals (Sweden)

    Mahtab Rahbar

    2018-03-01

    We report a case of splenorenal fusion in a 5-year old girl with only right functional kidney who initially presented with a large renal mass which mimicked a primary renal neoplasm with no history of splenic trauma or splenectomy in past medical history. Also, the child didn't present with symptoms of hypersplenism. The child underwent right nephrectomy for a renal mass that was subsequently confirmed as right splenorenal fusion pathologically.

  8. Peritoneal carcinomatosis-like implants of extramedullary hematopoiesis. An insolite occurrence during splenectomy for myelofibrosis

    OpenAIRE

    Casaccia, Marco; Fornaro, Rosario; Frascio, Marco; Palombo, Denise; Stabilini, Cesare; Firpo, Emma; Gianetta, Ezio

    2017-01-01

    Introduction: Primary myelofibrosis (MF) is a myeloproliferative neoplasm that results in debilitating constitutional symptoms, splenomegaly, and cytopenias. In patients with symptomatic splenomegaly, splenectomy remains a viable treatment option for MF patients with medically refractory symptomatic splenomegaly that precludes the use of ruxolitinib. Case presentation: We present the clinical case of a patient who was admitted to our Department to perform a splenectomy in MF as a therapeutic ...

  9. A Case of Mediastinal Extramedullary Plasmacytoma Associated with Multiple Myeloma

    Energy Technology Data Exchange (ETDEWEB)

    Min, Ji Hye; Kim, Tae Sung; Ko, Young Hyeh; Kim, Ki Hyun [Samsung Medical Center, Sungkyunkwan University, Seoul (Korea, Republic of)

    2010-08-15

    Extramedullary plasmacytoma is a rare manifestation of multiple myeloma, and involvement of the mediastinum by extramedullary plasmacytoma is very rare. We report here on a rare case of a large mediastinal extramedullary plasmacytoma and several pleural nodules with pleural effusions in a 45-year-old male patient with multiple myeloma that involved the thoracic spine and the calvarium. The mediastinal extramedullary plasmacytoma manifested on CT as an 11 x 4.5 cm-sized, relatively homogeneous, mildly enhancing, anterior mediastinal mass with several pleural nodules, and this simulated malignant lymphoma or malignant thymic epithelial tumor.

  10. MRI of perineural extramedullary granulocytic sarcoma

    Energy Technology Data Exchange (ETDEWEB)

    Graham, A. [Rehabilitation Medicine, Hunters Moor Neurological Rehabilitation Centre, Newcastle-Upon-Tyne (United Kingdom); Hodgson, T. [Neuroradiology Dept., Royal Hallamshire Hospital, Sheffield (United Kingdom); Jacubowski, J. [Neurosurgical Dept., Royal Hallamshire Hospital, Sheffield (United Kingdom); Norfolk, D. [Haematology Department, Leeds General Infirmary, Leeds LS1 3EX (United Kingdom); Smith, C. [Pathology Dept., Royal Hallamshire Hospital, Sheffield (United Kingdom)

    2001-06-01

    Granulocytic sarcoma is an extramedullary solid tumour consisting of myelogenous leukaemic blast cells, usually seen in acute myeloid leukaemia and less commonly in patients with chronic myeloid leukaemia or myeloproliferative disorders. Blast cells have a predilection for periosteal and perineural regions and rarely precede evidence of systemic disease. We present two patients, aleukaemic on peripheral blood counts, both at presentation and during subsequent treatment. We present the MRI features of this rare but important condition. (orig.)

  11. Intrahepatic cholestasis in pregnancy

    Directory of Open Access Journals (Sweden)

    Savić Ž.

    2014-01-01

    Full Text Available Abnormal liver function tests occur in 3-5% of pregnancies, with many potential causes, including coincidental liver disease (most commonly viral hepatitis or gallstones and underlying chronic liver disease. Pruritus in pregnancy is common, affecting 23% of pregnancies, of which a small proportion will have obstetric cholestasis. Intrahepatic cholestasis of pregnancy (ICP is a cholestatic disorder characterized by pruritus with onset in the second or third trimester of pregnancy, elevated serum aminotransferases and bile acid levels, and spontaneous relief of signs and symptoms within two to three weeks after delivery. ICP is observed in 0.4-1% of pregnancies in most areas of Central and Western Europe and North America. Genetic and hormonal factors, but also environmental factors may contribute to the pathogenesis of ICP. Intrahepatic cholestasis of pregnancy increases the risk of preterm delivery (19­60%, meconium staining of amniotic fluid (27%, fetal bradycardia (14%, fetal distress (22-41%, and fetal loss (0.4-4.1%, particularly when associated with fasting serum bile acid levels >40 μmol/L. Important ICP-induced changes in serum profiles of amidated bile acids were observed, involving both a marked increase in cholic acid concentration and a shift towards a higher proportion of taurine-conjugated species. Ursodeoxycholic acid (10-20 mg/kg/d is today regarded as the first line treatment for intrahepatic cholestasis of pregnancy. Delivery has been recommended in the 37-38th week when lung maturity has been established.

  12. Transjugular intrahepatic portosystemic shunt

    Energy Technology Data Exchange (ETDEWEB)

    Park, Jae Hyung; Han, Joon Koo; Chung, Jin Wook; Han, Man Chung [Seoul National University College of Medicidne, Seoul (Korea, Republic of)

    1992-05-15

    As a new interventional procedure for the control of variceal bleeding, a portosystemic shunt can be established with the installment of metallic stent through the transjugular approach. In order to evaluate the clinical usefulness of the procedure, transjugular intrahepatic portosystemic shunt procedure were performed in 5 patients with variceal bleeding due to liver cirrhosis. The metallic stents were mainly a self expandable Wallstent (Schneider, Switzerland), An 8 to 10 mm shunt was formed by the insertion of the stent and balloon dilatation after puncture of the proximal portal vein from the right or middle hepatic vein. The patency of the shunt was proven by portography after the procedure. The portal pressure measured in 3 patients before and after the procedure improved with decrease from 31 mmHg to 25 mmHg. The procedure failed in 1 patient due to pre-existing portal vein thrombosis. During the follow-up period from 1 month to 4 months, shunts were patent in all 4 patients. However, hepatic encephalopathy occurred in one patient one week following the procedure. Though the follow-up period was not long enough for full evaluation, we found the transjugular intrahepatic portosystemic shunt was a safe and effective procedure for the control of variceal bleeding by lowering the portal pressure. For the appropriate application for this procedure, the optimal size of the shunt and optical degree of the resultant decompression are yet to be determined in the future.

  13. Extramedullary plasmacytoma. Fine needle aspiration findings.

    Science.gov (United States)

    Kumar, P V; Owji, S M; Talei, A R; Malekhusseini, S A

    1997-01-01

    To determine the role of fine needle aspiration cytology in the diagnosis of extramedullary plasmacytoma. The study group consisted of 13 patients with palpable masses at various sites. The tumors were aspirated for cytologic study. The smears revealed groups of mature and immature plasma cells at various stages of maturation. Mature plasma cells showed an eccentric nucleus and abundant, deep, basophilic cytoplasm with a paranuclear halo. Plasmablasts (immature plasma cells) showed a prominent, eccentric nucleus with single, large nucleolus and abundant, deep, basophilic cytoplasm with no paranuclear halo. Binucleate and multinucleate forms were also seen quite often. The tumors were excised, and the histologic sections confirmed the cytologic diagnosis. All the patients received radiotherapy. One patient (18 years old) developed recurrence and died due to extensive infiltration into the maxilla and mandible. Two patients (57 and 62 years) developed multiple myeloma one to two years after the excision of tumors, and both died two to three months later. The remaining 10 patients were alive and well at this writing. The smears from all 13 patients were diagnosed as extramedullary plasmacytomas by fine needle aspiration cytology.

  14. Transjugular Intrahepatic Portosystemic Shunt (TIPS)

    Medline Plus

    Full Text Available ... bear denotes child-specific content. Related Articles and Media Radiation Dose in X-Ray and CT Exams Contrast Materials Venography Images related to Transjugular Intrahepatic Portosystemic ...

  15. Ultrasonographic findings of intrahepatic hematoma

    International Nuclear Information System (INIS)

    Moon, Jang Ho; Lim, Hyo Keum; Ham, Eun Jae; Choo, In Wook; Bae, Sang Hoon; Yoon, Jong Sup

    1989-01-01

    Sequential ultrasonography was performed in 22 cases of intrahepatic hematomas diagnosed by operation, laboratory data, and clinical manifestation. We analyzed the shape, location, size of hematoma, and change in size and echogenicity by age of hematoma. The results were as follows: 1. The most common shape of intrahepatic hematoma was round. 2. The most common site of intrahepatic hematoma was the posterior segment of the right lobe of the liver. 3. Size of hematoma was decreased from 3rd day, and most hematomas were nearly or completely absorbed from 2nd week to 4th week. 4. Echogenecities of intrahepatic hematoma in immediate ultrasonography after injury showed mainly echogenic or mixed form, and then the echogenecities were decreased and nearly or completely not seen from 2nd week to 4th week

  16. Transjugular Intrahepatic Portosystemic Shunt (TIPS)

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    Full Text Available ... bear denotes child-specific content. Related Articles and Media Radiation Dose in X-Ray and CT Exams Contrast Materials Venography Images related to Transjugular Intrahepatic Portosystemic Shunt (TIPS) Sponsored ...

  17. Transjugular Intrahepatic Portosystemic Shunt (TIPS)

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    Full Text Available ... story about radiology? Share your patient story here Images × Image Gallery Radiologist and patient consultation. View full size ... X-Ray and CT Exams Contrast Materials Venography Images related to Transjugular Intrahepatic Portosystemic Shunt (TIPS) Sponsored ...

  18. Transjugular Intrahepatic Portosystemic Shunt (TIPS)

    Medline Plus

    Full Text Available ... Intrahepatic Portosystemic Shunt (TIPS)? What are some common uses of the procedure? How should I prepare? What does the equipment look like? How does the procedure work? How is the procedure performed? What will I ...

  19. Extramedullary plasmacytoma: clinical and histopathologic study

    International Nuclear Information System (INIS)

    Strojan, Primoz; Soba, Erika; Lamovec, Janez; Munda, Anton

    2002-01-01

    Purpose: To review the histories of extramedullary plasmacytoma patients diagnosed in Slovenia between 1969 and 1999, to determine the relationship between radiotherapy (XRT) dose and local tumor control, and to clarify the role of elective nodal XRT and the prognostic value of Bartl's histologic grading criteria (originally devised for multiple myeloma [MM]). Methods and Materials: The database of the Cancer Registry of Slovenia was used for the identification of patients. The inclusion criteria were as follows: bone marrow biopsy showing less than 10% plasma cells, normal skeletal survey, and immunohistochemically determined tumor monoclonality. Simulation/portal films were reviewed to assess the extent of elective nodal XRT. Results: Twenty-six patients with 31 tumors fulfilled the inclusion criteria. In 4 patients, nine metachronously appearing solitary tumors were diagnosed. The head-and-neck region and other body sites were the sites of origin of primary tumors in 84% and 16% of patients, respectively, whereas in the two regions, regional disease was seen in 15% and 60% of patients, respectively. Therapy was as follows: XRT, 12 patients; surgery and postoperative XRT, 15 patients; and surgery, 4 patients. Ultimate local and regional control rates were 90% and 97%, respectively, and MM developed in 2 (8%) patients. The 10-year disease-specific and overall survival rates were 87% and 61%, respectively. The analysis of the dose-effect relationship showed that more conservative treatment is justified: for macroscopic disease, 40-50 Gy (2 Gy/day), adjusted to the bulk of disease; for microscopic disease, 36-40 Gy; after R0 surgery, no XRT is required, but close observation is needed. No attempts should be made to treat uninvolved nodal regions. Using Bartl's histologic grading criteria, trends were detected in patients with higher tumor grades: regional lymph node involvement (p=0.04) and shorter disease-specific survival (p=0.08). Conclusions: Extramedullary

  20. Extramedullary plasmacytoma: clinical and histopathologic study.

    Science.gov (United States)

    Strojan, Primoz; Soba, Erika; Lamovec, Janez; Munda, Anton

    2002-07-01

    To review the histories of extramedullary plasmacytoma patients diagnosed in Slovenia between 1969 and 1999, to determine the relationship between radiotherapy (XRT) dose and local tumor control, and to clarify the role of elective nodal XRT and the prognostic value of Bartl's histologic grading criteria (originally devised for multiple myeloma [MM]). The database of the Cancer Registry of Slovenia was used for the identification of patients. The inclusion criteria were as follows: bone marrow biopsy showing less than 10% plasma cells, normal skeletal survey, and immunohistochemically determined tumor monoclonality. Simulation/portal films were reviewed to assess the extent of elective nodal XRT. Twenty-six patients with 31 tumors fulfilled the inclusion criteria. In 4 patients, nine metachronously appearing solitary tumors were diagnosed. The head-and-neck region and other body sites were the sites of origin of primary tumors in 84% and 16% of patients, respectively, whereas in the two regions, regional disease was seen in 15% and 60% of patients, respectively. Therapy was as follows: XRT, 12 patients; surgery and postoperative XRT, 15 patients; and surgery, 4 patients. Ultimate local and regional control rates were 90% and 97%, respectively, and MM developed in 2 (8%) patients. The 10-year disease-specific and overall survival rates were 87% and 61%, respectively. The analysis of the dose-effect relationship showed that more conservative treatment is justified: for macroscopic disease, 40-50 Gy (2 Gy/day), adjusted to the bulk of disease; for microscopic disease, 36-40 Gy; after R0 surgery, no XRT is required, but close observation is needed. No attempts should be made to treat uninvolved nodal regions. Using Bartl's histologic grading criteria, trends were detected in patients with higher tumor grades: regional lymph node involvement (p = 0.04) and shorter disease-specific survival (p = 0.08). Extramedullary plasmacytoma is a highly curable disease when XRT is

  1. Transjugular Intrahepatic Portosystemic Shunt (TIPS)

    Medline Plus

    Full Text Available Toggle navigation Test/Treatment Patient Type Screening/Wellness Disease/Condition Safety En Español More Info Images/Videos About Us News Physician Resources Professions Site Index A-Z Transjugular Intrahepatic Portosystemic Shunt ( ...

  2. Computed tomography of intrahepatic cholangiocarcinoma

    International Nuclear Information System (INIS)

    Kamimura, Ryoichi; Takashima, Tsutomu; Matsui, Osamu; Tsuji, Masahiko; Hirose, Shoichiro.

    1983-01-01

    Intrahepatic cholangiocarcinoma is an uncommon tumor as primary hepatic neoplasm. Five cases of cholangiocarcinoma, mass forming peripheral type, are reported about its CT findings. They were manifested as a poorly marginated low density mass with a irregular stellate area. In one case, a cut section of the gross specimen following surgery showed a central callagenous scar and vessels within the necrotic tumor. (author)

  3. Stochasticity and determinism in models of hematopoiesis.

    Science.gov (United States)

    Kimmel, Marek

    2014-01-01

    This chapter represents a novel view of modeling in hematopoiesis, synthesizing both deterministic and stochastic approaches. Whereas the stochastic models work in situations where chance dominates, for example when the number of cells is small, or under random mutations, the deterministic models are more important for large-scale, normal hematopoiesis. New types of models are on the horizon. These models attempt to account for distributed environments such as hematopoietic niches and their impact on dynamics. Mixed effects of such structures and chance events are largely unknown and constitute both a challenge and promise for modeling. Our discussion is presented under the separate headings of deterministic and stochastic modeling; however, the connections between both are frequently mentioned. Four case studies are included to elucidate important examples. We also include a primer of deterministic and stochastic dynamics for the reader's use.

  4. Extramedullary plasmacytoma of the larynx. A report of three cases

    International Nuclear Information System (INIS)

    Strojan, P.

    2002-01-01

    Purpose. To report three cases of extramedullary plasmacytoma of the larynx treated at the Institute of Oncology in Ljubljana between 1969-1999. Results. All three patients were treated with radiotherapy only, which resulted in permanent local and regional control of 7.8, 4.7 and 3.5 years. The function of the larynx was preserved in all of them. Two patients died, both to the causes other than plasmacytoma. In none of the patients disease progressed to multiple myeloma. Conclusions. Extramedullary plasmacytoma of the larynx is a rare disease, highly curable when radiotherapy is used. Moderate radiation doses and limited fields ensure excellent cosmetic and functional result. (author)

  5. Extramedullary plasmacytomas in the context of multiple myeloma.

    Science.gov (United States)

    Aguado, Beatriz; Iñigo, Belen; Sastre, Jose L; Oriol, Albert

    2011-11-01

    Plasmacytoma is a frequent complication of multiple myeloma, either at diagnosis or within disease progression. The extramedullary disease confers a poorer prognosis and is biologically distinct with high-risk molecular and histological features, being resistant to conventional treatments. Radiation therapy remains the most effective treatment for extramedullary lesions to achieve local control. There are very limited data from randomized trials regarding the most appropriate systemic treatment. Case reports such as those presented here, as well as retrospective analysis of series, suggest that lenalidomide is an effective agent, in combination with dexamethasone, in this setting. Additional studies are needed to define the proper management of this condition.

  6. Extramedullary plasmacytoma in the trachea of a dog.

    Science.gov (United States)

    Chaffin, K; Cross, A R; Allen, S W; Mahaffey, E A; Watson, S K

    1998-05-15

    A 10-year-old spayed female mixed-breed dog was examined because of acute inspiratory dyspnea. Radiography and tracheoscopy revealed a discrete, solitary mass originating from the membranous portion of the trachea at the level of the thoracic inlet. Tracheal resection and anastomosis were performed, and on histologic examination of the resected tissue, extramedullary plasmacytoma was diagnosed. Although tracheal tumors are rare in dogs, they should be considered during evaluation of dogs with signs of airway obstruction. Prognosis is excellent for dogs with extramedullary plasmacytoma in which surgical excision is complete.

  7. Adrenal extramedullary hematopoiesis associated with beta-thalassemia trait in an adult woman: a case report and review of literature

    Directory of Open Access Journals (Sweden)

    Hassan Al-Thani

    2016-01-01

    Conclusions: EMH could be presented with several clinical hematological disorders. Surgical management becomes inevitable in certain adrenal EMH cases especially in the presence of a large adrenal mass.

  8. Clonal hematopoiesis in acquired aplastic anemia

    OpenAIRE

    Ogawa, Seishi

    2016-01-01

    Clonal hematopoiesis (CH) in aplastic anemia (AA) has been closely linked to the evolution of late clonal disorders, including paroxysmal nocturnal hemoglobinuria and myelodysplastic syndromes (MDS)/acute myeloid leukemia (AML), which are common complications after successful immunosuppressive therapy (IST). With the advent of high-throughput sequencing of recent years, the molecular aspect of CH in AA has been clarified by comprehensive detection of somatic mutations that drive clonal evolut...

  9. Dissection of vertebrate hematopoiesis using zebrafish thrombopoietin

    Czech Academy of Sciences Publication Activity Database

    Svoboda, Ondřej; Stachura, D.L.; Machoňová, Olga; Pajer, Petr; Brynda, Jiří; Zon, L.I.; Traver, D.; Bartůněk, Petr

    2014-01-01

    Roč. 124, č. 2 (2014), s. 220-228 ISSN 0006-4971 R&D Projects: GA ČR GAP305/10/0953 Grant - others:NIH(US) K01-DK087814-01A1; NIH(US) R01-DK074482 Keywords : Zebrafish * hematopoiesis * progenitors * thrombopoietin * erythropoietin Subject RIV: EB - Genetics ; Molecular Biology Impact factor: 10.452, year: 2014

  10. Familial cholestasis: progressive familial intrahepatic cholestasis, benign recurrent intrahepatic cholestasis and intrahepatic cholestasis of pregnancy

    NARCIS (Netherlands)

    van der Woerd, Wendy L.; van Mil, Saskia W. C.; Stapelbroek, Janneke M.; Klomp, Leo W. J.; van de Graaf, Stan F. J.; Houwen, Roderick H. J.

    2010-01-01

    Progressive familial intrahepatic cholestasis (PFIC) type 1, 2 and 3 are due to mutations in ATP8B1, ABCB11 and ABCB4, respectively. Each of these genes encodes a hepatocanalicular transporter, which is essential for the proper formation of bile. Mutations in ABCB4 can result in progressive

  11. [Symptomatic extramedullary haematopoiesis in β-thalassemia: A retrospective single centre study].

    Science.gov (United States)

    Maazoun, F; Gellen Dautremer, J; Boutekadjirt, A; Pissard, S; Habibi, A; Bachir, D; Rahmouni, A; Bartolucci, P; Debbache, K; Lagrange, J-L; Michel, M; Galacteros, F

    2016-01-01

    Symptomatic extramedullary hematopoiesis (EH) is a rare but potentially severe phenomenon which occurs in β-thalassemia. There are no treatment guidelines. Retrospective single centre study including the cases of symptomatic EH encountered between 1997 and 2014 in a unit specialised in red blood cell genetic disorders. Description of clinical, biological and radiological characteristics of the patients, treatments received, and outcomes. Among 182 β-thalassemia patients followed during the study period, 7 cases of symptomatic EH were diagnosed. They were 5 men and 2 women, and their mean age was 37 years. Four patients were splenectomised, two patients were regularly transfused, and four patients had already received erythropoietin. EH was localised in intravertebral areas and responsible for dorsal spinal cord compression in 5 patients, in paravertebral dorsal area in 1 patient, and in presacral area in 1 patient. The mean hemoglobin level at diagnosis was 7.9 g/dL. Treatment administered included: red cell transfusion in 6 cases, associated with hydroxyurea in 5 cases and/or radiotherapy in 3 patients. One patient was treated with surgery and HU. After a median follow-up of 41 months, clinical recovery was complete in 2 patients and partial in 5 patients. EH must be suspected in β-thalassemia in patients presenting clinical signs of organ compression, and a typical radiological aspect. The functional prognosis depends on the rapidity of treatment, which includes red blood cell transfusion, hydroxyurea, radiotherapy, and rarely surgery. Long-term outcome is uncertain. Copyright © 2015. Published by Elsevier SAS.

  12. Extramedullary leukemia in children with acute myeloid leukemia

    DEFF Research Database (Denmark)

    Støve, Heidi Kristine; Sandahl, Julie Damgaard; Abrahamsson, Jonas

    2017-01-01

    BACKGROUND: The prognostic significance of extramedullary leukemia (EML) in childhood acute myeloid leukemia is not clarified. PROCEDURE: This population-based study included 315 children from the NOPHO-AML 2004 trial. RESULTS: At diagnosis, 73 (23%) patients had EML: 39 (12%) had myeloid sarcoma...... the OS. No patients relapsed at the primary site of the myeloid sarcoma despite management without radiotherapy....

  13. Solitary extramedullary plasmacytoma of the colon, rectum and anus ...

    African Journals Online (AJOL)

    Solitary extramedullary plasmacytoma (SEP) is a neoplastic proliferation of a single clone of plasma cells that occur outside of the bone and bone marrow. It is rare, commonly occurring in the head and neck region, followed by the gastrointestinal tract. The aetiology, risk factors, natural history and consequent treatment are ...

  14. Intrahepatic splenosis: a case report

    International Nuclear Information System (INIS)

    Pekkafali, Zekai; Karsli, Fevzi A.; Silit, Emir; Basekim, Cinar C.; Mutlu, Hakan; Kizilkaya, Esref; Narin, Yavuz

    2002-01-01

    Splenosis is heterotopic autotransplantation and seeding of splenic tissue. In the literature, only a few cases of splenosis involving the liver and the radiologic characteristics of these lesions have been reported. We report a case of intrahepatic splenosis diagnosed by ultrasound, computed tomography, magnetic resonance imaging and scintigraphic features. To our knowledge, our patient is the first case diagnosed only by radiologic and radionuclide examination without any intervention. (orig.)

  15. Compartmental architecture and dynamics of hematopoiesis.

    Directory of Open Access Journals (Sweden)

    David Dingli

    Full Text Available BACKGROUND: Blood cell formation is maintained by the replication of hematopoietic stem cells (HSC that continuously feed downstream "compartments" where amplification and differentiation of cells occurs, giving rise to all blood lineages. Whereas HSC replicate slowly, committed cells replicate faster as they become more differentiated. METHODOLOGY/SIGNIFICANT FINDING: We propose a multi-compartment model of hematopoiesis, designed on the principle of cell flow conservation under stationary conditions. Cells lost from one compartment due to differentiation are replaced by cells from the upstream compartment. We assume that there is a constant relationship between cell input and output in each compartment and fix the single parameter of the model using data available for granulocyte maturation. We predict that approximately 31 mitotic events separate the HSC from the mature cells observed in the circulation. Besides estimating the number of compartments, our model allows us to estimate the size of each compartment, the rate of cell replication within each compartment, the mean time a given cell type contributes to hematopoiesis, the amplification rate in each compartment, as well as the mean time separating stem-cell replication and mature blood-cell formation. CONCLUSIONS: Despite its simplicity, the model agrees with the limited in vivo data available and can make testable predictions. In particular, our prediction of the average lifetime of a PIG-A mutated clone agrees closely with the experimental results available for the PIG-A gene mutation in healthy adults. The present elucidation of the compartment structure and dynamics of hematopoiesis may prove insightful in further understanding a variety of hematopoietic disorders.

  16. Radiotherapy for extramedullary leukaemic manifestation (Chloroma)

    Energy Technology Data Exchange (ETDEWEB)

    Oertel, Michael; Elsayad, Khaled; Haverkamp, Uwe; Eich, Hans Theodor [University Hospital of Muenster, Department of Radiation Oncology, Muenster (Germany); Stelljes, Matthias [University Hospital of Muenster, Department of Internal Medicine, Muenster (Germany)

    2018-02-15

    Extramedullary leukaemic disease (EMD, synonym chloroma) is a rare solid manifestation of myeloid leukaemia for which the value of radiotherapy (RT) as a treatment strategy remains controversial. The aim of this study is to analyse the effectiveness of various RT doses for EMD in the modern treatment era. Between January 2000 and June 2016, 20 patients with total of 45 lesions underwent RT for EMD at our institution. With a median radiation dose of 26 Gy (range 4-42 Gy), local remission could be achieved in 91% of patients (complete remission rate: 71%). The median duration of local control (DOLC) was 17 months (95% confidence interval [CI] 0.5-33) and the median overall survival (OS) after chloroma onset was 24 months (95% CI 11-38). No noticeable difference between high- and low-dose regimens has been observed (74% versus 68%; P = 0.5). In the multivariate analysis, only Eastern Cooperative Oncology Group (ECOG) score and bone marrow state during RT have proven to be determinant for durable local control and OS. Low-dose RT (≤26 Gy) achieves good local control compared to high-dose regimes. Bone marrow state during RT and ECOG score during RT may play a crucial role, influencing both DOLC and OS. (orig.) [German] Extramedullaere leukaemische Infiltrate (EMD) sind seltene Manifestationen myeloischer Leukaemien, in deren Behandlungskonzepten der Stellenwert der Radiotherapie (RT) unklar ist. Das Ziel der vorgelegten Studie ist es, die Wirksamkeit verschiedener Strahlentherapiedosen fuer EMD in der modernen Behandlungsaera zu untersuchen. Zwischen Januar 2000 und Juni 2016 durchliefen 20 Patienten mit insgesamt 45 Laesionen eine RT fuer EMD in unserer Klinik. Mit einer mittleren RT-Dosis von 26 Gy (Spanne 4-42 Gy) konnte eine lokale Remission bei 91 % der Patienten erzielt werden (komplette Remissionsrate, CRR: 71 %). Die mittlere Dauer der Lokalkontrolle (DOLC) betrug 17 Monate (95 %-KI 0,5-33) und das mediane Gesamtueberleben (OS) nach Chloromadiagnose war 24

  17. Extramedullary intradural spinal tumors; Extramedullaere intradurale spinale Tumoren

    Energy Technology Data Exchange (ETDEWEB)

    Papanagiotou, P. [Universitaetsklinikum des Saarlandes, Klinik fuer Diagnostische und Interventionelle Neuroradiologie, Homburg/Saar (Germany)

    2011-12-15

    The category of extramedullary intradural tumors includes a variety of lesions ranging from meningiomas originating from meningeal cells and nerve sheath tumors (neurofibromas, schwannomas) to less common primary tumors, such as lipomas, ependymomas, hemangiopericytomas, epidermoid cysts and dermoid cysts. Extramedullary metastases can occur as transcoelomic metastases in tumors of the central nervous system (CNS) or metastasization from other tumors. Magnetic resonance imaging (MRI) is the method of choice for localization and characterization of these lesions before treatment. (orig.) [German] Die Kategorie der extramedullaeren intraduralen Tumoren enthaelt Laesionen, die von den Nervenhuellen (Schwannome und Neurofibrome) oder von den meningealen Zellen ausgehen (Meningeome). Ependymome, Lipome, Haemangioperizytome, Epidermoidzysten und Dermoidzysten entsprechen selteneren primaeren Tumoren. Extramedullaere Metastasen koennen als Abtropfmetastasen bei ZNS-Tumoren oder als Metastasierung anderer Karzinomerkrankungen auftreten. Die Magnetresonanztomographie (MRT) ist die Methode der Wahl zur Abklaerung einer intraduralen Raumforderung. (orig.)

  18. Primary extramedullary plasmacytoma of the penis: a case report.

    Science.gov (United States)

    Wang, Yao; Li, Hong-Yan; Liang, Ting-Ting; Han, Yu-Ping; Wang, Xue-Ju; Wei, Xin; Fan, Li; Wang, Wei-Hua

    2013-10-01

    Extramedullary plasmacytoma involving the penis is extremely rare. Here, we describe a case of primary extramedullary plasmacytoma of the penis in a 64-year-old man who presented with a palpable penile mass. Nuclear magnetic resonance imaging revealed the presence of a large, round non-encapsulated mass in the perineum. A contrast-enhanced computed tomography scan of the pelvis showed that the mass was located in the tunica albuginea and corpora cavernosa at the base of the penis. The mass encased the urethra and demonstrated no marked enhancement during the arterial phase. The patient underwent successful surgical resection of the tumor. Histologically, the tumor was composed primarily of neoplastic plasma cells that were positive for CD38, vimentin and Ki 67. Postoperatively, the patient recovered well and exhibited no evidence of development of multiple myeloma, local recurrence or distant metastasis at 2 months post-surgery. To the best of our knowledge, our case represents the first documented case of human primary extramedullary plasmacytoma of the penis.

  19. Intrahepatic splenosis mimicking hepatic neoplasia

    Directory of Open Access Journals (Sweden)

    Gabriel Neves Saad Teles

    Full Text Available Introduction: Splenosis is defined as the heterotopic autoimplantation of splenic tissue following trauma to or surgery on the spleen. Clinical case: We present a case of an asymptomatic 73-year-old male in whom hypervascular lesions were detected during routine exams. The patient reported a history of carotid artery surgery and cholecystectomy; he had a laparotomy incision from childhood but was unaware of the reason for it. The patient exhibited slightly elevated carcinoembryonic antigen (CEA levels. Histopathology revealed intrahepatic heterotopic splenic parenchyma, with no evidence of neoplasia in either of the two lesions, the diameters of which were 1.5 cm and 3.6 cm. Patient received outpatient follow-up care for 24 months and experienced no complications. Discussion: Our clinical, laboratory, and imaging exams failed to reveal the etiology of the lesion. Because the masses were hypervascular lesions, a percutaneous liver biopsy was not feasible. Conclusion: Through this report, we emphasize the importance of considering intrahepatic splenosis as a remote possibility in patients with hepatic nodules who have a history of splenectomy. Keywords: Splenosis, Neoplasia, Liver, Splenectomy

  20. Spinal cord compression due to epidural extramedullary haematopoiesis in thalassaemia: MRI

    International Nuclear Information System (INIS)

    Aydingoez, Ue.; Oto, A.; Cila, A.

    1997-01-01

    Spinal epidural extramedullary haematopoiesis is very rare in thalassaemia. A 27-year-old man with thalassaemia intermedia presented with symptoms and signs of spinal cord compression. MRI showed a thoracic spinal epidural mass, representing extramedullary haematopoietic tissue, compressing the spinal cord. Following radiotherapy, serial MRI revealed regression of the epidural mass and gradual resolution of spinal cord oedema. (orig.)

  1. Intrahepatic cholestasis with parental alimentation.

    Science.gov (United States)

    Rodgers, B M; Hollenbeck, J I; Donnelly, W H; Talbert, J L

    1976-02-01

    From July 1971 to March 1975, elevan infants receiving total or partial parenteral alimentation at the University of Florida showed histologic evidence of intrahepatic cholestasis. The clinical records of these patients have been examined. These infants were critically ill and had protracted hospital courses with only two survivors. Liver biopsies demonstrated marked cholestasis with some fibrosis and thickening of the limiting membrane of the hepatocyte. In those patients in whom serial liver biopsies were obtained, hepatic histology returned toward normal, paralleling improvement in liver function studies, as intravenous alimentation was discontinued. Careful monitoring of the liver function tests is essential to detect this progressive abnormality as early as possible and discontinue intravenous alimentation. Follow-up as long as two and a half years in the two surviving patients has demonstrated no chronic dysfunction.

  2. Molecular profiling of intrahepatic cholangiocarcinoma

    DEFF Research Database (Denmark)

    Oliveira, Douglas V N P; Zhang, Shanshan; Chen, Xin

    2017-01-01

    . Areas covered: The present review article outlines the main studies and resulting discoveries on the molecular profiling of iCCA, with a special emphasis on the different techniques used for this purpose, the diagnostic and prognostic markers identified, as well as the genes and pathways that could......INTRODUCTION: Intrahepatic cholangiocarcinoma (iCCA) is the second most frequent primary tumor of the liver and a highly lethal disease. Therapeutic options for advanced iCCA are limited and ineffective due to the largely incomplete understanding of the molecular pathogenesis of this deadly tumor...... be potentially targeted with innovative therapies. Expert commentary: Molecular profiling has led to the identification of distinct iCCA subtypes, characterized by peculiar genetic alterations and transcriptomic features. Targeted therapies against some of the identified genes are ongoing and hold great promise...

  3. Four stages of hepatic hematopoiesis in human embryos and fetuses.

    Science.gov (United States)

    Fanni, D; Angotzi, F; Lai, F; Gerosa, C; Senes, G; Fanos, V; Faa, G

    2018-03-01

    The liver is a major hematopoietic organ during embryonic and fetal development in humans. Its hematopoietic activity starts during the first weeks of gestation and continues until birth. During this period the liver is colonized by undifferentiated hematopoietic stem cells (HSCs) that gradually differentiate and once mature, enter the circulatory system through the hepatic sinusoids, this process is called hepatic hematopoiesis. The morphology of hepatic hematopoiesis, has been studied in humans through the years, and led to a characterization of all the cell types that make up these phenomena. Studies on murine models also helped to describe the extent of hepatic hematopoiesis at different gestational ages. Using this knowledge, we attempted to describe how hepatic hematopoiesis morphologically evolves as gestation progresses, in human embryos and fetuses. Thus, we observed a total of 32 tissue specimens obtained from the livers of embryos and fetuses at different gestational ages. Basing our observations on the four stages of liver hematopoiesis identified by Sasaki and Sonoda in mice, we also described four consecutive stages of liver hematopoiesis in humans, which resulted to be highly similar to those described in murine models.

  4. Triple manifestation of extramedullary plasmacytoma in the upper airway: an unusual clinical entity.

    LENUS (Irish Health Repository)

    Morariu, I

    2012-02-01

    OBJECTIVE: We report an extremely rare case of extramedullary plasmacytoma. METHOD: Case report and review of the English-literature concerning extramedullary plasmacytoma and multiple myeloma. RESULT: We present an unusual case of multiple extramedullary plasmacytomas, which, over a protracted course of 30 years, presented on different occasions at three separate sites in the head and neck. The patient was managed surgically on all occasions, and was disease-free at the time of writing. CONCLUSION: Following review of the literature, we believe this to be the only case with this extremely unusual presentation. This case is noteworthy, not only because of the rarity of extramedullary plasmacytoma, but also because it highlights a number of important clinical issues. The diagnosis and management of extramedullary plasmacytoma require close cooperation between multiple disciplines.

  5. Radiotherapy of extramedullary plasmacytoma of the head and neck

    International Nuclear Information System (INIS)

    Harwood, A.R.; Knowling, M.A.; Bergsagel, D.E.

    1981-01-01

    The purpose of this study is to report the results of megavoltage irradiation with cobalt-60 in 23 previously unreported cases of extramedullary plasmacytoma of the head and neck. It has been found that 3500 cGy (rad) in three weeks provides good local control of disease with minimal morbidity and a significant proportion do not go on to multiple myeloma. Prognostic factors of significance with respect to subsequent development of multiple myeloma include site and presence or absence of bone destruction. The presence or absence of an M protein peak appears to be of no significance. (author)

  6. Hematopoiesis-activating effects of androgen (fluoxymesterone)

    International Nuclear Information System (INIS)

    Shirakura, Takuo; Inoue, Tatsu; Maekawa, Tadashi.

    1978-01-01

    Hematopoiesis-activating effect of fluoxymesterone (FMT) (androgen preparation) on hematopoietic hematoblast was evaluated. The results were as follows. (1) To C 3 H/He strain male mice, 2 mg/day of FMT was injected into each femoral muscle alternatively. On the 5th day of the first injection, radioactive iron was injected intraperitoneally; after 48 hrs, blood was collected for examining the incorporation rate of 59 Fe. The results showed significant increase. (2) After 4 mg of FMT was injected once into the femoral muscle, spleen colony-forming cells (CFU-S) of the mouse femur were examined. Calculation of either of surface colonies and microscopic colonies showed absolute increase of the number of CFU-S in the femoral marrow. The microscopic colonies were classified into three colony types of erythrocytes, granulocytes, and megakaryocytes on the basis of histology of the cellular component; and changes were evaluated in each type of the colonies. This evaluation clarified that an increase in colonies which appeared 24 hrs after the injection of the hormon was due to an increase in the erythrocyte and megakaryocyte colonies and that which appeared 48 hrs after the injection was due to an increase in granulocyte colonies. (Ueda, J.)

  7. Hematopoiesis and hematopoietic organs in arthropods.

    Science.gov (United States)

    Grigorian, Melina; Hartenstein, Volker

    2013-03-01

    Hemocytes (blood cells) are motile cells that move throughout the extracellular space and that exist in all clades of the animal kingdom. Hemocytes play an important role in shaping the extracellular environment and in the immune response. Developmentally, hemocytes are closely related to the epithelial cells lining the vascular system (endothelia) and the body cavity (mesothelia). In vertebrates and insects, common progenitors, called hemangioblasts, give rise to the endothelia and blood cells. In the adult animal, many differentiated hemocytes seem to retain the ability to proliferate; however, in most cases investigated closely, the bulk of hemocyte proliferation takes place in specialized hematopoietic organs. Hematopoietic organs provide an environment where undifferentiated blood stem cells are able to self-renew, and at the same time generate offspring that differentiate into different blood cell types. Hematopoiesis in vertebrates, taking place in the bone marrow, has been subject to intensive research by immunologists and stem cell biologists. Much less is known about blood cell formation in invertebrate animals. In this review, we will survey structural and functional properties of invertebrate hematopoietic organs, with a main focus on insects and other arthropod taxa. We will then discuss similarities, at the molecular and structural level, that are apparent when comparing the development of blood cells in hematopoietic organs of vertebrates and arthropods. Our comparative review is intended to elucidate aspects of the biology of blood stem cells that are more easily missed when focusing on one or a few model species.

  8. Clonal hematopoiesis in acquired aplastic anemia.

    Science.gov (United States)

    Ogawa, Seishi

    2016-07-21

    Clonal hematopoiesis (CH) in aplastic anemia (AA) has been closely linked to the evolution of late clonal disorders, including paroxysmal nocturnal hemoglobinuria and myelodysplastic syndromes (MDS)/acute myeloid leukemia (AML), which are common complications after successful immunosuppressive therapy (IST). With the advent of high-throughput sequencing of recent years, the molecular aspect of CH in AA has been clarified by comprehensive detection of somatic mutations that drive clonal evolution. Genetic abnormalities are found in ∼50% of patients with AA and, except for PIGA mutations and copy-neutral loss-of-heterozygosity, or uniparental disomy (UPD) in 6p (6pUPD), are most frequently represented by mutations involving genes commonly mutated in myeloid malignancies, including DNMT3A, ASXL1, and BCOR/BCORL1 Mutations exhibit distinct chronological profiles and clinical impacts. BCOR/BCORL1 and PIGA mutations tend to disappear or show stable clone size and predict a better response to IST and a significantly better clinical outcome compared with mutations in DNMT3A, ASXL1, and other genes, which are likely to increase their clone size, are associated with a faster progression to MDS/AML, and predict an unfavorable survival. High frequency of 6pUPD and overrepresentation of PIGA and BCOR/BCORL1 mutations are unique to AA, suggesting the role of autoimmunity in clonal selection. By contrast, DNMT3A and ASXL1 mutations, also commonly seen in CH in the general population, indicate a close link to CH in the aged bone marrow, in terms of the mechanism for selection. Detection and close monitoring of somatic mutations/evolution may help with prediction and diagnosis of clonal evolution of MDS/AML and better management of patients with AA. © 2016 by The American Society of Hematology.

  9. Myelomatous ascites as an initial manifestation of extramedullary involvement of multiple myeloma

    Energy Technology Data Exchange (ETDEWEB)

    Choi, Seo Youn; Lee, Hae Kyung; Yi, Boem Ha; Lee, Min Hee; Kim, Hee Kyung; Park, Seong Kyu [Soonchunhyang University Bucheon Hospital, Bucheon (Korea, Republic of)

    2017-03-15

    Multiple myeloma is a common hematological malignancy. Aggressive myeloma invades the organs outside the bone marrow, lymph, or reticuloendothelial systems. Among the extramedullary involvements of multiple myeloma, myelomatous ascites are extremely rare and are associated with a poor prognosis. We describe a case of myelomatous ascites as an initial manifestation of extramedullary involvement of multiple myeloma in 39-year-old patient. The patient was treated with high-dose chemotherapy, but extensive extramedullary involvement progressed, and the patient expired approximately five months after the initial detection of ascites.

  10. Extramedullary plasmacytoma of small bowel mesentery in associated with cecal cancer: a case report

    International Nuclear Information System (INIS)

    Kim, Sung Kyu; Kim, Yong Soo; Kim, Young Sun; Cho, On Koo; Koh, Byung Hee; Rhim, Hyun Chul; Park, Choog Ki; Park, Dong Woo; Park, Yong Wook; Oh, Young Ha

    2005-01-01

    Extramedullary plasmacytoma is a rare disease that is histopathologically defined as a solitary tumor composed of a monoclonal proliferation of cells with plasmacytic differentiation in an extramedullary site. Most of these tumors occur in the submucosa of the upper aerodigestive tract, and they rarely occur in the small bowel mesentery. We report here on a case of extramedullary plasmacytoma of the small bowel mesentery that was in association with a cecal cancer. Abdominal ultrasound and CT revealed a lobulated soft tissue mass with a cystic portion and peripheral calcification. In this case, the preoperative radiological diagnosis was difficult due to accompanying cecal cancer

  11. Extramedullary plasmacytoma of small bowel mesentery in associated with cecal cancer: a case report

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Sung Kyu; Kim, Yong Soo; Kim, Young Sun; Cho, On Koo; Koh, Byung Hee; Rhim, Hyun Chul; Park, Choog Ki; Park, Dong Woo [Hanyang University College of Medicine, Seoul (Korea, Republic of); Park, Yong Wook; Oh, Young Ha [Hanyang University Guri Hospital, Guri (Korea, Republic of)

    2005-07-15

    Extramedullary plasmacytoma is a rare disease that is histopathologically defined as a solitary tumor composed of a monoclonal proliferation of cells with plasmacytic differentiation in an extramedullary site. Most of these tumors occur in the submucosa of the upper aerodigestive tract, and they rarely occur in the small bowel mesentery. We report here on a case of extramedullary plasmacytoma of the small bowel mesentery that was in association with a cecal cancer. Abdominal ultrasound and CT revealed a lobulated soft tissue mass with a cystic portion and peripheral calcification. In this case, the preoperative radiological diagnosis was difficult due to accompanying cecal cancer.

  12. Localized extramedullary relapse after autologous hematopoietic stem cell transplantation in multiple myeloma

    International Nuclear Information System (INIS)

    Erkus, Muhan; Meteoglu, Ibrahim; Bolaman, Zahit; Kadikoylu, Gurhan

    2005-01-01

    Extramedullary plasmacytomas are rare manifestation of plasma cell malignancies. After hematopoietic stem cell transplantation HSCT, presentation of localized plasmacytoma with extramedullary growth is very unusual. We report a case of a 56-year-old woman with Dune-Salmon stage IIIA immunoglobulin A-kappa multiple myeloma, which presented 120 days after autologous HSCT with extramedullary plasmacytoma arising from a lymph node in supraclavicular region. The patient had no pretransplant-history related with extramedullary disease. There was no increase of plasma cells in bone marrow or monoclonal protein in urine or serum. Aspiration smears of lymph node revealed a population of plasmacytoid cells at various stages of maturation. The patient was successfully treated with local radiotherapy and has remained progression-free for more than 20 months. (author)

  13. Solitary extramedullary plasmacytomas of thyroid in Hashimoto's thyroiditis: Mimicking benign cystic nodule on ultrasonography

    International Nuclear Information System (INIS)

    Kwon, Yohan; Kim, Soo Jin; Hur, Joon Ho; Park, Sung Hee; Lee, Sun Jin; Lee, Tae Jin

    2013-01-01

    Solitary extramedullary plasmacytoma (SEP) of the thyroid is uncommon and mostly occur in patients with a Hashimoto's thyroiditis (82%). We present a case on SEP of thyroid in Hashimoto's thyroiditis, which mimics growing benign cystic masses on serial ultrasonography.

  14. Dorsal approaches to intradural extramedullary tumors of the craniovertebral junction

    Directory of Open Access Journals (Sweden)

    D Refai

    2010-01-01

    Full Text Available Tumors of the craniovertebral junction (CVJ pose significant challenges to cranial and spine surgeons. Familiarity with the complex anatomy and avoidance of injury to neurologic and vascular structures are essential to success. Multiple surgical approaches to address lesions at the CVJ have been promoted, including ventral and dorsal-based trajectories. However, optimal selection of the surgical vector to manage the pathology requires a firm understanding of the limitations and advantages of each approach. The selection of the best surgical trajectory must include several factors, such as obtaining the optimal exposure of the region of interest, avoiding injury to critical neurologic or vascular structures, identification of normal anatomical landmarks, the familiarity and comfort level of the surgeon to the approach, and the need for fixation. This review article focuses on dorsal approaches to the CVJ and the advantages and limitations in managing intradural extramedullary tumors.

  15. Extramedullary plasmacytoma in the carotid space: Expanding the differential diagnosis

    International Nuclear Information System (INIS)

    Deshpande, Sneha Satish; Kane, Shubhada; Arya, Supreeta

    2014-01-01

    Plasma cell neoplasms have been classified into various types, with a range of clinical and radiological presentations. Extramedullary plasmacytoma (EMP) is a subset of plasma cell neoplasms which presents as an isolated non-osseous soft tissue mass. Though carotid space neoplasms are commonly encountered, EMP in the carotid space is rare and seldom considered in the initial differential diagnosis of a carotid space mass. These tumors can be treated by surgery or radiotherapy. On the other hand, the commonly encountered tumors in the carotid space are treated surgically. Also, it is mandatory to exclude multiple myeloma in the patients presenting with EMP. Hence, accurate and early diagnosis has therapeutic and prognostic implications. We report a rare case of EMP of the carotid space, describing the imaging features and the differential diagnoses with clues pointing to this rare entity

  16. Extramedullary spinal teratoma presenting with recurrent aseptic meningitis.

    Science.gov (United States)

    Mpayo, Lucy L; Liu, Xiao-Hong; Xu, Man; Wang, Kai; Wang, Jiao; Yang, Li

    2014-06-01

    Spinal teratomas are extremely rare; they constitute meningitis. A 7-year-old boy presented with paroxysmal abdominal pain and a history of recurrent aseptic meningitis. Kernig and Brudzinski signs were present. Lumber puncture revealed pleocytosis with no evidence of bacteria growth. Imaging of the spine revealed a cystic lesion in spinal cord at thoracic level 9-11. Endoscopic excision of the cyst was successfully performed. Surgical and histopathological findings confirmed extramedullary matured teratoma. As the symptomatic attacks of spontaneous rupture of spinal teratoma resemble presentations of Mollaret meningitis, spinal teratoma should be considered in the differential diagnosis of Mollaret meningitis. We describe a rare example of spinal teratoma causing recurrent meningitis. Spine imaging should be considered in individuals with recurrent aseptic meningitis as this promotes earlier diagnosis, more appropriate treatment, and improved neurological outcome. Copyright © 2014 Elsevier Inc. All rights reserved.

  17. A case report of craniovertebral junction intradural extramedullary neurenteric cyst

    Directory of Open Access Journals (Sweden)

    Rajeshwari S Vhora

    2014-01-01

    Full Text Available A neurenteric cyst of the craniocervical (CV junction, as a cause of bulbomedullary compression, is very rare. An abnormal communication between the endoderm and neuroectoderm during the third week of embryogenesis may be responsible for its formation. It is a rare spinal condition. The most frequent location is at the lower cervical and higher thoracic spine. Neurenteric cysts of the craniocervical junction are even rarer. We report the case of a CV junction intradural neurenteric cyst. Magnetic Resonance Imaging (MRI of our patient demonstrated an intradural extramedullary process of the craniocervical junction. A surgical posterior approach allowed gross total resection of the lesion. The histopathology of the surgical specimen showed that the cyst wall was made up of fibrocollagen walls lined with a partially ciliated columnar epithelium.

  18. Extramedullary versus intramedullary tibial cutting guides in megaprosthetic total knee replacement

    Directory of Open Access Journals (Sweden)

    Karade Vikas

    2012-10-01

    Full Text Available Abstract Background In a standard total knee replacement, tibial component alignment is a key factor for the long term success of the surgery. The purpose of this study is to compare the accuracy of extramedullary and intramedullary tibial cutting guides used in indigenous and imported implants respectively, in positioning of the tibial components in megaprosthetic knee replacements. Methods A comparative study of the accuracy of extramedullary and intramedullary tibial cutting guides was carried out in 92 megaprosthetic knee replacements for distal femoral tumors. For the proximal tibia cut for tibial component placement, an extramedullary guide was used in 65 patients and an intramedullary guide was used in 27 patients. Tibial component alignment angles were measured in postoperative X-rays with the help of CAD software. Results There was more varus placement in coronal plane with extramedullary cutting guide (−1.18 +/− 2.4 degrees than the intramedullary guide (−0.34 +/− 2.31 degrees but this did not reach statistical significance. The goal of 90 +/− 2 degrees alignment of tibial component was achieved in 54% of patients in the extramedullary group versus 67% in the intramedullary group. In terms of sagittal plane alignment, extramedullary guide showed less accurate results (2.09 +/− 2.4 degrees than intramedullary guide (0.50 +/− 3.80 degrees for tibial component alignment, though 78% of patients were aligned within the goal of 0–5 degrees of tibial slope angle in extramedullary group versus 63% in intramedullary group. The mean error in the measurements due to rotation of the knee during taking the X-rays was less than 0.1 degrees and distribution of the X-rays with the rotation of knee was similar in both the groups. Conclusions Overall, in megaprosthetic knee replacement intramedullary guides gave more accurate results in sagittal plane and exhibited similar variability as of extramedullary guides in coronal plane.

  19. Spinal cord compression due to epidural extramedullary haematopoiesis in thalassaemia: MRI

    Energy Technology Data Exchange (ETDEWEB)

    Aydingoez, Ue.; Oto, A.; Cila, A. [Department of Radiology, Hacettepe University School of Medicine, Ankara (Turkey)

    1997-12-01

    Spinal epidural extramedullary haematopoiesis is very rare in thalassaemia. A 27-year-old man with thalassaemia intermedia presented with symptoms and signs of spinal cord compression. MRI showed a thoracic spinal epidural mass, representing extramedullary haematopoietic tissue, compressing the spinal cord. Following radiotherapy, serial MRI revealed regression of the epidural mass and gradual resolution of spinal cord oedema. (orig.) With 3 figs., 6 refs.

  20. Intrahepatic biliary tract adenocarcinoma. Review of literature

    International Nuclear Information System (INIS)

    Encalada, Edmundo; Engracia, Ruth; Calle, Carlos; Rivera, Tania; Marengo, Carlos

    2002-01-01

    A seven years old patient, with a biliary tract tumoration, diagnosed by computerized tomography and eco, which had practice an exploratory laparotomy, finding an intrahepatic tumor at the left hepatic tract level, with a pathological diagnosis of papillary adenocarcinoma moderately differentiated the biliary tract. The surgery is the main treatment, auxiliary treatments with chemotherapy and radiotherapy. (The author)

  1. The role of adenosine receptor agonists in regulation of hematopoiesis

    Czech Academy of Sciences Publication Activity Database

    Hofer, Michal; Pospíšil, Milan; Weiterová, Lenka; Hoferová, Zuzana

    2011-01-01

    Roč. 16, č. 1 (2011), s. 675-685 ISSN 1420-3049 R&D Projects: GA MO OVBIOFYZ20101; GA ČR(CZ) GA305/08/0158 Institutional research plan: CEZ:AV0Z50040507; CEZ:AV0Z50040702 Keywords : adenosine receptors * hematopoiesis * myelosuppression Subject RIV: BO - Biophysics Impact factor: 2.386, year: 2011

  2. Computed tomographic findings of intrahepatic peripheral cholangiocarcinoma

    International Nuclear Information System (INIS)

    Woo, Seong Ku; Suh, Soo Jhi; Kim, Ho Joon; Chun, Byung Hee

    1986-01-01

    Cholangiocarcinoma is synonymous with bile duct carcinoma, and can originate in a small intrahepatic bile duct (peripheral type), a major intrahepatic duct including the hepatic hills, an extrahepatic duct, or near the papilla of Vater (central type). In a sense bile duct carcinoma of the peripheral type is cholangiocarcinoma of the liver; it has the same gross configuration as hepatocellular carcinoma, resulting in difficulty to differentiate on the CT. The authors studied CT findings of 14 cases of pathologically proven peripheral type cholangiocarcinoma of the liver during the last 4 years. The results were as follows: 1. Of 14 cases, 8 were female and 6 were male, and the age ranged from 5th to 7th decades. 2. Preoperative clinical diagnosis were as follows: hepatoma 8 cases, abscess 5 cases and metastasis 1 case in order of frequency. 3. Diagnosis were confirmed by hepatic lobectomy in 7 cases, wedge resection in 5 cases and needle biopsy in 2 case. 4. Laboratory findings were not specific, but there were only 2 cases with elevated alpha-fetoprotein level. 5. Associated diseases were gallstones in 1 case, intrahepatic duct stones in 1 case, extrahepatic duct stones in 2 cases, acute or chronic cholecystitis in 5 cases and CS in 3 cases. 6. Angiographic and scintigraphic findings were helpful in differential diagnosis from hepatoma but ultrasonography was non-specific. 7. The number of tumor were solitary in 12 cases and multiple in 2 cases. Among solitary cases, the site of involvement of the liver were right lobe in 8 cases and left lobe in 4 cases. 8. Common CT features of the intrahepatic peripheral cholangiocinoma of the liver were irregular, inhomogeneous, occasionally peripherally enhancing, low density liver mass, frequently accompanied by diffuse or segmental dilatation of the intrahepatic bile duct. If there were normal alpha fetoprotein level, positive skin and/or stool examination for CS and diffuse or segmental dilatation of the intrahepatic duct

  3. Extracorporeal Shockwave Lithotripsy of Primary Intrahepatic Stones

    Science.gov (United States)

    Kim, Myung Hwan; Lee, Sung Koo; Min, Young Il; Lee, Mun Gyu; Sung, Kyu Bo; Cho, Kyung Sik; Lee, Sung Gyu; Min, Pyung Chul

    1992-01-01

    Extracorporeal shockwave lithothripsy (ESWL) was performed in intrahepatic stone patients (n = 18) by Dornier MPL 9,000 with ultrasound guidance. The patients had T-tube (n = 9) or percutaneous transhepatic biliary drainge tube (n = 9). Average treatment session was four and shock-wave numbers were in the range of 3,064 to 12,000 (average 6,288 shocks). Intrahepatic stones were removed completely in 16 patients over a 3 month period by ESWL and combined stone extraction maneuver such as cholangioscopic or interventional radiologic method. Extracorporeal shockwave lithothripsy was very helpful in facilitating extraction of stones in unfavorable locations or located above the severe stricture. In summary, extracorporeal Shockwave lithotripsy, followed by percutaneous stone extraction, will provide an improvement in the success rate and duration of treatment required for complete removal of primary hepatolithiasis. PMID:1477027

  4. Computed tomography of hepatocellular carcinoma. Dilatation of intrahepatic bile duct

    Energy Technology Data Exchange (ETDEWEB)

    Choi, Soomi; Nakamura, Hitonobu; Tanaka, Ken; Hori, Shinichi; Tokunaga, Kou [Osaka Univ. (Japan). Faculty of Medicine

    1983-10-01

    Based on a series of CT of the liver in 125 patients with hepatoma and 45 patients with metastatic hepatic tumors, the mode of dilatation of the intrahepatic bile duct was examined. In patients with hepatoma, partia dilatations of intrahepatic bile duct were more commonly seen than general dilatations. On the other hand, there was no case of partial dilatation of the intrahepatic bile duct in patients with metastatic hepatic tumors. It could be concluded that partial dilatation of the intrahepatic bile duct is an useful CT finding to make a diagnosis of hepatoma, particularly to differentiate hepatoma from metastatic hepatic tumor.

  5. Intrahepatic chemoembolization in unresectable pediatric liver malignancies

    International Nuclear Information System (INIS)

    Arcement, C.M.; Towbin, R.B.; Meza, M.P.; Kaye, R.D.; Carr, B.I.; Gerber, D.A.; Mazariegos, G.V.; Reyes, J.

    2000-01-01

    Objective. To determine the effectiveness of a new miltidisciplinary approach using neoadjuvant intrahepatic chemoembolization (IHCE) and liver transplant (OLTx) in patients with unresectable hepatic tumors who have failed systemic chemotherapy. Materials and methods. From November 1989 to April 1998, 14 children (2-15 years old) were treated with 50 courses of intra-arterial chemotherapy. Baseline and post-treatment contrast-enhanced CT and alpha-fetoprotein levels were performed. Seven had hepatoblastoma, and 7 had hepatocellular carcinoma (1 fibrolamellar variant). All patients had subselective hepatic angiography and infusion of cisplatin and/or adriamycin (36 courses were followed by gelfoam embolization). The procedure was repeated every 3-4 weeks based on hepatic function and patency of the hepatic artery. Results. Six of 14 children received orthotopic liver transplants (31 courses of IHC). Pretransplant, 3 of 6 showed a significant decrease in alpha-fetoprotein, while only 1 demonstrated a significant further reduction in tumor size. Three of 6 patients are disease free at this time. Three of 6 patients died of metastatic tumor 6, 38, and 58 months, respectively post-transplant. One of 14 is currently undergoing treatment, has demonstrated a positive response, and is awaiting OLTx. Three of 14 withdrew from the program and died. Four of 14 patients developed an increase in tumor size, developed metastatic disease, and were not transplant candidates. Two hepatic arteries thrombosed, and one child had a small sealed-off gastric ulcer as complications of intrahepatic chemoembolization. Conclusion. The results of intrahepatic chemoembolization are promising and suggest that some children who do not respond to systemic therapy can be eventually cured by a combination of intrahepatic chemoembolization orthotopic liver transplant. Alpha-fetoprotein and cross-sectional imaging appear to be complementary in evaluating tumor response. IHCE does not appear to convert

  6. Intrahepatic chemoembolization in unresectable pediatric liver malignancies

    Energy Technology Data Exchange (ETDEWEB)

    Arcement, C.M.; Towbin, R.B.; Meza, M.P.; Kaye, R.D.; Carr, B.I. [Department of Radiology, Children' s Hospital of Pittsburgh, PA (United States); Gerber, D.A.; Mazariegos, G.V.; Reyes, J. [Department of Transplant Surgery, Children' s Hospital of Pittsburgh, PA (United States)

    2000-11-01

    Objective. To determine the effectiveness of a new miltidisciplinary approach using neoadjuvant intrahepatic chemoembolization (IHCE) and liver transplant (OLTx) in patients with unresectable hepatic tumors who have failed systemic chemotherapy. Materials and methods. From November 1989 to April 1998, 14 children (2-15 years old) were treated with 50 courses of intra-arterial chemotherapy. Baseline and post-treatment contrast-enhanced CT and alpha-fetoprotein levels were performed. Seven had hepatoblastoma, and 7 had hepatocellular carcinoma (1 fibrolamellar variant). All patients had subselective hepatic angiography and infusion of cisplatin and/or adriamycin (36 courses were followed by gelfoam embolization). The procedure was repeated every 3-4 weeks based on hepatic function and patency of the hepatic artery. Results. Six of 14 children received orthotopic liver transplants (31 courses of IHC). Pretransplant, 3 of 6 showed a significant decrease in alpha-fetoprotein, while only 1 demonstrated a significant further reduction in tumor size. Three of 6 patients are disease free at this time. Three of 6 patients died of metastatic tumor 6, 38, and 58 months, respectively post-transplant. One of 14 is currently undergoing treatment, has demonstrated a positive response, and is awaiting OLTx. Three of 14 withdrew from the program and died. Four of 14 patients developed an increase in tumor size, developed metastatic disease, and were not transplant candidates. Two hepatic arteries thrombosed, and one child had a small sealed-off gastric ulcer as complications of intrahepatic chemoembolization. Conclusion. The results of intrahepatic chemoembolization are promising and suggest that some children who do not respond to systemic therapy can be eventually cured by a combination of intrahepatic chemoembolization orthotopic liver transplant. Alpha-fetoprotein and cross-sectional imaging appear to be complementary in evaluating tumor response. IHCE does not appear to convert

  7. The transjugular intrahepatic portosystemic shunt (TIPS)

    International Nuclear Information System (INIS)

    Owen, A.R.; Stanley, A.J.; Vijayananthan, A.; Moss, J.G.

    2009-01-01

    The creation of an intrahepatic portosystemic shunt via a transjugular approach (TIPS) is an interventional radiological procedure used to treat the complications of portal hypertension. TIPS insertion is principally indicated to prevent or arrest variceal bleeding when medical or endoscopic treatments fail, and in the management refractory ascites. This review discusses the development and execution of the technique, with focus on its clinical efficacy. Patient selection, imaging surveillance, revision techniques, and complications are also discussed.

  8. The transjugular intrahepatic portosystemic shunt (TIPS)

    Energy Technology Data Exchange (ETDEWEB)

    Owen, A.R. [Department of Radiology, Austin Health, Heidelberg, Melbourne (Australia)], E-mail: andrewowen@doctors.org.uk; Stanley, A.J. [Department of Gastroenterology, Glasgow Royal Infirmary, Glasgow (United Kingdom); Vijayananthan, A. [Department of Biomedical Imaging, University of Malaya, Kuala Lumpur (Malaysia); Moss, J.G. [Department of Radiology, Gartnavel General Hospital, Glasgow (United Kingdom)

    2009-07-15

    The creation of an intrahepatic portosystemic shunt via a transjugular approach (TIPS) is an interventional radiological procedure used to treat the complications of portal hypertension. TIPS insertion is principally indicated to prevent or arrest variceal bleeding when medical or endoscopic treatments fail, and in the management refractory ascites. This review discusses the development and execution of the technique, with focus on its clinical efficacy. Patient selection, imaging surveillance, revision techniques, and complications are also discussed.

  9. Radiotherapy for Extramedullary Plasmacytoma of the Head and Neck

    International Nuclear Information System (INIS)

    Creach, Kimberly M.; Foote, Robert L.; Neben-Wittich, Michelle A.; Kyle, Robert A.

    2009-01-01

    Purpose: To define the effectiveness of radiotherapy in the treatment of patients with extramedullary plasmacytoma of the head and neck (EMPHN). Methods and Materials: We searched the Mayo Clinic Rochester Department of Radiation Oncology electronic Tumor Registry and identified 18 consecutive patients with a diagnosis of solitary EMPHN. Sixteen patients were treated with radiotherapy at initial diagnosis and 2 received salvage radiotherapy for local failure after surgery. Median dose administered was 50.4 Gy. Median follow-up was 6.8 years. Results: One patient (6%) developed a marginal recurrence 12 months after treatment. Six patients (33%) developed multiple myeloma (2 patients) or plasmacytomas at distant sites (4 patients) at a median of 3.1 years after diagnosis (range, 0.02 to 9.6 years). Median and 5- and 10-year overall survival rates from the date of diagnosis are 12.5 years, 88%, and 55%, respectively. Two patients (11%) developed a radiation-induced malignancy at 6.5 and 6.9 years after treatment. Conclusions: Radiotherapy provides excellent local and regional tumor control and survival in patients with EMPHN. To the best of our knowledge, this is the first report of presumed radiation-induced malignancy in this patient population

  10. Definitive radiotherapy for extramedullary plasmacytomas of the head and neck.

    Science.gov (United States)

    Michalaki, V J; Hall, J; Henk, J M; Nutting, C M; Harrington, K J

    2003-10-01

    Extramedullary plasmacytoma of the head and neck region (EMPHN) is an uncommon malignant plasma cell neoplasm. In this study we conducted a retrospective analysis of our experience of EMPHN with particular emphasis on the role of definitive radiotherapy. From 1982 to 2001, 10 patients (6 males, 4 females) with EMPHN were treated in our institution. Of nine patients treated at initial diagnosis, all received definitive radiotherapy. One patient treated at relapse underwent surgical resection followed by post-operative radiotherapy. The median age at diagnosis was 55 years (range 35-84 years). The disease was most frequently localized in the paranasal sinuses (50%). All nine patients who received definitive radiotherapy at a dose of 40-50 Gy achieved a complete response. The median follow up period was 29 months (range 7-67 months). Four patients (40%) relapsed, three have died of their disease. Two patients (20%) with paranasal sinus disease subsequently relapsed with multiple myeloma at 10 months and 24 months, respectively. Our results indicate that treatment of EMPHN with radiotherapy achieves excellent rates of local control. The relapse rate in neck nodes of 10% does not justify elective irradiation of the uninvolved neck.

  11. Hepatic Rupture Induced by Spontaneous Intrahepatic Hematoma

    Directory of Open Access Journals (Sweden)

    Jin-bao Zhou

    2018-01-01

    Full Text Available The etiology of hepatic rupture is usually secondary to trauma, and hepatic rupture induced by spontaneous intrahepatic hematoma is clinically rare. We describe here a 61-year-old female patient who was transferred to our hospital with hepatic rupture induced by spontaneous intrahepatic hematoma. The patient had no history of trauma and had a history of systemic lupus erythematosus for five years, taking a daily dose of 5 mg prednisone for treatment. The patients experienced durative blunt acute right upper abdominal pain one day after satiation, which aggravated in two hours, accompanied by dizziness and sweating. Preoperative diagnosis was rupture of the liver mass. Laparotomy revealed 2500 mL fluid consisting of a mixture of blood and clot in the peritoneal cavity. A 3.5 cm × 2.5 cm rupture was discovered on the hepatic caudate lobe near the vena cava with active arterial bleeding, and a 5  × 6 cm hematoma was reached on the right posterior lobe of the liver. Abdominal computed tomography (CT and laparotomy revealed spontaneous rupture of intrahepatic hematoma with hemorrhagic shock. The patient was successfully managed by suturing the rupture of the hepatic caudate lobe and clearing part of the hematoma. The postoperative course was uneventful, and the patient was discharged after two weeks of hospitalization.

  12. Suppression of hepatic hematopoiesis with radioactive gold (198Au)

    International Nuclear Information System (INIS)

    Turner, A.R.; Gummerman, L.W.; Boggs, D.R.

    1985-01-01

    A patient with idiopathic myelofibrosis of some 20 yr duration developed esophageal varices and ascites. No explanation for increased portal pressure other than hepatic hematopoiesis was found. Consequently, a trial of cobalt irradiation to the liver was undertaken with definite but transient decrease in ascites. Subsequently, two courses of radioactive colloidal gold were given, again with definite but transient beneficial effects on the degree of ascites. This latter benefit occurred without suppression of marrow function

  13. Influence on radiogenic alterations in hematopoiesis - Situation and possibilities

    International Nuclear Information System (INIS)

    Kehrberg, G.; Rose, H.; Saul, G.; Riessbeck, K.H.

    1985-01-01

    Radiogenic alterations of hematopoiesis are a main topic in radiobiological investigations. By further elucidation of the regulatory mechanisms possibly follow new starting points to accelerate the regeneration of stem cells, still capable of proliferating, or differentiation of most endangered and nearly irretrievable cell populations by drugs. The present state is discussed concerning the application of anabolics, endotoxins, thymic extracts, cyanoethylurea, and lithium carbonate as well as parenteral nutrition and competition of stem cells. (author)

  14. Influence on radiogenic alterations in hematopoiesis - Situation and possibilities

    Energy Technology Data Exchange (ETDEWEB)

    Kehrberg, G; Rose, H; Saul, G; Riessbeck, K H [Humboldt-Universitaet, Berlin (German Democratic Republic). Bereich Medizin (Charite)

    1985-01-01

    Radiogenic alterations of hematopoiesis are a main topic in radiobiological investigations. By further elucidation of the regulatory mechanisms possibly follow new starting points to accelerate the regeneration of stem cells, still capable of proliferating, or differentiation of most endangered and nearly irretrievable cell populations by drugs. The present state is discussed concerning the application of anabolics, endotoxins, thymic extracts, cyanoethylurea, and lithium carbonate as well as parenteral nutrition and competition of stem cells.

  15. Ex vivo tools for the clonal analysis of zebrafish hematopoiesis

    Czech Academy of Sciences Publication Activity Database

    Svoboda, Ondřej; Stachura, D.L.; Machoňová, Olga; Zon, L.I.; Traver, D.; Bartůněk, Petr

    2016-01-01

    Roč. 11, č. 5 (2016), s. 1007-1020 ISSN 1754-2189 R&D Projects: GA MŠk LO1419; GA ČR GA16-21024S Institutional support: RVO:68378050 Keywords : stem-cells * in-vitro * vertebrate hematopoiesis * transgenic zebrafish * progenitor cells * danio-rerio * fish * thrombopoietin * identification * specification Subject RIV: EB - Genetics ; Molecular Biology Impact factor: 10.032, year: 2016

  16. Single cell analysis of normal and leukemic hematopoiesis.

    Science.gov (United States)

    Povinelli, Benjamin J; Rodriguez-Meira, Alba; Mead, Adam J

    2018-02-01

    The hematopoietic system is well established as a paradigm for the study of cellular hierarchies, their disruption in disease and therapeutic use in regenerative medicine. Traditional approaches to study hematopoiesis involve purification of cell populations based on a small number of surface markers. However, such population-based analysis obscures underlying heterogeneity contained within any phenotypically defined cell population. This heterogeneity can only be resolved through single cell analysis. Recent advances in single cell techniques allow analysis of the genome, transcriptome, epigenome and proteome in single cells at an unprecedented scale. The application of these new single cell methods to investigate the hematopoietic system has led to paradigm shifts in our understanding of cellular heterogeneity in hematopoiesis and how this is disrupted in disease. In this review, we summarize how single cell techniques have been applied to the analysis of hematopoietic stem/progenitor cells in normal and malignant hematopoiesis, with a particular focus on recent advances in single-cell genomics, including how these might be utilized for clinical application. Copyright © 2017. Published by Elsevier Ltd.

  17. Extramedullary Relapse of Acute Lymphoblastic Leukemia Presenting as Abnormal Uterine Bleeding: A Case Report.

    Science.gov (United States)

    Robillard, Diana T; Kutny, Matthew A; Chewning, Joseph H; Arbuckle, Janeen L

    2017-06-01

    Acute lymphoblastic leukemia (ALL) is the most common childhood malignancy. Relapse of ALL occurs in 15%-20% of patients, with 2%-6% occurring exclusively in extramedullary sites. Relapse of ALL in gynecologic organs is extremely rare. We present a case of a 12-year-old girl with a history of ALL who was referred to the pediatric gynecology clinic with abnormal uterine bleeding. She was determined to have an extramedullary uterine relapse of her ALL. Abnormal uterine bleeding in the setting of childhood malignancy is a frequent reason for consultation to pediatric and adolescent gynecology services. This bleeding is commonly attributed to thrombocytopenia due to bone marrow suppressive chemotherapeutic agents. However, as shown in this report, abnormal uterine bleeding might be a manifestation of an extramedullary relapse. Copyright © 2017 North American Society for Pediatric and Adolescent Gynecology. Published by Elsevier Inc. All rights reserved.

  18. Synchronous infiltrating ductal carcinoma and primary extramedullary plasmacytoma of the breast

    Directory of Open Access Journals (Sweden)

    Liu Yan-Xue

    2009-04-01

    Full Text Available Abstract Background Extramedullary plasmacytomas are seldom solitary and usually progress to diffuse myelomatosis. Plasmacytomas of the breast are rare, especially when not associated multiple myeloma. Synchronous infiltrating ductal carcinoma and primary extramedullary plasmacytoma of the breast have not previously reported. Case presentation A 27-years-old woman with an untreated upper outer quadrant breast mass for 1-year was referred to our cancer hospital for surgical evaluation of increasing breast pain. Postoperatively, microscopic examination revealed an infiltrating ductal carcinoma complicated by an extramedullary plasmacytoma divided by fibrous tissue in one section. Following surgery, the patient received chemotherapy for the carcinoma and radiotherapy for the plasmacytoma. Conclusion In this case, careful histopathology examination was essential to make the correct diagnosis and therapy for these synchronous lesions. The patient finished chemotherapy and radiotherapy without significant adverse effects.

  19. Electrochemotherapy treatment of oral extramedullary plasmacytoma of the tongue: a retrospective study of three dogs

    Directory of Open Access Journals (Sweden)

    Rúbia Monteiro de Castro Cunha

    2017-11-01

    Full Text Available ABSTRACT: Extramedullary plasmacytomas (EPs are responsible for 2.5% of neoplasms in dogs. They are solitary, smooth, elevated, pink or red nodules, of 1 to 2cm in diameter. Cutaneous and oral extramedullary plasmacytomas in dogs are usually benign tumors, treated with local therapies. Prognosis is generally good. Recurrence and metastatic rates are low. Electrochemotherapy is a local treatment that combines chemotherapy and electroporation and shows objective responses of 70% to 94% with few local and systemic side effects. This scientific communication has the objective to report treatment of three canine patients with oral extramedullary plasmacytoma. Nodules were located on the tongue and patients were submitted to one or two electrochemotherapy sessions, which preserved the tongue without mutilation and cured the patients.

  20. Ginger Stimulates Hematopoiesis via Bmp Pathway in Zebrafish

    Science.gov (United States)

    Ferri-Lagneau, Karine F.; Moshal, Karni S.; Grimes, Matthew; Zahora, Braden; Lv, Lishuang; Sang, Shengmin; Leung, TinChung

    2012-01-01

    Background Anemia is a hematologic disorder with decreased number of erythrocytes. Erythropoiesis, the process by which red blood cells differentiate, are conserved in humans, mice and zebrafish. The only known agents available to treat pathological anemia are erythropoietin and its biologic derivatives. However, erythropoietin therapy elicits unwanted side-effects, high cost and intravenous or subcutaneous injection, warranting the development of a more cost effective and non-peptide alternative. Ginger (Zingiber officinale) has been widely used in traditional medicine; however, to date there is no scientific research documenting the potential of ginger to stimulate hematopoiesis. Methodology/Principal Findings Here, we utilized gata1:dsRed transgenic zebrafish embryos to investigate the effect of ginger extract on hematopoiesis in vivo and we identified its bioactive component, 10-gingerol. We confirmed that ginger and 10-gingerol promote the expression of gata1 in erythroid cells and increase the expression of hematopoietic progenitor markers cmyb and scl. We also demonstrated that ginger and 10-gingerol can promote the hematopoietic recovery from acute hemolytic anemia in zebrafish, by quantifying the number of circulating erythroid cells in the dorsal aorta using video microscopy. We found that ginger and 10-gingerol treatment during gastrulation results in an increase of bmp2b and bmp7a expression, and their downstream effectors, gata2 and eve1. At later stages ginger and 10-gingerol can induce bmp2b/7a, cmyb, scl and lmo2 expression in the caudal hematopoietic tissue area. We further confirmed that Bmp/Smad pathway mediates this hematopoiesis promoting effect of ginger by using the Bmp-activated Bmp type I receptor kinase inhibitors dorsomorphin, LND193189 and DMH1. Conclusions/Significance Our study provides a strong foundation to further evaluate the molecular mechanism of ginger and its bioactive components during hematopoiesis and to investigate their

  1. Differential diagnosis of benign intrahepatic tumours

    International Nuclear Information System (INIS)

    Koenig, R.; Herter, M.; Deutsches Krebsforschungszentrum, Heidelberg

    1983-01-01

    Differential diagnosis of benign intrahepatic tumours can be very difficult despite numerous non-invasive diagnostic approaches, as is evident from two case reports presented here. The problem appears particularly intricate if two or more masses or space-occupying growths are present at the same time, the diagnostic aspects being different. In the first case, echinococcus alveolaris occurred simultaneously with a cavernous haemangioma and a focal nodular hyperplasia (FNH). In the second case, FNH as a pendulating tumour was combined with a second focus in the superior part of the liver. These two examples are used as basis for discussing various diagnostic approaches, such as sonography, computed tomography and scintiscanning. (orig.) [de

  2. Differential diagnosis of benign intrahepatic tumours

    Energy Technology Data Exchange (ETDEWEB)

    Koenig, R.; Herter, M.

    1983-01-01

    Differential diagnosis of benign intrahepatic tumours can be very difficult despite numerous non-invasive diagnostic approaches, as is evident from two case reports presented here. The problem appears particularly intricate if two or more masses or space-occupying growths are present at the same time, the diagnostic aspects being different. In the first case, echinococcus alveolaris occurred simultaneously with a cavernous haemangioma and a focal nodular hyperplasia (FNH). In the second case, FNH as a pendulating tumour was combined with a second focus in the superior part of the liver. These two examples are used as basis for discussing various diagnostic approaches, such as sonography, computed tomography and scintiscanning.

  3. Successful radiotherapy at the rare extramedullary localization of a chemotherapy refractory relapse Burkitt-All

    International Nuclear Information System (INIS)

    Menschel, E.; Koller, E.; Bernhart, M.; Noesslinger, T.; Pfeilstoecker, M.

    2003-01-01

    The intensive therapy of the Burkitt-All achieved higher remission rates in the last years. Relapses and therapy refractory are a big medial problem. Radiotherapy in contribution with radiosensitizer used by an extramedullary large relapse of Burkitt-All is documented in this article as a successful therapy method. (boteke)

  4. Multidisciplinary management of intrahepatic cholangiocarcinoma: Current approaches.

    Science.gov (United States)

    Guro, Hanisah; Kim, Jin Won; Choi, YoungRok; Cho, Jai Young; Yoon, Yoo-Seok; Han, Ho-Seong

    2017-06-01

    Intrahepatic cholangiocarcinoma (ICC) is a common primary hepatic tumor. However, its outcomes are usually worse than those of hepatocellular carcinoma owing to its non-specific presentation and detection at an advanced stage. The most widely used serum marker, carbohydrate antigen 19-9, is non-specific. Furthermore, imaging studies rarely identify any pathognomonic features. Surgery is the only treatment option that offers a chance of long-term survival. However, the resectability rate is low owing to the high frequencies of intrahepatic metastases, peritoneal carcinomatosis, or extrahepatic metastases. Surgical treatment should be tailored according to the macroscopic classification of ICC (e.g. mass-forming, periductal infiltrating, and intraductal growth types) because it reflects the tumor's dissemination pattern. Although lymph node metastasis is a negative prognostic factor, the importance and extent of lymph node dissection is still controversial. To improve patient survival, liver transplantation is considered in some patients with unresectable ICC, especially in those with an insufficient remnant liver volume. Minimally invasive procedures, including laparoscopic and robotic liver resection, have been tested and achieved comparable outcomes to conventional surgery in preliminary studies. No randomized trials have confirmed the efficacy of adjuvant chemotherapy in ICC, and several trials have evaluated molecular-targeted agents as monotherapy or in combination with cytotoxic chemotherapy. Multidisciplinary approaches are necessary to improve the outcomes of ICC. Copyright © 2017 Elsevier Ltd. All rights reserved.

  5. Current research on progressive familial intrahepatic cholestasis

    Directory of Open Access Journals (Sweden)

    DENG Baocheng

    2015-09-01

    Full Text Available Progressive familial intrahepatic cholestasis (PFIC refers to a heterogeneous group of autosomal-recessive disorders. The estimated incidence varies between 1/50,000 and 1/100,000 births. Three types of PFIC have been identified and related to mutations in hepatocellular transport system genes involved in bile formation. PFIC-1, PFIC-2, and PFIC-3 are due to mutations in ATP8B1, ABCB11, and ABCB4 genes involved in bile secretion, respectively. Serum gamma-glutamyl transpeptidase is normal in patients with PFIC-1 and PFIC-2, while it is raised in patients with PFIC3. The main clinical manifestation of PFIC is severe intrahepatic cholestasis. PFIC usually appears in infancy or childhood and rapidly progresses to end-stage liver disease before adulthood. Diagnosis of this disease is based on clinical manifestations, liver function tests, liver ultrasonography, liver histology, and genetic testing. Ursodeoxycholic acid therapy is the initial treatment in all PFIC patients to prevent liver damage. In some PFIC1 and PFIC2 patients, biliary diversion may also relieve pruritus and slow disease progression. However, most PFIC patients are ultimately candidates for liver transplantation.

  6. The Differentiation Balance of Bone Marrow Mesenchymal Stem Cells Is Crucial to Hematopoiesis

    Directory of Open Access Journals (Sweden)

    Jiang Wu

    2018-01-01

    Full Text Available Bone marrow mesenchymal stem cells (BMSCs, the important component and regulator of bone marrow microenvironment, give rise to hematopoietic-supporting stromal cells and form hematopoietic niches for hematopoietic stem cells (HSCs. However, how BMSC differentiation affects hematopoiesis is poorly understood. In this review, we focus on the role of BMSC differentiation in hematopoiesis. We discussed the role of BMSCs and their progeny in hematopoiesis. We also examine the mechanisms that cause differentiation bias of BMSCs in stress conditions including aging, irradiation, and chemotherapy. Moreover, the differentiation balance of BMSCs is crucial to hematopoiesis. We highlight the negative effects of differentiation bias of BMSCs on hematopoietic recovery after bone marrow transplantation. Keeping the differentiation balance of BMSCs is critical for hematopoietic recovery. This review summarises current understanding about how BMSC differentiation affects hematopoiesis and its potential application in improving hematopoietic recovery after bone marrow transplantation.

  7. Intrahepatic ascariasis – Common parasite at an uncommon site

    Directory of Open Access Journals (Sweden)

    Udit Chauhan

    2016-08-01

    Full Text Available Bacterial infections of the biliary tree are common infections of the biliary system which frequently lead to life-threatening sepsis. Parasitic infections of the biliary tree like ascariasis are not uncommon. Most adult worms reside into the extrahepatic biliary system. Intrahepatic existence is not commonly described. Urgent recognition of the intrahepatic existence of this common parasite is of paramount importance in order to start timely treatment of this lifethreatening infection. Authors described a case of intrahepatic ascariasis in a young male who was diagnosed radiologically and thereafter managed with endoscopic retrograde cholangiopancreatography and antibiotics.

  8. Computed tomography of localized dilatation of the intrahepatic bile ducts

    International Nuclear Information System (INIS)

    Araki, T.; Itai, Y.; Tasaka, A.

    1981-01-01

    Twenty-nine patients showed localized dilatation of the intrahepatic bile ducts on computed tomography, usually unaccompanied by jaundice. Congenital dilatation was diagnosed when associated with a choledochal cyst, while cholangiographic contrast material was helpful in differentiating such dilatation from a simple cyst by showing its communication with the biliary tract when no choledochal cyst was present. Obstructive dilatation was associated with intrahepatic calculi in 4 cases, hepatoma in 9, cholangioma in 5, metastatic tumor in 5, and polycystic disease in 2. Cholangioma and intrahepatic calculi had a greater tendency to accompany such localized dilatation; in 2 cases, the dilatation was the only clue to the underlying disorder

  9. Effects of deuteration on hematopoiesis in the mouse

    International Nuclear Information System (INIS)

    Adams, W.H.; Adams, D.G.

    1988-01-01

    Mice ingesting 30 to 50% D 2 O (heavy water, deuterium oxide) developed a dose-dependent depression of formed peripheral blood elements in 4 to 9 days. The principal mechanism of anemia and thrombocytopenia is impaired hematopoiesis. Despite pancytopenia in the peripheral blood, bone marrow cellularity and morphology remained normal. Upon replacement of D 2 O with tap water, platelet and neutrophil concentrations returned to normal within 48 to 72 hr. In contrast, blood lymphocyte concentrations remained low for several weeks. B-lymphocytes may be more affected by deuteration than other lymphocyte subsets. In vivo reticuloendothelial cell function, as assessed by 51 Cr-labeled sheep erythrocyte clearance, was unaffected by D 2 O. Although a dose-dependent decrease in fluid intake occurred during deuteration, hematocytopenia was not a consequence of dehydration. In view of the known kinetics of D 2 O in biological systems, the rapid response of myeloid elements to deuteration must be due primarily to the solvent (nonmetabolic) isotope effect. Prolonged deuteration has proven toxic when included in regimens for treatment of neoplasia, including leukemia, in animal models. The present study shows that modulation of hematopoiesis by D 2 O is possible without invoking the toxicities associated with prolonged deuteration

  10. Endothelial Jagged-1 Is Necessary for Homeostatic and Regenerative Hematopoiesis

    Directory of Open Access Journals (Sweden)

    Michael G. Poulos

    2013-09-01

    Full Text Available The bone marrow (BM microenvironment is composed of multiple niche cells that, by producing paracrine factors, maintain and regenerate the hematopoietic stem cell (HSC pool (Morrison and Spradling, 2008. We have previously demonstrated that endothelial cells support the proper regeneration of the hematopoietic system following myeloablation (Butler et al., 2010; Hooper et al., 2009; Kobayashi et al., 2010. Here, we demonstrate that expression of the angiocrine factor Jagged-1, supplied by the BM vascular niche, regulates homeostatic and regenerative hematopoiesis through a Notch-dependent mechanism. Conditional deletion of Jagged-1 in endothelial cells (Jag1(ECKO mice results in a profound decrease in hematopoiesis and premature exhaustion of the adult HSC pool, whereas quantification and functional assays demonstrate that loss of Jagged-1 does not perturb vascular or mesenchymal compartments. Taken together, these data demonstrate that the instructive function of endothelial-specific Jagged-1 is required to support the self-renewal and regenerative capacity of HSCs in the adult BM vascular niche.

  11. Ischaemic colitis and lung infiltrates caused by extramedullary haematopoiesis in a patient with an acute erythroid leukaemia following polycythaemia vera

    NARCIS (Netherlands)

    Brada, SJL; de Wolf, JTM; Poppema, S; Vellenga, E

    A patient with 'spent' polycythaemia vera showed extensive extramedullary haematopoiesis (EMH) in non-haematopoietic tissue clinically resulting in an ischaemic colitis and respiratory symptoms due to lung infiltrates. On laboratory investigation, the EMH also included immature erythroblasts due to

  12. Clinical grading of Intrahepatic Cholestasis Pregnancy

    Directory of Open Access Journals (Sweden)

    Meena Pradhan

    2013-06-01

    Full Text Available Intrahepatic cholestasis of pregnancy (ICP is the pregnancy induced liver disorder causing intense itching of palm, sole or even whole body especially in the evening at late second and third trimester. This disease categorized into mild and severe ICP according to raised level of LFT including serum level of Bile acid and Cholic acid. ICP has less morbidity to mother but significant risk for fetus in uterus. The predisposing element to cause intense pruritus is because of high amount of bile acid in maternal serum. Toxic bile acids affect fetal cardiomyocytes that retained in fetus body causing sudden intrauterine fetal death. ICP is commonly found in winter months and mostly itching of body occurs in the evening after sunset. Fetus in uterus is unsafe if mother’s bile acid exceeds normal value. ICP is successfully treated with Ursodeoxycholic acid (UDCA, S-adenosyl methionine (SAME, Dexamethasone and vitamin K. Keywords: itching; bile acid; obstetric cholestasis; Ursodeoxycholic cholic acid.

  13. Solitary intrahepatic bile-duct cyst presenting with Jaundice

    International Nuclear Information System (INIS)

    Park, Jeong Mi; Chun, Ki Sung; Ha, Hyun Kwon; Shinn, Kyung Sub; Bahk, Yong Whee; Kim, Jun Gi

    1989-01-01

    Caroli's disease is an uncommon condition, and characterized by congenital segmental saccular dilatation of intrahepatic bile ducts. A case of Caroli's disease, manifested by only a large communicating cystic dilatation of left intrahepatic bile duct and causing extrinsic pressure over the extrahepatic bile duct, is presented. The patient was 43-year-old housewife, hospitalized because of abdominal distension and severe jaundice. To relieve jaundice and alleviate surgical intervention, percutaneous drainage of the bile-duct cyst preceded surgery

  14. Grappling with the HOX network in hematopoiesis and leukemia.

    Science.gov (United States)

    McGonigle, Glenda J; Lappin, Terence R J; Thompson, Alexander

    2008-05-01

    The mammalian HOX gene network encodes a family of proteins which act as master regulators of developmental processes such as embryogenesis and hematopoiesis. The complex arrangement, regulation and co-factor association of HOX has been an area of intense research, particularly in cancer biology, for over a decade. The concept of redeployment of embryonic regulators in the neoplastic arena has received support from many quarters. Observations of altered HOX gene expression in various solid tumours and leukemia appear to support the thesis that 'oncology recapitulates ontogeny' but the identification of critical HOX subsets and their functional role in cancer onset and maintenance requires further investigation. The application of novel techniques and model systems will continue to enhance our understanding of the HOX network in the years to come. Better understanding of the intricacy of the complex as well as identification of functional pathways and direct targets of the encoded proteins will permit harnessing of this family of genes for clinical application.

  15. miRNAs in Normal and Malignant Hematopoiesis

    Directory of Open Access Journals (Sweden)

    Ryutaro Kotaki

    2017-07-01

    Full Text Available Lineage specification is primarily regulated at the transcriptional level and lineage-specific transcription factors determine cell fates. MicroRNAs (miRNAs are 18–24 nucleotide-long non-coding RNAs that post-transcriptionally decrease the translation of target mRNAs and are essential for many cellular functions. miRNAs also regulate lineage specification during hematopoiesis. This review highlights the roles of miRNAs in B-cell development and malignancies, and discusses how miRNA expression profiles correlate with disease prognoses and phenotypes. We also discuss the potential for miRNAs as therapeutic targets and diagnostic tools for B-cell malignancies.

  16. Somatic Mutations and Clonal Hematopoiesis in Aplastic Anemia.

    Science.gov (United States)

    Yoshizato, Tetsuichi; Dumitriu, Bogdan; Hosokawa, Kohei; Makishima, Hideki; Yoshida, Kenichi; Townsley, Danielle; Sato-Otsubo, Aiko; Sato, Yusuke; Liu, Delong; Suzuki, Hiromichi; Wu, Colin O; Shiraishi, Yuichi; Clemente, Michael J; Kataoka, Keisuke; Shiozawa, Yusuke; Okuno, Yusuke; Chiba, Kenichi; Tanaka, Hiroko; Nagata, Yasunobu; Katagiri, Takamasa; Kon, Ayana; Sanada, Masashi; Scheinberg, Phillip; Miyano, Satoru; Maciejewski, Jaroslaw P; Nakao, Shinji; Young, Neal S; Ogawa, Seishi

    2015-07-02

    In patients with acquired aplastic anemia, destruction of hematopoietic cells by the immune system leads to pancytopenia. Patients have a response to immunosuppressive therapy, but myelodysplastic syndromes and acute myeloid leukemia develop in about 15% of the patients, usually many months to years after the diagnosis of aplastic anemia. We performed next-generation sequencing and array-based karyotyping using 668 blood samples obtained from 439 patients with aplastic anemia. We analyzed serial samples obtained from 82 patients. Somatic mutations in myeloid cancer candidate genes were present in one third of the patients, in a limited number of genes and at low initial variant allele frequency. Clonal hematopoiesis was detected in 47% of the patients, most frequently as acquired mutations. The prevalence of the mutations increased with age, and mutations had an age-related signature. DNMT3A-mutated and ASXL1-mutated clones tended to increase in size over time; the size of BCOR- and BCORL1-mutated and PIGA-mutated clones decreased or remained stable. Mutations in PIGA and BCOR and BCORL1 correlated with a better response to immunosuppressive therapy and longer and a higher rate of overall and progression-free survival; mutations in a subgroup of genes that included DNMT3A and ASXL1 were associated with worse outcomes. However, clonal dynamics were highly variable and might not necessarily have predicted the response to therapy and long-term survival among individual patients. Clonal hematopoiesis was prevalent in aplastic anemia. Some mutations were related to clinical outcomes. A highly biased set of mutations is evidence of Darwinian selection in the failed bone marrow environment. The pattern of somatic clones in individual patients over time was variable and frequently unpredictable. (Funded by Grant-in-Aid for Scientific Research and others.).

  17. Intramedullary versus extramedullary alignment of the tibial component in the Triathlon knee

    LENUS (Irish Health Repository)

    Cashman, James P

    2011-08-20

    Abstract Background Long term survivorship in total knee arthroplasty is significantly dependant on prosthesis alignment. Our aim was determine which alignment guide was more accurate in positioning of the tibial component in total knee arthroplasty. We also aimed to assess whether there was any difference in short term patient outcome. Method A comparison of intramedullary versus extramedullary alignment jig was performed. Radiological alignment of tibial components and patient outcomes of 103 Triathlon total knee arthroplasties were analysed. Results Use of the intramedullary was found to be significantly more accurate in determining coronal alignment (p = 0.02) while use of the extramedullary jig was found to give more accurate results in sagittal alignment (p = 0.04). There was no significant difference in WOMAC or SF-36 at six months. Conclusion Use of an intramedullary jig is preferable for positioning of the tibial component using this knee system.

  18. Extramedullary plasmocytoma of the rhino pharynx associated to intrathoracic mass - a case report

    International Nuclear Information System (INIS)

    Souza, Andrea Silveira de; Fernandes, Artur da Rocha Correa; Yamashita, Helio Kiitiro

    1996-01-01

    The authors report a case of a 56-year-old while male, who had occipital headache, libido and visual acuity (bitemporal field) reduction as the clinical picture, with progressive evolution within a four years period. The image diagnosis methods (roentgenologic studies, (MRI) demonstrated extensive mass involving the sella, clivus and sphenoid sinus, and an intrathoracic mass on the right. The final diagnosis was extramedullary plasmocytoma with rhinopharynx origin. (author)

  19. Extramedullary haematopoiesis in Thalassaemia: results of radiotherapy: a report of three patients

    International Nuclear Information System (INIS)

    Pistevou-Gompaki, K.; Paraskevopoulos, P.; Kotsa, K.; Skaragas, G.; Repanta, E.

    1996-01-01

    Extramedullary haematopoiesis is sometimes encountered in serve anaemia. Rarely, it may cause neurological symptoms, leading to spinal cord or cauda equina compression. Three patients with thalassaemia intermedia, who developed neurological complications, are described. The diagnoses were based on the clinical findings, computed tomography and magnetic resonance imaging. Small doses of radiotherapy (10-20 Gy in 5-10 fractions) relieved symptoms in all of these patients. Our experience supports the role of radiation therapy as a treatment for this complication. (Author)

  20. Correlation of magnetic resonance imaging findings of spinal intradural extramedullary schwannomas with pathologic findings

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Yeo Ju; Park, In Suh; Yoon, Seung Hwan; Choi, Suk Jin; Kim, Youn Jeong; Kang, Young Hye; Lee, Ha Young; Kim, Woo Chul; Han, Jun Gu; Cho, Soon Gu [Inha University Hospital, Incheon (Korea, Republic of)

    2015-06-15

    To evaluate the magnetic resonance imaging (MRI) findings of spinal intradural extramedullary schwannomas with pathologic correlation and to determine whether these schwannomas share the imaging features of schwannomas in the peripheral nerves. The MRIs of 17 cases of pathologically proven spinal intradural extramedullary schwannomas were reviewed retrospectively, and cystic changes, enhancement, and intratumoral hemorrhage of the tumors were evaluated. Imaging features known to be common findings of schwannoma in the peripheral nerves, such as encapsulation, the target sign, the fascicular sign, and visualization of entering or exiting nerve rootlets, were also evaluated. The histopathology of the tumors was correlated with the MRI findings. Cystic changes were detected in 14 cases by MRI and in 16 cases by pathology. The most common pattern of enhancement was a thick peripheral septal pattern (70.59%). Intratumoral hemorrhage was detected in four cases on MRI, but in all cases on pathology. Encapsulation was observed in all cases. The fascicular sign was seen in only four cases, and thickening of an exiting rootlet was visualized in one case. None of the cases showed the target sign. Spinal intradural extramedullary schwannomas were typical encapsulated cystic tumors and had few imaging features of schwannomas in the peripheral nerves.

  1. [Extramedullary fixation combined with intramedullary fixation in the surgical reduction of sagittal mandibular condylar fractures].

    Science.gov (United States)

    Chuanjun, Chen; Xiaoyang, Chen; Jing, Chen

    2016-10-01

    This study aimed to evaluate the clinical effect of extramedullary fixation combined with intramedullary fixation during the surgical reduction of sagittal mandibular condylar fractures. Twenty-four sagittal fractures of the mandibular condyle in18 patients were fixed by two appliances: intramedullary with one long-screw osteosynthesis or Kirschner wire and extramedullary with one micro-plate. The radiologically-recorded post-operative stability-associated com-plications included the screw/micro-plate loosening, micro-plate twisting, micro-plate fractures, and fragment rotation. The occluding relations, the maximalinter-incisal distances upon mouth opening, and the mandibular deflection upon mouth opening were evaluated based on follow-up clinical examination. Postoperative panoramic X-ray and CT scans showed good repositioning of the fragment, with no redislocation or rotation, no screw/plate loosening, and no plate-twisting or fracture. Clinical examination showed that all patients regained normal mandibular movements, ideal occlusion, and normal maximal inter-incisal distances upon mouth opening. Extramedullary fixation combined with intramedullary fixation is highly recommended for sagittal condylar fractures because of the anti-rotation effect of the fragment and the reasonable place-ment of the fixation appliances.

  2. Perioperative and long-term outcome of intrahepatic cholangiocarcinoma involving the hepatic hilus after curative-intent resection: comparison with peripheral intrahepatic cholangiocarcinoma and hilar cholangiocarcinoma.

    Science.gov (United States)

    Zhang, Xu-Feng; Bagante, Fabio; Chen, Qinyu; Beal, Eliza W; Lv, Yi; Weiss, Matthew; Popescu, Irinel; Marques, Hugo P; Aldrighetti, Luca; Maithel, Shishir K; Pulitano, Carlo; Bauer, Todd W; Shen, Feng; Poultsides, George A; Soubrane, Olivier; Martel, Guillaume; Koerkamp, B Groot; Guglielmi, Alfredo; Itaru, Endo; Pawlik, Timothy M

    2018-05-01

    Intrahepatic cholangiocarcinoma with hepatic hilus involvement has been either classified as intrahepatic cholangiocarcinoma or hilar cholangiocarcinoma. The present study aimed to investigate the clinicopathologic characteristics and short- and long-term outcomes after curative resection for hilar type intrahepatic cholangiocarcinoma in comparison with peripheral intrahepatic cholangiocarcinoma and hilar cholangiocarcinoma. A total of 912 patients with mass-forming peripheral intrahepatic cholangiocarcinoma, 101 patients with hilar type intrahepatic cholangiocarcinoma, and 159 patients with hilar cholangiocarcinoma undergoing curative resection from 2000 to 2015 were included from two multi-institutional databases. Clinicopathologic characteristics and short- and long-term outcomes were compared among the 3 groups. Patients with hilar type intrahepatic cholangiocarcinoma had more aggressive tumor characteristics (eg, higher frequency of vascular invasion and lymph nodes metastasis) and experienced more extensive resections in comparison with either peripheral intrahepatic cholangiocarcinoma or hilar cholangiocarcinoma patients. The odds of lymphadenectomy and R0 resection rate among patients with hilar type intrahepatic cholangiocarcinoma were comparable with hilar cholangiocarcinoma patients, but higher than peripheral intrahepatic cholangiocarcinoma patients (lymphadenectomy incidence, 85.1% vs 42.5%, P hilar type intrahepatic cholangiocarcinoma experienced a higher rate of technical-related complications compared with peripheral intrahepatic cholangiocarcinoma patients. Of note, hilar type intrahepatic cholangiocarcinoma was associated with worse disease-specific survival and recurrence-free survival after curative resection versus peripheral intrahepatic cholangiocarcinoma (median disease-specific survival, 26.0 vs 54.0 months, P hilar cholangiocarcinoma (median disease-specific survival, 26.0 vs 49.0 months, P = .003; median recurrence-free survival

  3. Daily targeting of intrahepatic tumors for radiotherapy

    International Nuclear Information System (INIS)

    Balter, James M.; Brock, Kristy K.; Litzenberg, Dale W.; McShan, Daniel L.; Lawrence, Theodore S.; Haken, Randall Ten; McGinn, Cornelius J.; Lam, Kwok L.; Dawson, Laura A.

    2002-01-01

    Introduction: A system has been developed for daily targeting of intrahepatic tumors using a combination of ventilatory immobilization, in-room diagnostic imaging, and on-line setup adjustment. By reducing geometric position uncertainty, as well as organ movement, this system permits reduction of margins and thus potentially higher treatment doses. This paper reports our initial experience treating 8 patients with focal liver tumors using this system. Methods and Materials: The system includes diagnostic X-ray tubes mounted on the wall and ceiling of a treatment room, an active matrix flat panel imager, in-room control for image acquisition and setup adjustment, and a ventilatory immobilization system via active breathing control (ABC). Eight patients participated in the study, two using an early prototype ABC unit, and the remaining six with a commercial ABC system and improved setup measurement tools. Treatment margins were reduced, and dose consequently increased because of increased confidence in target position under this protocol. After daily setup via skin marks, orthogonal radiographs were acquired at suspended ventilation. The images were aligned to the CT model using the diaphragm for inferior-superior (IS) alignment, and the skeleton for left-right (LR) and anterior-posterior (AP) alignment. Adjustments were made for positioning errors greater than a threshold (3 or 5 mm). After treatment, retrospective analysis determined the final setup accuracy, as well as the error in initial setup measurement performed before setup adjustment. Results: Two hundred sixty-two treatment fractions were delivered on eight patients, with 171 treatments requiring repositioning. Typical treatment times were 25-30 min. Patients were able to tolerate ABC throughout the course of treatment. Breath holds up to 35 s long were used for treatment. The use of on-line imaging and setup adjustment reduced setup errors (σ) from 4.0 mm (LR), 6.7 mm (IS), and 3.8 mm (AP) to 2.1 mm (LR

  4. Intrahepatic cholangiocarcinoma : gross appearance and corresponding pathologic and radiologic features

    International Nuclear Information System (INIS)

    Yoon, Kwon Ha; Kim, Chang Guhn; Lee, Moon Gyu; Ha, Hyun Kwon; Auh, Yong Ho; Lim, Jae Hoon

    1999-01-01

    To assess the clinical and pathologic features of each type of intrahepatic cholangiocarcinoma, which is divided into three types according to gross appearance, and to determine the efficacy of CT in detecting this tumor. The pathologic and CT features of 53 surgically proven cases of intrahepatic cholangio-carcinoma were reviewed. On the basis of their gross appearance, the tumors were divided into three types, as follows : mass forming (n=33), periductal infiltrating (n=6), and intraductal growth type (n=14). CT scans were analyzed for sensitivity of detection and correlation between a tumors appearance and its histopathology. The most common histopathologic feature of mass forming and periductal infiltrating type was tubular adenocarcinoma, while in the intraductal growth type, papillary adenocarcinoma (100%) was common. With regard to pattern of tumor spread, intrahepatic and lymph node metastasis were more common in the mass forming and periductal infiltrating type than in the intraductal growth type. CT findings including intrahepatic mass, ductal wall thickening or intraductal mass associated with segmental dilatation of intrahepataic bile ducts, corresponded with these morphologic types. This classification according to gross appearance is of considerable value when interpreting the pathologic features of intrahepatic cholangiocarcinoma. CT seems to be a useful modality for the detection of tumors and may be consistent with their gross morphologic findings

  5. Autosomal dominant cyclic hematopoiesis: Genetics, phenotype, and natural history

    Energy Technology Data Exchange (ETDEWEB)

    Palmer, S.E.; Stephens, K.; Dale, D.C. [Univ. of Washington, Seattle, WA (United States)

    1994-09-01

    Autosomal dominant cyclic hematopoiesis (ADCH; cyclic neutropenia) is a rare disorder manifested by transient neutropenia that recurs every three weeks. To facilitate mapping the ADCH gene by genetic linkage analysis, we studied 9 ADCH families with 42 affected individuals. Pedigrees revealed AD inheritance with no evidence for decreased penetrance. Similar intra- and interfamilial variable expression was observed, with no evidence to support heterogeneity. At least 3 families displayed apparent new mutations. Many adults developed chronic neutropenia, while offspring always cycled during childhood. Children displayed recurrent oral ulcers, gingivitis, lymphadenopathy, fever, and skin and other infections with additional symptoms. Interestingly, there were no cases of neonatal infection. Some children required multiple hospitalizations for treatment. Four males under age 18 died of Clostridium sepsis following necrotizing enterocolitis; all had affected mothers. No other deaths due to ADCH were found; most had improvement of symptoms and infections as adults. Adults experienced increased tooth loss prior to age 30 (16 out of 27 adults, with 9 edentulous). No increase in myelodysplasia, malignancy, or congenital anomalies was observed. Recombinant G-CSF treatment resulted in dramatic improvement of symptoms and infections. The results suggest that ADCH is not a benign disorder, especially in childhood, and abdominal pain requires immediate evaluation. Diagnosis of ADCH requires serial blood counts in the proband and at least one CBC in relatives to exclude similar disorders. Genetic counseling requires specific histories as well as CBCs of each family member at risk to determine status regardless of symptom history, especially to assess apparent new mutations.

  6. A Computer Navigation System Analysis of the Accuracy of the Extramedullary (Tibial Alignment Technique in Total Knee Arthroplasty (TKA

    Directory of Open Access Journals (Sweden)

    EK Chee

    2010-07-01

    Full Text Available In total knee arthroplasty, mechanical alignment guides have improved the accuracy of implant alignment, but errors are not uncommon. In the present study, an image-free computer-assisted navigation system was used to analyse the accuracy of an extramedullary (tibial alignment system, which is based on predetermined, fixed anatomical landmarks. Comparisons were made between two surgeons, with different levels of competency in order to determine if experience affected the accuracy of extramedullary tibial jig placement, in either the coronal and sagittal planes or both planes. The results showed that the accuracy of the extramedullary tibial alignment system, in the coronal plane (in up to 80-87% of cases was much better than for posterior slope, and sagittal plane. Surgeon experience was not a significant factor.

  7. Biomechanical Comparison of an Intramedullary and Extramedullary Free-Tissue Graft Reconstruction of the Acromioclavicular Joint Complex

    Science.gov (United States)

    Garg, Rishi; Javidan, Pooya; Lee, Thay Q.

    2013-01-01

    Background Several different surgical techniques have been described to address the coracoclavicular (CC) ligaments in acromioclavicular (AC) joint injuries. However, very few techniques focus on reconstructing the AC ligaments, despite its importance in providing stability. The purpose of our study was to compare the biomechanical properties of two free-tissue graft techniques that reconstruct both the AC and CC ligaments in cadaveric shoulders, one with an extramedullary AC reconstruction and the other with an intramedullary AC reconstruction. We hypothesized intramedullary AC reconstruction will provide greater anteroposterior translational stability and improved load to failure characteristics than an extramedullary technique. Methods Six matched cadaveric shoulders underwent translational testing at 10 N and 15 N in the anteroposterior and superoinferior directions, under AC joint compression loads of 10 N, 20 N, and 30 N. After the AC and CC ligaments were transected, one of the specimens was randomly assigned the intramedullary free-tissue graft reconstruction while its matched pair received the extramedullary graft reconstruction. Both reconstructed specimens then underwent repeat translational testing, followed by load to failure testing, via superior clavicle distraction, at a rate of 50 mm/min. Results Intramedullary reconstruction provided significantly greater translational stability in the anteroposterior direction than the extramedullary technique for four of six loading conditions (p < 0.05). There were no significant differences in translational stability in the superoinferior direction for any loading condition. The intramedullary reconstructed specimens demonstrated improved load to failure characteristics with the intramedullary reconstruction having a lower deformation at yield and a higher ultimate load than the extramedullary reconstruction (p < 0.05). Conclusions Intramedullary reconstruction of the AC joint provides greater stability in the

  8. Intradural extramedullary Ewing's sarcoma: A case report and review of the literature.

    Science.gov (United States)

    Paterakis, Konstantinos; Brotis, Alexandros; Tasiou, Anastasia; Kotoula, Vasiliki; Kapsalaki, Eftychia; Vlychou, Marianna

    Extra-skeletal Ewing's sarcomas are very rare lesions to the spine surgeon, with the intradural, extramedullary lesions being even rarer. Herein we present a patient with an intradural, extramedullary form of Ewing's sarcoma and review the relevant literature. The medical records, operative reports, radiographical studies and histological examinations of a single patient are retrospectively reviewed. A 31-year old male presented with back-pain, right-leg progressive paraparesis, and inability to walk. Both motor and sensory disturbances were revealed on the right leg at the clinical examination. Lumbar MRI showed two lesions. The first one was an intradural, extramedullary lesion at the L2-L3 level, while the second was smaller, located at the bottom of the dural sac. The patient underwent gross total resection of the L2-L3 lesion after a bilateral laminectomy. Histological examination was compatible with Ewing's sarcoma, and was verified by molecular analysis. No other extra-skeletal or skeletal lesion was found. A chemotherapy scheme was tailored to the patients' histological diagnosis. The patient presented with local recurrence and bone metastasis 2 years after his initial diagnosis. A second operation was performed and the follow up of the patient showed no disease progression 18 months after revision surgery. The spine surgeon should be aware of the existence of such rare entities, in order to timely fulfill the staging process and institute the proper therapy. The management of patients with extra-skeletal Ewing's sarcomas involves professionals as members of a multidisciplinary team, all of which should co-operate for the patient's optimal outcome. Copyright © 2016. Published by Elsevier Urban & Partner Sp. z o.o.

  9. Intradural extramedullary spinal cord tumours: A retrospective study of sur­gical outcomes

    Directory of Open Access Journals (Sweden)

    Md. Kamrul Ahsan

    2016-07-01

    Full Text Available Background: Intradural extramedullary spinal cord tumours (IESCT accounts for approximately two thirds of all intraspinal neoplasm and are of important clinical consideration and surgery is the essence in cases with neurological deterioration.Objective: To share our experience on the outcome of surgical excision of intradural extramedullary spinal cord tumours. Methods: Results of 60 patients surgically treated intradural extramedullary spinal tumours between Octo­ber 2003 and October 2015 at Bangabandhu Sheikh Mujib Medical University and in our private settings, Dhaka, were analyzed retrospectively. There were 32 males, 28 females with an average age of 52.4 years (13-70 years and followed up for at least a year. The preoperative symptom with duration, tumours location and intradural space occupancy and the histopathological diagnosis were analyzed. Pain was evaluated by the visual analogue scale (VAS and the neurologic function was assessed by Nurick's grade.Results: The tumours were located as, thoracic 32 (53.33%, lumbar 16 (26.67%, cervical 04 (6.67%, and junctional 08 (13.33%, CervicoThoracic-01, Thoracolumbar-07. The histopathological diagnosis included schwannoma 35 (58.33%, meningiomas 14 (23.33%, neurofbroma 4 (6.67%, arachnoid cyst and myxopapillary ependymoma 03 (05.00% each and paraganglioma 01 (01.67%. The VAS score was reduced in all cases from 8.0 ± 1.2 to 1.2 ± 0.8 (p < 0.003 and the Nurick's grade was improved in all cases from 3.0 ± 1.3 to 1.0 ± 0.0 (p < 0.005. The preoperative neurological deficit improved within 8 postoperative weeks in most cases and within 1 postoperative year in all cases. Complications included cerebrospinal fluid leakage, parasthesia, dependant bedsore 02 (3.33% each and recurrence 03 (05.00%. and further neurological deterioration 1 (01.67% case.Conclusion: lntradural extramedullary tumors detected by MRI are mostly benign and good clinical results can be obtained when treated surgi

  10. Emergence of clonal hematopoiesis in the majority of patients with acquired aplastic anemia.

    Science.gov (United States)

    Babushok, Daria V; Perdigones, Nieves; Perin, Juan C; Olson, Timothy S; Ye, Wenda; Roth, Jacquelyn J; Lind, Curt; Cattier, Carine; Li, Yimei; Hartung, Helge; Paessler, Michele E; Frank, Dale M; Xie, Hongbo M; Cross, Shanna; Cockroft, Joshua D; Podsakoff, Gregory M; Monos, Dimitrios; Biegel, Jaclyn A; Mason, Philip J; Bessler, Monica

    2015-04-01

    Acquired aplastic anemia (aAA) is a nonmalignant disease caused by autoimmune destruction of early hematopoietic cells. Clonal hematopoiesis is a late complication, seen in 20-25% of older patients. We hypothesized that clonal hematopoiesis in aAA is a more general phenomenon, which can arise early in disease, even in younger patients. To evaluate clonal hematopoiesis in aAA, we used comparative whole exome sequencing of paired bone marrow and skin samples in 22 patients. We found somatic mutations in 16 patients (72.7%) with a median disease duration of 1 year; of these, 12 (66.7%) were patients with pediatric-onset aAA. Fifty-eight mutations in 51 unique genes were found primarily in pathways of immunity and transcriptional regulation. Most frequently mutated was PIGA, with seven mutations. Only two mutations were in genes recurrently mutated in myelodysplastic syndrome. Two patients had oligoclonal loss of the HLA alleles, linking immune escape to clone emergence. Two patients had activating mutations in key signaling pathways (STAT5B (p.N642H) and CAMK2G (p.T306M)). Our results suggest that clonal hematopoiesis in aAA is common, with two mechanisms emerging-immune escape and increased proliferation. Our findings expand conceptual understanding of this nonneoplastic blood disorder. Future prospective studies of clonal hematopoiesis in aAA will be critical for understanding outcomes and for designing personalized treatment strategies. Copyright © 2015 Elsevier Inc. All rights reserved.

  11. Genomic Perturbations Reveal Distinct Regulatory Networks in Intrahepatic Cholangiocarcinoma

    DEFF Research Database (Denmark)

    Nepal, Chirag; O'Rourke, Colm J; Oliveira, Douglas Vnp

    2018-01-01

    Intrahepatic cholangiocarcinoma (iCCA) remains a highly heterogeneous malignancy that has eluded effective patient stratification to date. The extent to which such heterogeneity can be influenced by individual driver mutations remains to be evaluated. Here, we analyzed genomic (whole-exome sequen...

  12. Embolization of nonvariceal portosystemic collaterals in transjugular intrahepatic portosystemic shunts

    International Nuclear Information System (INIS)

    Bilbao, Jose Ignacio; Arias, Mercedes; Longo, Jesus Maria; Alejandre, Pedro Luis; Betes, Maria Teresa; Elizalde, Arlette Maria

    1997-01-01

    Percutaneous embolization of large portosystemic collaterals was performed in three patients following placement of a transjugular intrahepatic portosystemic shunt in order to improve hepatopetal portal flow. Improved hepatic portal perfusion was achieved in these cases, thereby theoretically reducing the risk of chronic hepatic encephalopathy

  13. An unusual case of intrahepatic portosystemic venous shunt

    African Journals Online (AJOL)

    vena cava (IVC) (most common). Intrahepatic portosystemic venous shunts are rare vascular anomalies that may be detected in asymptomatic patients, given the recent advances in radiological imaging techniques. Accurate shunt evaluation and classification can be performed with ultrasound and multi-detector computed.

  14. Incidence, clinical features, laboratory findings and outcome of patients with multiple myeloma presenting with extramedullary relapse.

    Science.gov (United States)

    Papanikolaou, Xenofon; Repousis, Panagiotis; Tzenou, Tatiana; Maltezas, Dimitris; Kotsopoulou, Maria; Megalakaki, Katerina; Angelopoulou, Maria; Dimitrakoloulou, Elektra; Koulieris, Efstathios; Bartzis, Vassiliki; Pangalis, Gerasimos; Panayotidis, Panagiotis; Kyrtsonis, Marie-Christine

    2013-07-01

    Extramedullary plasmacytomas constitute a rare and not well studied subset of multiple myeloma (MM) relapses. We report the incidence, clinical-laboratory features and outcome of patients with MM and extramedullary relapse (ExMeR). A total of 303 patients with symptomatic MM were recorded in a 13-year period in two institutions. Twenty-eight cases of ExMeR (9%) were recorded. There was an increased frequency of elevated lactate dehydrogenase (LDH) (p = 0.026), bone plasmacytomas (p = 0.001) and fractures (p = 0.002) at diagnosis, in patients with ExMeR compared to the others. ExMeR was associated with an ominous outcome, high LDH, constitutional symptoms and a statistically significant decrease of monoclonal paraprotein compared to levels at diagnosis (p = 0.009). Prior treatment with bortezomib was associated with a decreased hazard of ExMeR (p = 0.041). Overall survival (OS) was decreased in patients with ExMeR compared to the others (38 vs. 59 months, p = 0.006). Patients with MM with ExMeR have a lower OS and their clinical and laboratory features differ from those without.

  15. Hematopoietic stem cell transplantation for isolated extramedullary relapse of acute lymphoblastic leukemia in children.

    Science.gov (United States)

    Gabelli, Maria; Zecca, Marco; Messina, Chiara; Carraro, Elisa; Buldini, Barbara; Rovelli, Attilio Maria; Fagioli, Franca; Bertaina, Alice; Lanino, Edoardo; Favre, Claudio; Rabusin, Marco; Prete, Arcangelo; Ripaldi, Mimmo; Barberi, Walter; Porta, Fulvio; Caniglia, Maurizio; Santarone, Stella; D'Angelo, Paolo; Basso, Giuseppe; Locatelli, Franco

    2018-06-13

    Relapse of acute lymphoblastic leukemia (ALL) may occur in extramedullary sites, mainly central nervous system (CNS) and testis. Optimal post-remissional treatment for isolated extramedullary relapse (IEMR) is still controversial. We collected data of children treated with hematopoietic stem cell transplantation (HSCT) for ALL IEMR from 1990 to 2015 in Italy. Among 281 patients, 167 had a relapse confined to CNS, 73 to testis, 14 to mediastinum, and 27 to other organs. Ninety-seven patients underwent autologous HSCT, 79 received allogeneic HSCT from a matched family donor, 75 from a matched unrelated donor, and 30 from an HLA-haploidentical donor. The 10-year overall survival was 56% and was not influenced by gender, ALL blast immune-phenotype, age, site of relapse, duration of first remission, and type of HSCT. In multivariable analysis, the only prognostic factors were disease status at HSCT and year of transplantation. Patients transplanted in third or subsequent complete remission (CR) had a risk of death 2.3 times greater than those in CR2. Children treated after 2000 had half the risk of death than those treated before that year. Our results suggest that both autologous and allogeneic HSCT may be considered for the treatment of pediatric ALL IEMR after the achievement of CR2.

  16. Solitary extramedullary plasmacytomas of thyroid in Hashimoto's thyroiditis: Mimicking benign cystic nodule on ultrasonography

    Energy Technology Data Exchange (ETDEWEB)

    Kwon, Yohan; Kim, Soo Jin; Hur, Joon Ho; Park, Sung Hee; Lee, Sun Jin; Lee, Tae Jin [Chung-Ang University Hospital, Chung-Ang University College of Medicine, Seoul (Korea, Republic of)

    2013-11-15

    Solitary extramedullary plasmacytoma (SEP) of the thyroid is uncommon and mostly occur in patients with a Hashimoto's thyroiditis (82%). We present a case on SEP of thyroid in Hashimoto's thyroiditis, which mimics growing benign cystic masses on serial ultrasonography.

  17. Hematopoiesis in 5-Fluorouracil-Treated Adenosine A(3) Receptor Knock-Out Mice

    Czech Academy of Sciences Publication Activity Database

    Hofer, Michal; Pospíšil, Milan; Dušek, L.; Hoferová, Zuzana; Komůrková, Denisa

    2015-01-01

    Roč. 64, č. 2 (2015), s. 255-262 ISSN 0862-8408 Institutional support: RVO:68081707 Keywords : Adenosine A(3) receptor knock-out mice * Hematopoiesis * 5-fluorouracil-induced hematotoxicity Subject RIV: BO - Biophysics Impact factor: 1.643, year: 2015

  18. Inhibitory effects of homeodomain-interacting protein kinase 2 on the aorta-gonad-mapharsen hematopoiesis

    International Nuclear Information System (INIS)

    Ohtsu, Naoki; Nobuhisa, Ikuo; Mochita, Miyuki; Taga, Tetsuya

    2007-01-01

    Definitive hematopoiesis starts in the aorta-gonad-mesonephros (AGM) region of the mouse embryo. Our previous studies revealed that STAT3, a gp130 downstream transcription factor, is required for AGM hematopoiesis and that homeodomain-interacting protein kinase 2 (HIPK2) phosphorylates serine-727 of STAT3. HIPK2 is a serine/threonine kinase known to be involved in transcriptional repression and apoptosis. In the present study, we examined the role of HIPK2 in hematopoiesis in mouse embryo. HIPK2 transcripts were found in fetal hematopoietic tissues such as the mouse AGM region and fetal liver. In cultured AGM cells, HIPK2 protein was detected in adherent cells. Functional analyses of HIPK2 were carried out by introducing wild-type and mutant HIPK2 constructs into AGM cultures. Production of CD45 + hematopoietic cells was suppressed by forced expression of HIPK2 in AGM cultures. This suppression required the kinase domain and nuclear localization signals of HIPK2, but the kinase activity was dispensable. HIPK2-overexpressing AGM-derived nonadherent cells did not form cobblestone-like colonies in cultures with stromal cells. Furthermore, overexpression of HIPK2 in AGM cultures impeded the expansion of CD45 low c-Kit + cells, which exhibit the immature hematopoietic progenitor phenotype. These data indicate that HIPK2 plays a negative regulatory role in AGM hematopoiesis in the mouse embryo

  19. Efficacy of intrahepatic absolute alcohol in unrespectable hepatocellular carcinoma

    International Nuclear Information System (INIS)

    Farooqi, J.I.; Hameed, K.; Khan, I.U.; Shah, S.

    2001-01-01

    To determine efficacy of intrahepatic absolute alcohol injection in researchable hepatocellular carcinoma. A randomized, controlled, experimental and interventional clinical trial. Gastroenterology Department, PGMI, Hayatabad Medical Complex, Peshawar during the period from June, 1998 to June, 2000. Thirty patients were treated by percutaneous, intrahepatic absolute alcohol injection sin repeated sessions, 33 patients were not given or treated with alcohol to serve as control. Both the groups were comparable for age, sex and other baseline characteristics. Absolute alcohol therapy significantly improved quality of life of patients, reduced the tumor size and mortality as well as showed significantly better results regarding survival (P< 0.05) than the patients of control group. We conclude that absolute alcohol is a beneficial and safe palliative treatment measure in advanced hepatocellular carcinoma (HCC). (author)

  20. Developmental intrahepatic shunts of childhood: radiological features and management

    International Nuclear Information System (INIS)

    Paley, M.R.; Farrant, P.; Kane, P.; Karani, J.B.; Heaton, N.D.; Howard, E.R.

    1997-01-01

    The purpose of this study was to evaluate the role of radiological techniques in the diagnosis and management of developmental intrahepatic shunts. Hepatic vascular fistulae are recognised sequelae of liver trauma and intrahepatic tumours. However, there are rare developmental malformations which may present in childhood or later life and which may carry life-threatening complications. Retrospective analysis of clinical and radiological data was carried out in 24 patients. Anomalies evaluated were: (a) direct communication between hepatic artery and hepatic veins; (b) congenital hepatoportal arteriovenous malformations; and (c) congenital portocaval anastomosis with persistent flow through the ductus venosus. Although rare, the prompt recognition of these vascular anomalies allows early surgical or radiological intervention and reversal of the haemodynamic complications. (orig.)

  1. Developmental intrahepatic shunts of childhood: radiological features and management

    Energy Technology Data Exchange (ETDEWEB)

    Paley, M.R.; Farrant, P.; Kane, P.; Karani, J.B. [Department of Radiology, King`s College Hospital, Denmark Hill, London SE5 9RS (United Kingdom); Heaton, N.D.; Howard, E.R. [Department of Paediatric Hepatobiliary Surgery, King`s College Hospital Denmark Hill, London SE5 9RS (United Kingdom)

    1997-12-01

    The purpose of this study was to evaluate the role of radiological techniques in the diagnosis and management of developmental intrahepatic shunts. Hepatic vascular fistulae are recognised sequelae of liver trauma and intrahepatic tumours. However, there are rare developmental malformations which may present in childhood or later life and which may carry life-threatening complications. Retrospective analysis of clinical and radiological data was carried out in 24 patients. Anomalies evaluated were: (a) direct communication between hepatic artery and hepatic veins; (b) congenital hepatoportal arteriovenous malformations; and (c) congenital portocaval anastomosis with persistent flow through the ductus venosus. Although rare, the prompt recognition of these vascular anomalies allows early surgical or radiological intervention and reversal of the haemodynamic complications. (orig.) With 7 figs., 4 tabs., 22 refs.

  2. Prevalence of nonalcoholic steatohepatitis among patients with resectable intrahepatic cholangiocarcinoma.

    Science.gov (United States)

    Reddy, Srinevas K; Hyder, Omar; Marsh, J Wallis; Sotiropoulos, Georgios C; Paul, Andreas; Alexandrescu, Sorin; Marques, Hugo; Pulitano, Carlo; Barroso, Eduardo; Aldrighetti, Luca; Geller, David A; Sempoux, Christine; Herlea, Vlad; Popescu, Irinel; Anders, Robert; Rubbia-Brandt, Laura; Gigot, Jean-Francois; Mentha, Giles; Pawlik, Timothy M

    2013-04-01

    The objective of this report was to determine the prevalence of underlying nonalcoholic steatohepatitis in resectable intrahepatic cholangiocarcinoma. Demographics, comorbidities, clinicopathologic characteristics, surgical treatments, and outcomes from patients who underwent resection of intrahepatic cholangiocarcinoma at one of eight hepatobiliary centers between 1991 and 2011 were reviewed. Of 181 patients who underwent resection for intrahepatic cholangiocarcinoma, 31 (17.1 %) had underlying nonalcoholic steatohepatitis. Patients with nonalcoholic steatohepatitis were more likely obese (median body mass index, 30.0 vs. 26.0 kg/m(2), p < 0.001) and had higher rates of diabetes mellitus (38.7 vs. 22.0 %, p = 0.05) and the metabolic syndrome (22.6 vs. 10.0 %, p = 0.05) compared with those without nonalcoholic steatohepatitis. Presence and severity of hepatic steatosis, lobular inflammation, and hepatocyte ballooning were more common among nonalcoholic steatohepatitis patients (all p < 0.001). Macrovascular (35.5 vs. 11.3 %, p = 0.01) and any vascular (48.4 vs. 26.7 %, p = 0.02) tumor invasion were more common among patients with nonalcoholic steatohepatitis. There were no differences in recurrence-free (median, 17.0 versus 19.4 months, p = 0.42) or overall (median, 31.5 versus 36.3 months, p = 0.97) survival after surgical resection between patients with and without nonalcoholic steatohepatitis. Nonalcoholic steatohepatitis affects up to 20 % of patients with resectable intrahepatic cholangiocarcinoma.

  3. Measurement of intrahepatic pressure during radiofrequency ablation in porcine liver.

    Science.gov (United States)

    Kawamoto, Chiaki; Yamauchi, Atsushi; Baba, Yoko; Kaneko, Keiko; Yakabi, Koji

    2010-04-01

    To identify the most effective procedures to avoid increased intrahepatic pressure during radiofrequency ablation, we evaluated different ablation methods. Laparotomy was performed in 19 pigs. Intrahepatic pressure was monitored using an invasive blood pressure monitor. Radiofrequency ablation was performed as follows: single-step standard ablation; single-step at 30 W; single-step at 70 W; 4-step at 30 W; 8-step at 30 W; 8-step at 70 W; and cooled-tip. The array was fully deployed in single-step methods. In the multi-step methods, the array was gradually deployed in four or eight steps. With the cooled-tip, ablation was performed by increasing output by 10 W/min, starting at 40 W. Intrahepatic pressure was as follows: single-step standard ablation, 154.5 +/- 30.9 mmHg; single-step at 30 W, 34.2 +/- 20.0 mmHg; single-step at 70 W, 46.7 +/- 24.3 mmHg; 4-step at 30 W, 42.3 +/- 17.9 mmHg; 8-step at 30 W, 24.1 +/- 18.2 mmHg; 8-step at 70 W, 47.5 +/- 31.5 mmHg; and cooled-tip, 114.5 +/- 16.6 mmHg. The radiofrequency ablation-induced area was spherical with single-step standard ablation, 4-step at 30 W, and 8-step at 30 W. Conversely, the ablated area was irregular with single-step at 30 W, single-step at 70 W, and 8-step at 70 W. The ablation time was significantly shorter for the multi-step method than for the single-step method. Increased intrahepatic pressure could be controlled using multi-step methods. From the shapes of the ablation area, 30-W 8-step expansions appear to be most suitable for radiofrequency ablation.

  4. Fps/Fes and Fer protein-tyrosinekinases play redundant roles in regulating hematopoiesis.

    Science.gov (United States)

    Senis, Yotis A; Craig, Andrew W B; Greer, Peter A

    2003-08-01

    The highly related protein-tyrosine kinases Fps (also called Fes) and Fer are sole members of a subfamily of kinases. In this study, knock-in mice harboring kinase-inactivating mutations in both fps and fer alleles were used to assess functional redundancy between Fps and Fer kinases in regulating hematopoiesis. Mice harboring kinase-inactivating mutations in fps and fer alleles were generated previously. Compound homozygous mice were bred that lack both Fps and Fer kinase activities and progeny were analyzed for potential defects in viability and fertility. Potential differences in hematopoiesis were analyzed by lineage analysis of bone marrow cells, peripheral blood counts, and hematopoietic progenitor cell colony-forming assays. Mice devoid of both Fps and Fer kinase activities were viable and displayed reduced fertility. Circulating levels of neutrophils, erythrocytes, and platelets were elevated in compound mutant mice compared to wild-type controls, suggesting that hematopoiesis is deregulated in the absence of Fps and Fer kinases. Compound mutant mice also showed reduced overall bone marrow cellularity, and lineage analysis revealed elevated CD11b(hi)Ly-6G(lo) myeloid cells, which may reflect increased granulocyte progenitors. Although no differences in the overall number of granulocyte/monocyte colony-forming progenitors were observed, qualitative differences in myeloid colonies from compound mutant mice suggested a role for Fps and Fer kinases in regulating cell-cell adhesion or a skewing in cellularity of colonies. Mice lacking both Fps and Fer kinase activities develop normally, show reduced fertility, and display defects in hematopoiesis, thus providing evidence for functional redundancy between Fps and Fer kinases in regulating hematopoiesis.

  5. Abnormalities of intrahepatic bile ducts in extrahepatic biliary atresia.

    Science.gov (United States)

    Raweily, E A; Gibson, A A; Burt, A D

    1990-12-01

    The infantile cholangiopathies are a group of conditions associated with neonatal jaundice, which include extrahepatic biliary atresia, paucity of intra-hepatic bile ducts and disorders associated with persistence of fetal biliary structures, the so-called ductal plate malformations. Although previously regarded as distinct entities, it has recently been suggested that they may represent parts of a disease spectrum in which the principal process is one of bile duct destruction, the morphological manifestations in individual cases being influenced by the stage of intra-uterine development at which such injury occurs and by the site within the biliary system at which there is maximum damage. To further examine this concept, we have studied liver biopsy specimens from 37 neonates with extrahepatic biliary atresia, with particular reference to abnormalities of the intrahepatic bile ducts. Paucity of intrahepatic ducts, defined as a bile duct: portal tract ratio of less than 0.9, was identified in six cases (16.2%). In eight cases (21.6%) we found concentric tubular ductal structures similar to those observed in ductal plate malformations. In one case, both abnormalities could be demonstrated. Our findings support the concept that there is overlap between the various types of infantile cholangiopathy.

  6. Sonography-guided percutaneous microwave ablation of intrahepatic primary cholangiocarcinoma

    Energy Technology Data Exchange (ETDEWEB)

    Yu Mingan [Department of Interventional Ultrasound, Chinese PLA General Hospital, 28 Fuxing Road, Beijing, 100853 (China); Liang Ping, E-mail: Liangping301@hotmail.com [Department of Interventional Ultrasound, Chinese PLA General Hospital, 28 Fuxing Road, Beijing, 100853 (China); Yu Xiaoling; Cheng Zhigang; Han Zhiyu; Liu Fangyi; Yu Jie [Department of Interventional Ultrasound, Chinese PLA General Hospital, 28 Fuxing Road, Beijing, 100853 (China)

    2011-11-15

    Objective: To evaluate the efficacy and safety of sonography-guided percutaneous microwave ablation of intrahepatic primary cholangiocarcinoma. Materials and methods: From May 2006 to March 2010, 15 patients (11 men, 4 women; mean age, 57.4 years) with 24 histologically proven intrahepatic primary cholangiocarcinoma lesions (mean tumor size, 3.2 {+-} 1.9 cm; range, 1.3-9.9 cm) were treated with microwave ablation. Results: Thirty-eight sessions were performed for 24 nodules in 15 patients. The follow-up period was 4-31 months (mean, 12.8 {+-} 8.0 months). The ablation success rate, the technique effectiveness rate, and the local tumor progression rate were 91.7% (22/24), 87.5% (21/24), and 25% (6/24) respectively according to the results of follow-up. The cumulative overall 6, 12, 24 month survival rates were 78.8%, 60.0%, and 60.0%, respectively. Major complication occurred including liver abscess in two patients (13.3%) and needle seeding in one patient (6.7%). Both complications were cured satisfied with antibiotic treatment combined to catheter drainage for abscess and resection for needle seeding. The minor complications and side effects were experienced by most patients which subsided with supportive treatment. Conclusion: Microwave ablation can be used as a safe and effective technique to treat intrahepatic primary cholangiocarcinoma.

  7. Extramedullary Solitary Plasmacytoma: Demonstrating the Role of 18F-FDG PET Imaging.

    Science.gov (United States)

    Gautam, Archana; Sahu, Kamal Kant; Alamgir, Ahsan; Siddiqi, Imran; Ailawadhi, Sikander

    2017-04-01

    An Extramedullary Plasmacytoma (EMP) is characterized by a neoplastic proliferation of clonal plasma cells outside the medullary cavity. EMPs are a rare occurrence compared to other malignant plasma cell disorders and account for approximately 3-5% of plasma-cell neoplasms. Although most cases of EMP are not immediately life threatening at diagnosis, EMPs can progress to Multiple Myeloma (MM) and thus, warrant monitoring. Currently, there are no standard guidelines for when and how to monitor patients who are diagnosed with or treated for a solitary plasmacytoma. We present a case of solitary EMP who was treated adequately and definitively but developed a distinct, non-contiguous subsequent solitary EMP and was only discovered due to surveillance 18 F-Fludeoxyglucose Positron Emission Tomography ( 18 F-FDG) (PET) scan. Uniform surveillance guidelines should be developed and the potential benefits of PET and other imaging techniques as well as their cost should be considered.

  8. Fine needle aspiration cytology in feline skeletal muscle as a diagnostic tool for extramedullary plasmacytoma

    Directory of Open Access Journals (Sweden)

    D.B. Martins

    Full Text Available ABSTRACT Extramedullary noncutaneous plasmacytoma (ENP is a myeloproliferative disorder of plasma cells that rarely affects cats. This paper describes an ENP case revealed by fine needle aspiration cytology (FNAC of the mass in the skeletal muscle of an 8-month-old, male, mixed breed cat, which had a nodule in the left hind limb. The rapid immunoassay test confirmed the presence of feline leukemia virus (FeLV. The animal necropsy macroscopically showed the nodule came from the semimembranosus muscle. Histopathological examination ratified the cytological findings. Thus, this paper alerts to the existence of plasmacytoma located in the skeletal muscle of feline species. FNAC is a quick and efficient method for diagnosis of ENP.

  9. Ferrokinetic study of splenic erythropoiesis: Relationships among clinical diagnosis, myelofibrosis, splenomegaly, and extramedullary erythropoiesis

    International Nuclear Information System (INIS)

    Beguin, Y.; Fillet, G.; Bury, J.; Fairon, Y.

    1989-01-01

    Splenic erythropoiesis was demonstrated by surface counting of 59 Fe in 129 of 1,350 ferrokinetic studies performed over a 15 year period. These 129 studies were carried out in 108 patients, including 40 with chronic myelogenous leukemia (CML), 24 with agnogenic myeloid metaplasia (AMM), 18 with polycythemia vera (PV), six with a myelodysplastic syndrome, five with acute leukemia, three with prostate or breast carcinoma, two each with aplastic anemia or Hodgkin's disease, and one each with idiopathic thrombocythemia, multiple myeloma, chronic renal failure, or treated hypopituitarism. Splenomegaly was present in 83% of the studies and hepatomegaly in 72%. Grade II-III myelofibrosis was demonstrated in 62% of the cases. Hepatic erythropoiesis was present in 77% of the studies (only 38% in PV), and marrow erythropoiesis was undetectable in 33%. Total erythropoiesis was about twice normal (range 0.2 to 8 times normal) but was ineffective to varying degrees in 86% of the studies. Relationships between organomegaly, myelofibrosis, and extramedullary erythropoiesis, as well as differences among clinical disorders, are discussed. Differences observed between CML in chronic or blastic phase suggested that the erythroid cell line was involved in the proliferative process. It is concluded that splenic erythropoiesis (1) is encountered in a variety of clinical conditions; (2) is not necessarily associated with splenomegaly or myelofibrosis, even in the myeloproliferative disorders; (3) is part of a predominantly extramedullary (in the liver as well as in the spleen), expanded, and largely inefficient total erythropoiesis; and (4) can be evaluated in a semiquantitative manner by surface counting

  10. Mixed phenotype (T/B/myeloid) extramedullary blast crisis as an initial presentation of chronic myelogenous leukemia.

    Science.gov (United States)

    Qing, Xin; Qing, Annie; Ji, Ping; French, Samuel W; Mason, Holli

    2018-04-01

    Chronic myelogenous leukemia (CML) is a myeloproliferative disorder characterized by the Philadelphia (Ph) chromosome generated by the reciprocal translocation t(9,22)(q34;q11). The natural progression of the disease follows a biphasic or triphasic course. Most cases of CML are diagnosed in the chronic phase. Extramedullary blast crisis rarely occurs during the course of CML, and is extremely rare as the initial presentation of CML. Here, we report the case of a 32-year-old female with enlarged neck lymph nodes and fatigue. She was diagnosed with B-lymphoblastic leukemia/lymphoma with possible mixed phenotype (B/myeloid) by right neck lymph node biopsy at an outside hospital. However, review of her peripheral blood smear and her bone marrow aspirate and biopsy showed features consistent with CML, which was confirmed by PCR and karyotyping. An ultrasound-guided right cervical lymph node core biopsy showed a diffuse infiltrate of blasts, near totally replacing the normal lymph node tissue, admixed with some hematopoietic cells including megakaryocytes, erythroid precursors and maturing myeloid cells. By flow cytometry and immunohistochemistry, the blasts expressed CD2, cytoplasmic CD3, CD5, CD7, CD56, TdT, CD10 (weak, subset), CD19 (subset), CD79a, PAX-5 (subset), CD34, CD38, CD117 (subset), HLA-DR (subset), CD11b, CD13 (subset), CD33 (subset), and weak cytoplasmic myeloperoxidase, without co-expression of surface CD3, CD4, CD8, CD20, CD22, CD14, CD15, CD16 and CD64, consistent with blasts with mixed phenotype (T/B/myeloid). A diagnosis of extramedullary blast crisis of CML was made. Chromosomal analysis performed on the lymph node biopsy tissue revealed multiple numerical and structural abnormalities including the Ph chromosome (46-49,XX,add(1)(p34),add(3)(p25),add(5)(q13),-6,t(9;22)(q34;q11.2),+10,-15,add(17)(p11.2),+19, +der(22)t(9;22),+mar[cp8]). After completion of one cycle of combined chemotherapy plus dasatinib treatment, she was transferred to City of Hope

  11. Fifteen-year follow-up of a patient with beta thalassaemia and extramedullary haematopoietic tissue compressing the spinal cord

    Energy Technology Data Exchange (ETDEWEB)

    Niggemann, P.; Krings, T.; Thron, A. [Dept. of Neuroradiology, RWTH-Aachen Hosital (Germany); Hans, F. [Dept. of Neurosurgery, RWTH Aachen Hospital (Germany); 1

    2005-04-01

    A long-term follow-up of a patient with beta thalassaemia with intra- and extraspinal extramedullary haematopoietic tissue compressing the spinal cord is presented. Extramedullary haematopoietic nodules are a rare cause of spinal cord compression and should be included in the differential diagnosis, especially in patients from Mediterranean countries. Treatment with radiation therapy solely failed, giving rise to the need of surgical intervention. Surgical decompression of the spine and the removal of the culprit lesion compressing the spine were performed. Postinterventional radiation therapy was applied to the spine. A relapse had to be treated again by surgical means combined with postinterventional radiation therapy. A complete relief of the symptoms and control of the lesion could be obtained.

  12. Fifteen-year follow-up of a patient with beta thalassaemia and extramedullary haematopoietic tissue compressing the spinal cord

    International Nuclear Information System (INIS)

    Niggemann, P.; Krings, T.; Thron, A.; Hans, F.

    2005-01-01

    A long-term follow-up of a patient with beta thalassaemia with intra- and extraspinal extramedullary haematopoietic tissue compressing the spinal cord is presented. Extramedullary haematopoietic nodules are a rare cause of spinal cord compression and should be included in the differential diagnosis, especially in patients from Mediterranean countries. Treatment with radiation therapy solely failed, giving rise to the need of surgical intervention. Surgical decompression of the spine and the removal of the culprit lesion compressing the spine were performed. Postinterventional radiation therapy was applied to the spine. A relapse had to be treated again by surgical means combined with postinterventional radiation therapy. A complete relief of the symptoms and control of the lesion could be obtained

  13. F-18-Fluorodeoxyglucose Positron Emission Tomography/Computed Tomography Appearance of Extramedullary Hematopoesis in a Case of Primary Myelofibrosis

    Science.gov (United States)

    Mukherjee, Anirban; Bal, Chandrasekhar; Tripathi, Madhavi; Das, Chandan Jyoti; Shamim, Shamim Ahmed

    2017-01-01

    A 44-year-old female with known primary myelofibrosis presented with shortness of breath. High Resolution Computed Tomography thorax revealed large heterogeneously enhancing extraparenchymal soft tissue density mass involving bilateral lung fields. F-18-fluorodeoxyglucose (FDG) positron emission tomography/computed tomography revealed mildly FDG avid soft tissue density mass with specks of calcification involving bilateral lung fields, liver, and spleen. Subsequent histopathologic evaluation from the right lung mass was suggestive of extramedullary hematopoesis. PMID:28533647

  14. VEGF and IHH rescue definitive hematopoiesis in Gata-4 and Gata-6-deficient murine embryoid bodies.

    Science.gov (United States)

    Pierre, Monique; Yoshimoto, Momoko; Huang, Lan; Richardson, Matthew; Yoder, Mervin C

    2009-09-01

    Murine embryonic stem cells can be differentiated into embryoid bodies (EBs), which serve as an in vitro model recapitulating many aspects of embryonic yolk sac hematopoiesis. Differentiation of embryonic stem cells deficient in either Gata-4 or Gata-6 results in EBs with disrupted visceral endoderm (VE). While lack of VE has detrimental effects on hematopoiesis in vivo, it is unclear whether lack of VE affects hematopoiesis in EBs. Therefore, we compared Gata-4 null (G4N) and Gata-6 null (G6N) EBs with wild-type EBs to assess their ability to commit to hematopoietic cells. EB VE formation was examined using cell-sorting techniques and analysis visceral endoderm gene expression. Hematopoietic progenitor potential of EBs cultured under various conditions was assessed using colony-forming assays. Definitive erythroid, granulocyte-macrophage, and mixed colonies were significantly reduced in G4N and G6N EBs compared to wild-type EBs. Vascular endothelial growth factor (VEGF) expression and secretion were also reduced in both G4N and G6N EBs, consistent with VE serving as a site of VEGF production. Addition of exogenous VEGF(165), to EB cultures completely rescued definitive colony-forming cells in G4N and G6N EBs. This rescue response could be blocked by addition of soluble Flk-1 Fc to EB cultures. Similarly, addition of exogenous Indian hedgehog to EB cultures also recovers the diminishment in definitive hematopoiesis in a reversible manner. These results suggest that the absence of VE in G4N and G6N EBs does not prevent emergence of definitive progenitors from EBs. However, the decreased level of VEGF and Indian hedgehog production in VE devoid G4N and G6N EBs attenuates definitive hematopoietic progenitor cell expansion.

  15. Pecular Features of Hematopoiesis in the Liver of Mature and Immature Green Frogs (Pelophylax Esculentus Complex

    Directory of Open Access Journals (Sweden)

    Akulenko N. M.

    2016-12-01

    Full Text Available The article describes characteristic features of the hematopoiesis in mature and immature green frogs (Pelophylax esculentus complex. Quantitative differences in liver myelograms were insignificant. However, in a sample of mature animals numerous significant correlations between the number of pigment inclusions in the liver and indicators of erythropoiesis and myelopoiesis were observed. Those correlations were absent in the immature frogs. We concluded that aft er the frogs’ breeding a lack of plastic resources, in particular, hemosiderin remains up to the hibernation.

  16. Perturbed hematopoiesis in the Tc1 mouse model of Down syndrome

    OpenAIRE

    Alford, Kate A.; Slender, Amy; Vanes, Lesley; Li, Zhe; Fisher, Elizabeth M. C.; Nizetic, Dean; Orkin, Stuart H.; Roberts, Irene; Tybulewicz, Victor L. J.

    2010-01-01

    Trisomy of human chromosome 21 (Hsa21) results in Down syndrome (DS), a disorder that affects many aspects of physiology, including hematopoiesis. DS children have greatly increased rates of acute lymphoblastic leukemia and acute megakaryoblastic leukemia (AMKL); DS newborns present with transient myeloproliferative disorder (TMD), a preleukemic form of AMKL. TMD and DS-AMKL almost always carry an acquired mutation in GATA1 resulting in exclusive synthesis of a truncated protein (GATA1s), sug...

  17. Scintigraphic appearance and selective arteriographic aspects in intrahepatic cholestasis

    Energy Technology Data Exchange (ETDEWEB)

    Valleteau de Moulliac, M [Hopital Saint-Michel, 75 - Paris (France); de Tovar, G; Mornet, P; Moinard, J F [Centre Medico-Chirurgical Foch, 92 - Suresnes (France)

    1978-01-21

    Based on one case history, it is reviewed that prolonged cases of obstructive jaundice can take on deceiving appearances, and all the more so when they fall within the framework of a 'biological gap'. Extensive distension of the extra and intrahepatic bile ducts are at the basis of images with multiple, radiating hilar gaps on scintigraphy, and of 'chicken nests' and 'Swiss cheese' at hepatographic times in selective hepatic arteriography. These rather uncommon images should lead to a diagnosis of a surgical cholestatic liver.

  18. Prevention and treatment of complications after transjugular intrahepatic portosystemic shunt

    Directory of Open Access Journals (Sweden)

    XUE Hui

    2016-02-01

    Full Text Available The application of transjugular intrahepatic portosystemic shunt (TIPS in the treatment of cirrhotic portal hypertension has been widely accepted both at home and abroad. This article focuses on the fatal complications of TIPS (including intraperitoneal bleeding and acute pulmonary embolism, shunt failure, and recurrent portosystemic hepatic encephalopathy, and elaborates on the reasons for such conditions and related preventive measures, in order to improve the accuracy and safety of intraoperative puncture, reduce common complications such as shunt failure and hepatic encephalopathy, and improve the clinical effect of TIPS in the treatment of cirrhotic portal hypertension.

  19. Each cell counts: Hematopoiesis and immunity research in the era of single cell genomics.

    Science.gov (United States)

    Jaitin, Diego Adhemar; Keren-Shaul, Hadas; Elefant, Naama; Amit, Ido

    2015-02-01

    Hematopoiesis and immunity are mediated through complex interactions between multiple cell types and states. This complexity is currently addressed following a reductionist approach of characterizing cell types by a small number of cell surface molecular features and gross functions. While the introduction of global transcriptional profiling technologies enabled a more comprehensive view, heterogeneity within sampled populations remained unaddressed, obscuring the true picture of hematopoiesis and immune system function. A critical mass of technological advances in molecular biology and genomics has enabled genome-wide measurements of single cells - the fundamental unit of immunity. These new advances are expected to boost detection of less frequent cell types and fuzzy intermediate cell states, greatly expanding the resolution of current available classifications. This new era of single-cell genomics in immunology research holds great promise for further understanding of the mechanisms and circuits regulating hematopoiesis and immunity in both health and disease. In the near future, the accuracy of single-cell genomics will ultimately enable precise diagnostics and treatment of multiple hematopoietic and immune related diseases. Copyright © 2015 Elsevier Ltd. All rights reserved.

  20. Altered hematopoiesis in trisomy 21 as revealed through in vitro differentiation of isogenic human pluripotent cells

    Science.gov (United States)

    MacLean, Glenn A.; Menne, Tobias F.; Guo, Guoji; Sanchez, Danielle J.; Park, In-Hyun; Daley, George Q.; Orkin, Stuart H.

    2012-01-01

    Trisomy 21 is associated with hematopoietic abnormalities in the fetal liver, a preleukemic condition termed transient myeloproliferative disorder, and increased incidence of acute megakaryoblastic leukemia. Human trisomy 21 pluripotent cells of various origins, human embryionic stem (hES), and induced pluripotent stem (iPS) cells, were differentiated in vitro as a model to recapitulate the effects of trisomy on hematopoiesis. To mitigate clonal variation, we isolated disomic and trisomic subclones from the same parental iPS line, thereby generating subclones isogenic except for chromosome 21. Under differentiation conditions favoring development of fetal liver-like, γ-globin expressing, definitive hematopoiesis, we found that trisomic cells of hES, iPS, or isogenic origins exhibited a two- to fivefold increase in a population of CD43+(Leukosialin)/CD235+(Glycophorin A) hematopoietic cells, accompanied by increased multilineage colony-forming potential in colony-forming assays. These findings establish an intrinsic disturbance of multilineage myeloid hematopoiesis in trisomy 21 at the fetal liver stage. PMID:23045682

  1. Detection of Extramedullary Multiple Myeloma in Liver by FDG-PET/CT

    International Nuclear Information System (INIS)

    Kim, Daeweung; Kim, Woo Hyoung; Kim, Myoung Hyoun; Choi, Keum Ha; Kim, Chang Guhn

    2014-01-01

    We present the case of a 42-year-old man with a painful mass lesion in the right shoulder that was detected by contrast-enhanced computed tomography (CT) and 18 F-fluoro-2-deoxyglucose ( 18 F-FDG) positron emission tomography (PET)/CT. Excisional biopsy revealed infiltration of plasma cells with anaplastic features, consistent with solitary plasmacytoma (PC). Serum analysis showed elevation of serum free lambda light chain levels (27.78 mg/l), with an abnormally high kappa:lambda ratio (2.33) and high total proteins (10.4 g/dl). Serum protein electrophoresis revealed an M spike in the gamma-globulin region (56.1 %=5.8 g/dl). Subsequently, 18 F-FDG PET/CT revealed another hypermetabolic mass in the right lobe of the liver. CT-guided biopsy of the liver lesion revealed plasma cell myeloma, consistent with multiple myeloma. Multiple myeloma presenting as nodular liver masses is very rare in clinical practice. In a retrospective review of more than 2,000 patients, Talamo et al. reported only nine cases where there was nodular involvement of the liver by multiple myeloma. The organ most commonly involved was the liver, followed by pancreas, stomach, peritoneum with malignant ascites, colon, rectum, duodenum and ileum. Therefore, the literature published thus far has been limited to a few reports and case series. Among these reports, some had demonstrated the PET or PET/CT findings of nodular liver involvement of multiple myeloma. About 10 % of the solitary myelomas appeared as extramedullary PC or solitary PC of bone. In spite of the advances in therapy, the treatment of multiple myeloma is still palliative. However, solitary PC could be cured by resection or radiation therapy. Thus, differentiation between PC and multiple myeloma is essential in making a decision for the appropriate therapeutic regimen. 18 F-FDG PET/CT has the unique ability to detect and characterize malignant lesions in one single examination. Schirrmeister et al. reported that 18 F-FDG PET revealed

  2. Detection of Extramedullary Multiple Myeloma in Liver by FDG-PET/CT

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Daeweung; Kim, Woo Hyoung; Kim, Myoung Hyoun; Choi, Keum Ha; Kim, Chang Guhn [Wonkwang Univ. School of Medicine, Iksan (Korea, Republic of)

    2014-06-15

    We present the case of a 42-year-old man with a painful mass lesion in the right shoulder that was detected by contrast-enhanced computed tomography (CT) and {sup 18}F-fluoro-2-deoxyglucose ({sup 18}F-FDG) positron emission tomography (PET)/CT. Excisional biopsy revealed infiltration of plasma cells with anaplastic features, consistent with solitary plasmacytoma (PC). Serum analysis showed elevation of serum free lambda light chain levels (27.78 mg/l), with an abnormally high kappa:lambda ratio (2.33) and high total proteins (10.4 g/dl). Serum protein electrophoresis revealed an M spike in the gamma-globulin region (56.1 %=5.8 g/dl). Subsequently, {sup 18}F-FDG PET/CT revealed another hypermetabolic mass in the right lobe of the liver. CT-guided biopsy of the liver lesion revealed plasma cell myeloma, consistent with multiple myeloma. Multiple myeloma presenting as nodular liver masses is very rare in clinical practice. In a retrospective review of more than 2,000 patients, Talamo et al. reported only nine cases where there was nodular involvement of the liver by multiple myeloma. The organ most commonly involved was the liver, followed by pancreas, stomach, peritoneum with malignant ascites, colon, rectum, duodenum and ileum. Therefore, the literature published thus far has been limited to a few reports and case series. Among these reports, some had demonstrated the PET or PET/CT findings of nodular liver involvement of multiple myeloma. About 10 % of the solitary myelomas appeared as extramedullary PC or solitary PC of bone. In spite of the advances in therapy, the treatment of multiple myeloma is still palliative. However, solitary PC could be cured by resection or radiation therapy. Thus, differentiation between PC and multiple myeloma is essential in making a decision for the appropriate therapeutic regimen. {sup 18}F-FDG PET/CT has the unique ability to detect and characterize malignant lesions in one single examination. Schirrmeister et al. reported that

  3. Anatomic relationship of intrahepatic bile ducts to portal veins revisited

    International Nuclear Information System (INIS)

    Bret, P.M.; Stempel, J.; Atri, M.; Lough, J.O.; Illescas, F.F.

    1987-01-01

    It is well accepted that intrahepatic bile ducts lie in front of corresponding portal vein branches. Since the authors' clinical experience with US was different, they studied 18 normal necropsy cadaver livers. The common bile duct, main portal vein, and hepatic artery were cannulated and injected respectively with air, dilute contrast medium, and mineral oil. The livers were then examined in anatomic position with CT. In the left lobe of the liver, the bile ducts were anterior to the portal vein in seven cases, posterior in seven cases, and were tortuous both anterior and posterior in three cases. In the right lobe, the bile ducts were anterior in nine cases, posterior in five cases, tortuous in one case, and not seen in two cases. In the porta hepatis, the bile ducts were anterior in eight cases, posterior in one case, tortuous in five cases, and not seen in three cases. Histologic specimens confirmed the anterior and posterior location of the bile ducts relative to the portal veins. In conclusion, intrahepatic bile ducts can be either anterior or posterior to the corresponding portal vein branches

  4. A study on CT features of intrahepatic bile duct abscess

    International Nuclear Information System (INIS)

    Min Pengqiu; Li Peng; He Zhiyan; Chen Weixia; Liu Yan

    2001-01-01

    Objective: To evaluate CT features of intrahepatic bile duct abscess (IBDA) and its pathologic basis. Methods: The CT imaging data of 31 consecutive cases of intrahepatic bile duct abscess proved by surgery or clinical treatments from October 1989 to February 1999 were retrospectively studied. The causes included acute obstructive suppurative cholangitis and retrograde infection due to different etiologies. For all the cases, the CT manifestations of liver abscess, bile duct abnormalities, and their relationship were observed respectively. Results: Manifestations of liver abscess were revealed in all cases (31/31, 100%). The CT manifestations of bile duct abnormalities included signs of etiologies caused bile duct obstruction and other signs including cholangiectasis (29/31, 93.5%), the dilated bile ducts communicated with (5/31, 16.1%) or abut on (8/31, 25.8%) the abscesses, and gas collection in bile ducts (10/31, 32.2%). The signs showing the relationship between liver abscess and bile duct abnormalities were that the abscesses complied with the obstructive site and the dilated bile ducts (15/31, 48.4%), and the liver abscesses located in different (7/31, 22.6%) or same (4/31, 12.9%) liver lobes or segments with gas collection in the dilated bile ducts. Conclusion: The CT manifestations of IBDA included signs of liver abscess, abnormalities of bile ducts, and signs showing their relationship. CT scanning was helpful in making comprehensive and accurate diagnosis of IBDA

  5. Incidence of extramedullary relapse after haploidentical SCT for advanced AML/myelodysplastic syndrome.

    Science.gov (United States)

    Yoshihara, S; Ikegame, K; Kaida, K; Taniguchi, K; Kato, R; Inoue, T; Fujioka, T; Tamaki, H; Okada, M; Soma, T; Ogawa, H

    2012-05-01

    Extramedullary (EM) relapse of leukemia after allo-SCT in patients with AML/myelodysplastic syndrome has been increasingly reported. The reduced effectiveness of the GVL effect in EM sites, as compared with BM, has been suggested to underlie this problem. We retrospectively analyzed the pattern of relapse after haploidentical SCT (haplo-SCT), performed as the first or second SCT. Among 38 patients who received haplo-SCT as their first SCT, the cumulative incidences of BM and EM relapse at 3 years were 40.5 and 10.9%, respectively. Among 19 patients who received haplo-SCT as their second SCT, the cumulative incidences of BM and EM relapse were 30.9 and 31.9%, respectively. Moreover, most of the patients who underwent repeat haplo-SCT for the treatment of EM relapse had further EM relapse at other sites. Post-relapse survival did not differ significantly with different patterns of relapse. The frequent occurrence of EM relapse after haplo-SCT, particularly when performed as a second SCT, suggests that the potent GVL effect elicited by an HLA disparity also occurs preferentially in BM. Our findings emphasize the need for a treatment strategy for EM relapse that recognizes the reduced susceptibility of EM relapse to the GVL effect.

  6. A RARE CASE OF EXTRAMEDULLARY PLASMACYTOMA MASQUERADING AS JUVENILE NASOPHARYNGEAL ANGIOFIBROMA

    Directory of Open Access Journals (Sweden)

    Harshita V. Sabhahit

    2016-09-01

    Full Text Available BACKGROUND Plasmacytomas are rare plasma cell tumours occurring consequent to monoclonal proliferation of plasma cells. They are divided into Solitary bone plasmacytoma, Extramedullary plasmacytoma (EMP and Multiple myeloma. EMPs are commonly housed in the head and neck region with a predilection to the mucosa associated lymphoid tissue in the aerodigestive tract. The nasal cavity, paranasal sinuses, and nasopharynx are the most common sites. OBJECTIVE We describe our experience with this tumour owing to its clinical rarity and a different way of presentation. METHODS After complete surgical excision of a nasal mass presenting in a young male with features masquerading that of Juvenile Nasopharyngeal Angiofibroma, a diagnosis of plasma cell tumour was made on histopathological analysis which was confirmed using immunohistochemistry. Serum electrophoresis, urine Bence Jones proteins, complete skeletal survey were done to rule out any progression into multiple myeloma. Radiation therapy was given with 45 Gy in 25 fractions at 1.8 Gy per day, 5 days a week. RESULTS Followup after 2 years showed no recurrence locally as well as in regional nodes. CONCLUSION Given to the rarity of the tumour, undefined manner of presentation and a predominant prevalence in the head and neck region, every otolaryngologist should keep EMP in mind while considering sinonasal masses. A multidisciplinary approach with a combination of surgery and radiotherapy is found to benefit the patient significantly. A long term watch out for progression to MM is mandatory to commence early treatment and thus prolonged disease-free survival from the same.

  7. Extramedullary Plasmacytoma of the Larynx: A Case Report of Subglottic Localization

    Directory of Open Access Journals (Sweden)

    Jaqueline Ramírez-Anguiano

    2012-01-01

    Full Text Available Extramedullary plasmacytoma (EMP is a rare neoplasm of plasma cells, described in soft tissue outside the bone marrow. EMP of the larynx represents 0.04 to 0.45% of malignant tumors of the larynx. A male of 57 years old presented with hoarseness, dyspnea, and biphasic stridor of 2 months. The indirect laryngoscopy (IL revealed severe edema of the posterior commissure and a polypoid mass in the right posterior lateral subglottic wall. A biopsy of the subglottic mass was performed by a direct laryngoscopy (DL. The histopathologic diagnosis was EMP CD138+, therefore radiotherapy was given at 54 Gy in 30 sessions. The patient had an adequate postoperative clinical course and a new biopsy was performed having tumor-free margins. All laryngeal lesions should be biopsied prior to treatment to determine an accurate diagnosis to guide a proper management of the condition. Radiation therapy to the EMP is considered the treatment of choice, having local control rates of 80% to 100%. The subglottis is the least accessible area of view and the least frequent location of a laryngeal mass, nevertheless the otolaryngologist should always do a complete and systematic exam of the larynx when a tumor is suspected, to detect diagnoses such as a subglottic plasmacytoma.

  8. A rare case of cervical epidural extramedullary plasmacytoma presenting with monoparesis

    Directory of Open Access Journals (Sweden)

    Turk Okan

    2017-03-01

    Full Text Available Multiple myeloma and other plasma cell disorders are characterized by production of a large number of plasma cells in the bone marrow. On the other hand, plasmacytoma results from proliferation of abnormal plasma cells in the soft tissue or skeletal system. Neurological complications are frequently observed in these diseases. The most commonly known complications among those complications are spine fractures, spinal cord compressions, and peripheral neuropathies. Although neurological involvements are common in plasmacytomas, extramedullary spinal epidural localizations have been reported very rarely. In this case report, we aimed to present a plasmacytoma case that presented with acute onset of upper extremity monoparesis. A 40-year-old woman was admitted to our clinic with complaints of sudden weakness and numbness in her left arm following neck and left arm pain. Emergency cervical magnetic resonance imaging (MRI revealed an epidural mass and the patient underwent emergency surgery. The patient showed improvement post-operatively and the pathology was reported as plasmacytoma. Following hematology consultation, systemic chemotherapy was initiated and radiotherapy was planned after wound healing.

  9. Neuroradiological evaluation of benign extramedullary tumors in the high cervical region and at the foramen magnum

    Energy Technology Data Exchange (ETDEWEB)

    Nishiura, Iwao; Koyama, Tsunemaro; Tanaka, Kimito; Aii, Heihachirou

    1986-06-01

    Twelve cases of benign extramedullary tumors in the high cervical region and at the foramen magnum were experienced during past five years among eighty all spinal and paraspinal tumors. The diagnosis of masses in this region is very difficult because of the variety of clinical course, symptoms and neurological findings as pointed out by many reporters. Also in our cases, 70 % of the patients complained of the deteriorated motor weakness of the upper or lower extremities on admission, though they had noticed the onset of slight neck or occipital pain a few years ago. Neurological examination on admission clearly showed the symptom of myelopathy except in two cases with a tumor at the foramen magnum and C/sub 1/ level. The percentage of positive findings of plain X-rays was 50 %, that of metrizamide myelography was 92 % and that of IV. e. CT and met. e. CT was 100 %. NMR-CT was performed in 2 cases, and in one of them it was useful in confirming the tumor configuration and extension. Five interesting cases were described mainly from the neuroradiological aspects. Finally the differentiation between meningioma and neurinoma was discussed from the aspects of myelogram, CT and NMR-CT. As already pointed out, it is most important not to forget the existence of tumors in this region when one comes across the confused symptoms, afterwards not to overlook the slight positive neurological and neuroradiological findings.

  10. Three-dimensional reconstructions of intrahepatic bile duct tubulogenesis in human liver

    DEFF Research Database (Denmark)

    Vestentoft, Peter S; Jelnes, Peter; Hopkinson, Branden M

    2011-01-01

    BACKGROUND: During liver development, intrahepatic bile ducts are thought to arise by a unique asymmetric mode of cholangiocyte tubulogenesis characterized by a series of remodeling stages. Moreover, in liver diseases, cells lining the Canals of Hering can proliferate and generate new hepatic...... in normal liver and in the extensive ductular reactions originating from intrahepatic bile ducts and branching into the parenchyma of the acetaminophen intoxicated liver. In the developing human liver, three-dimensional reconstructions using multiple marker proteins confirmed that the human intrahepatic...

  11. A new method for the measurement of intrahepatic shunts

    International Nuclear Information System (INIS)

    Hoefs, J.C.; Reynolds, T.B.; Pare, P.; Sakimura, I.

    1984-01-01

    After transhepatic portal pressure determination, 96 patients were assessed for the presence of intrahepatic shunts by injection of microspheres (25 +/- 5 micron diameter) into the portal vein using RISA-131I as an indicator of dilution. Multiple portal vein injections in each patient allowed blood sampling from the hepatic vein (site 1) and from two inferior vena cava sampling sites (site 2, at the junction of the hepatic vein orifice with the inferior vena cava, and site 3, 2 to 3 cm closer to or within the right atrium). Intrahepatic shunting was calculated from each site: hepatic vein in 57 patients and inferior vena cava, site 2 in 43 patients and site 3 in 77 patients. At least one valid IHS calculation was available in 92 of the patients. Intrahepatic shunting calculated from sequential portal vein injections with sampling from the hepatic vein was highly correlated (r . 0.98, p less than 0.0001, slope . 1.0), with a mean difference of 1.9% +/- 1.9%. There was no significant difference by t test comparison of the mean IHS calculated from sites 1, 2, and 3. Occasional marked discrepancies were noted between IHS calculated from site 1 or site 2 compared with site 3, and the site 3 calculation was always greater. A shunt index in all patients included shunts calculated from the hepatic vein in 57 patients plus shunt calculation from the inferior vena cava in the remaining patients (site 2 in 26 patients and site 3 in nine). The 82 patients with portal hypertension or chronic liver disease had a higher portal pressure, 13.8 +/- 4.6 mm Hg, and a significantly greater shunt index, 13.7% +/- 24.5% compared with controls. The frequency distribution of IHS in patients with chronic liver disease demonstrated less than 2% IHS in 49% of patients and less than 5% IHS in 63%. The validity of our methods and the implications of the infrequent demonstration of a large IHS are discussed

  12. Intra- and Extramedullary Dumbbell-Shaped Schwannoma of the Medulla Oblongata: A Case Report and Review of the Literature.

    Science.gov (United States)

    Zhang, Qing; Ni, Ming; Liu, Wei-Ming; Jia, Wang; Jia, Gui-Jun; Zhang, Jun-Ting

    2017-02-01

    Brainstem intramedullary schwannomas (ISs) are extremely rare. Various theories have been suggested to explain its origin. It was first speculated that ISs arise from the region where the nerve roots lose their sheaths on penetrating the pia mater. Later, it was further predicted that ISs would contain both intra- and extramedullary parts and would be shaped like a dumbbell. However, no cases reported previously can support this assumption adequately. A 40-year-old woman presented with constant cervical pain, accompanied by progressive weakness of upper extremities and glove distribution numbness. Magnetic resonance imaging of the brain revealed a rare intra- and extramedullary dumbbell-shaped lesion of the medulla oblongata, which was partially removed via a midline suboccipital craniectomy. Histologic and immunohistochemical examinations confirmed the diagnosis of schwannoma. Routine imaging performed 20 months after the initial resection revealed a regrowth of the intramedullary part, which was subsequently partially removed through a far-lateral approach, with symptoms alleviated. At 2-year follow-up, there continued to be no radiologic or clinical evidence of regrowth. To date and to our knowledge, there are only 16 reported cases of brainstem ISs, none of which contained both intra- and extramedullary components. We believe this is the first report of dumbbell schwannoma of the medulla oblongata with adequate radiologic evidence. The relevant literature is reviewed, and an assumption has been proposed that dumbbell or surfacing ISs arising near entry zones of sensory nerves, mixed cranial nerves, or ventral root may originate from the aberrant Schwann cells. Copyright © 2016 Elsevier Inc. All rights reserved.

  13. Isolated Extramedullary Relapse of Acute Leukemia after Allogeneic Stem Cell Transplantation: Different Kinetics and Better Prognosis than Systemic Relapse.

    Science.gov (United States)

    Shem-Tov, Noga; Saraceni, Francesco; Danylesko, Ivetta; Shouval, Roni; Yerushalmi, Ronit; Nagler, Arnon; Shimoni, Avichai

    2017-07-01

    Allogeneic stem cell transplantation (SCT) is curative treatment in patients with acute leukemia and myelodysplastic syndrome. However, recurrent disease is the major cause of treatment failure. Isolated extramedullary relapse (iEMR) after SCT is relatively rare and not well characterized. We performed a retrospective analysis of 566 consecutive patients with acute myeloid leukemia (n = 446) and acute lymphoblastic leukemia (ALL; n = 120) after SCT to study the incidence, risk factors, treatment options, and outcome of iEMR. The 5-year cumulative incidence of bone marrow relapse (BMR) and iEMR was 41.0% and 5.8%, respectively. iEMR occurred significantly later than BMR at 10 and 4 months, respectively (P BMR but did not protect against iEMR. Most patients with iEMR received systemic treatment combined with local radiation and donor lymphocyte infusions when feasible. The 3-year survival after relapse was 8.5% and 30.1% after BMR and iEMR, respectively (P = .002). Patients with a first iEMR continued to have recurrent EMRs, and only a minority progressed to BMR. Second iEMR was also common after first BMR and associated with longer survival than second BMR. iEMR is more frequent in patients with ALL and prior extramedullary disease. It occurs later than BMR and more commonly in patients with chronic GVHD, suggesting less effective graft-versus-leukemia effect in extramedullary sites. Second iEMR is common after a first iEMR but also after a first BMR. Long-term survival is feasible with aggressive treatment. Copyright © 2017 The American Society for Blood and Marrow Transplantation. Published by Elsevier Inc. All rights reserved.

  14. Intra-extramedullary drainage as an effective option for treatment of intramedullary ependymal cyst of thoracic spine: technical note.

    Science.gov (United States)

    Landi, Alessandro; Pietrantonio, Andrea; Marotta, Nicola; Mancarella, Cristina; Delfini, Roberto

    2014-03-01

    Intramedullary neuroepithelial cysts are extremely rare and only 15 cases have been reported in the literature. Clinico-radiological features are not indicative of a specific diagnosis; for this reason, diagnosis is based mainly on the histological features. In the literature, total surgical removal is considered the treatment of choice. The risk of recurrence is higher after partial removal and in cases of occlusion of intra-extramedullary shunt. For this reason, a surgical strategy that ensures the shunt patency in case of incomplete removal of the cyst becomes a very safe option for treatment of this pathology. We report the case of a 51-year-old woman who was found to have a dorsal (D9) intramedullary neuroepithelial cyst. She underwent surgical treatment with partial removal and placement of a Nelaton drainage device (8 French) inside the intra-extramedullary shunt. The patient experienced a complete regression of preoperative symptoms and magnetic resonance imaging (MRI) follow-up showed no radiological evidence of recurrence 24 months after surgical treatment. Spinal ependymal cysts show a high frequency of recurrence, especially in cases of partial removal of the cyst wall. Unfortunately, the cyst walls are often closely adherent to the spinal cord, making total removal impossible. Intra-extramedullary shunting is a viable option, although there is a high frequency of recurrence in cases of obstruction of the shunt. Placing an 8 Ch Nelaton drain between the dorsal columns is a reliable technique, especially in cases of partial removal. In fact, it allows continuous drainage of cyst fluid and subsequent resolution of symptoms, and it decreases the incidence of recurrences due to obstruction of the shunt. Georg Thieme Verlag KG Stuttgart · New York.

  15. Daunorubicin, Cytarabine, and Cladribine Regimen Plus Radiotherapy and Donor Lymphocyte Infusion for Extramedullary Relapse of Acute Myeloid Leukemia after Hematopoietic Stem Cell Transplantation

    Directory of Open Access Journals (Sweden)

    Marco Sanna

    2013-01-01

    Full Text Available Myeloid sarcoma is a rare tumor consisting of myeloid blasts that involve anatomic sites outside the bone marrow. Fatal prognosis is inevitable in patients with extramedullary relapse after hematopoietic stem cell transplantation (HSCT, and no standard treatments are available yet. We report the first case of extramedullary relapse after HSCT treated with a combination of daunorubicin, cytarabine, and cladribine (DAC regimen plus radiotherapy and donor lymphocyte infusion (DLI. This treatment induced a new and durable remission in our patient. The favorable toxicity profile and the reduced cost make this combination worthy of further investigations.

  16. Isthmin 1 (ism1) is required for normal hematopoiesis in developing zebrafish.

    Science.gov (United States)

    Berrun, Arturo; Harris, Elena; Stachura, David L

    2018-01-01

    Hematopoiesis is an essential and highly regulated biological process that begins with hematopoietic stem cells (HSCs). In healthy organisms, HSCs are responsible for generating a multitude of mature blood cells every day, yet the molecular pathways that instruct HSCs to self-renew and differentiate into post-mitotic blood cells are not fully known. To understand these molecular pathways, we investigated novel genes expressed in hematopoietic-supportive cell lines from the zebrafish (Danio rerio), a model system increasingly utilized to uncover molecular pathways important in the development of other vertebrate species. We performed RNA sequencing of the transcriptome of three stromal cell lines derived from different stages of embryonic and adult zebrafish and identified hundreds of highly expressed transcripts. For our studies, we focused on isthmin 1 (ism1) due to its shared synteny with its human gene ortholog and because it is a secreted protein. To characterize ism1, we performed loss-of-function experiments to identify if mature blood cell production was disrupted. Myeloid and erythroid lineages were visualized and scored with transgenic zebrafish expressing lineage-specific markers. ism1 knockdown led to reduced numbers of neutrophils, macrophages, and erythrocytes. Analysis of clonal methylcellulose assays from ism1 morphants also showed a reduction in total hematopoietic stem and progenitor cells (HSPCs). Overall, we demonstrate that ism1 is required for normal generation of HSPCs and their downstream progeny during zebrafish hematopoiesis. Further investigation into ism1 and its importance in hematopoiesis may elucidate evolutionarily conserved processes in blood formation that can be further investigated for potential clinical utility.

  17. Isthmin 1 (ism1) is required for normal hematopoiesis in developing zebrafish

    Science.gov (United States)

    Berrun, Arturo; Harris, Elena

    2018-01-01

    Hematopoiesis is an essential and highly regulated biological process that begins with hematopoietic stem cells (HSCs). In healthy organisms, HSCs are responsible for generating a multitude of mature blood cells every day, yet the molecular pathways that instruct HSCs to self-renew and differentiate into post-mitotic blood cells are not fully known. To understand these molecular pathways, we investigated novel genes expressed in hematopoietic-supportive cell lines from the zebrafish (Danio rerio), a model system increasingly utilized to uncover molecular pathways important in the development of other vertebrate species. We performed RNA sequencing of the transcriptome of three stromal cell lines derived from different stages of embryonic and adult zebrafish and identified hundreds of highly expressed transcripts. For our studies, we focused on isthmin 1 (ism1) due to its shared synteny with its human gene ortholog and because it is a secreted protein. To characterize ism1, we performed loss-of-function experiments to identify if mature blood cell production was disrupted. Myeloid and erythroid lineages were visualized and scored with transgenic zebrafish expressing lineage-specific markers. ism1 knockdown led to reduced numbers of neutrophils, macrophages, and erythrocytes. Analysis of clonal methylcellulose assays from ism1 morphants also showed a reduction in total hematopoietic stem and progenitor cells (HSPCs). Overall, we demonstrate that ism1 is required for normal generation of HSPCs and their downstream progeny during zebrafish hematopoiesis. Further investigation into ism1 and its importance in hematopoiesis may elucidate evolutionarily conserved processes in blood formation that can be further investigated for potential clinical utility. PMID:29758043

  18. Computer tomographic and angiographic studies of histologically confirmed intrahepatic masses

    International Nuclear Information System (INIS)

    Janson, R.; Lackner, K.; Paquet, K.J.; Thelen, M.; Thurn, P.

    1980-01-01

    The computer tomographic and angiographic findings in 53 patients with intrahepatic masses were compared. The histological findings show that 17 were due to echinococcus, 12 were due to hepatic carcinoma, ten were metastases, five patients had focal nodular hyperplasia, three an alveolar echinococcus and there were three cases with an haemangioma of the liver and a further three liver abscesses. Computer tomography proved superior in peripherally situated lesions, and in those in the left lobe of the liver. Arteriography was better at demonstrating lesions below 2 cm in size, particularly vascular tumours. As a pre-operative measure, angiography is to be preferred since it is able to demonstrate anatomic anomalies and variations in the blood supply, as well as invasion of the portal vein or of the inferior vena cava. (orig.) [de

  19. Computer tomographic and angiographic studies of histologically confirmed intrahepatic masses

    Energy Technology Data Exchange (ETDEWEB)

    Janson, R.; Lackner, K.; Paquet, K.J.; Thelen, M.; Thurn, P.

    1980-06-01

    The computer tomographic and angiographic findings in 53 patients with intrahepatic masses were compared. The histological findings show that 17 were due to echinococcus, 12 were due to hepatic carcinoma, ten were metastases, five patients had focal nodular hyperplasia, three an alveolar echinococcus and there were three cases with an haemangioma of the liver and a further three liver abscesses. Computer tomography proved superior in peripherally situated lesions, and in those in the left lobe of the liver. Arteriography was better at demonstrating lesions below 2 cm in size, particularly vascular tumours. As a pre-operative measure, angiography is to be preferred since it is able to demonstrate anatomic anomalies and variations in the blood supply, as well as invasion of the portal vein or of the inferior vena cava.

  20. Molecular Pathogenesis and Current Therapy in Intrahepatic Cholangiocarcinoma

    DEFF Research Database (Denmark)

    Høgdall, Dan Taksony Solyom; O'Rourke, Colm J; Taranta, Andrzej

    2016-01-01

    , the context of tumor plasticity and the causative features driving the disease. Molecular profiling and pathological techniques have begun to underline persistent alterations that may trigger inherited drug resistance (a hallmark of hepatobiliary and pancreatic cancers), metastasis and disease recurrence......Intrahepatic cholangiocarcinoma (iCCA) comprises one of the most rapidly evolving cancer types. An underlying chronic inflammatory liver disease that precedes liver cancer development for several decades and creates a pro-oncogenic microenvironment frequently impairs progress in therapeutic...... clinical strategies and patient outcome. This was achieved for other cancers, such as breast carcinoma, facilitated by the delineation of patient subsets and of precision therapies. In iCCA, many questions persevere as to the evolutionary process and cellular origin of the initial transforming event...

  1. Intrahepatic cholestasis in subclinical and overt hyperthyroidism: two case reports

    Directory of Open Access Journals (Sweden)

    Soylu Aliye

    2008-04-01

    Full Text Available Abstract Introduction Non-specific abnormalities in liver function tests might accompany the clinical course of hyperthyroidism. Hyperthyroidism can cause the elevation of hepatic enzymes and bilirubin. Jaundice is rare in overt hyperthyroidism, especially in subclinical hyperthyroidism. On the other hand, the use of anti-thyroid drugs has rarely been associated with toxic hepatitis and cholestatic jaundice. Case presentation Here we present two cases of cholestasis that accompanied two distinct forms of clinical hyperthyroidism. The first patient had a clinical presentation of severe cholestasis in the absence of congestive failure related to hyperthyroidism. The second case had developed intrahepatic cholestasis in the presence of subclinical hyperthyroidism, and improved with rifampicin treatment. Conclusion Hyperthyroidism should be a consideration in non-specific liver dysfunction.

  2. A case of an intrahepatic fish bone penetration

    International Nuclear Information System (INIS)

    Tsuboi, Kazuhiko; Nakajima, Yoshiro; Yamamoto, Shunji; Nagao, Masatoshi; Nishimura, Kazumasa.

    1981-01-01

    A 56-year-old man was admitted to our hospital because of epigastric discomfort, appetite loss and body weight loss. A gallstone, signs of chronic inflammation and CEA-Z: 12.5 ng/ml were found. Abdominal CT scan revealed an intrahepatic low density nodule and an intra-and-extrahepatic high-dense, needle-like foreign body. By laparotomy a fish bone penetrating into the left lateral segment of the liver from the anterior wall of the prepyloric region of the stomach was found. Cholecystectomy was performed. The penetrating fish bone was withdrawn from the liver easily. The Postoperative course was smooth. The possibility of the definitive preoperative diagnosis of the intestinal fish bone penetration by abdominal CT scan was suggested. (author)

  3. Cervical Hemilaminoplasty with Miniplates in Long Segment Intradural Extramedullary Ependymoma: Case Report and Technical Note.

    Science.gov (United States)

    Oral, Sukru; Tumturk, Abdulfettah; Kucuk, Ahmet; Menku, Ahmet

    2018-01-01

    The surgical approaches for spinal tumors, to a great extent, have been developed in accordance with the developments in medical technology. Today, many surgical techniques are implemented as anterior, anterolateral, posterior, posterolateral and combined approaches. Due to its low morbidity, the posterior approach is the more preferred one. Laminectomy is a widely used technique, especially in neoplastic lesions. However, following laminectomy, there are numerous complications such as instability, kyphotic deformity and scar formation. In this paper, the excision of a tumor that was located intradural-extramedullary at the C3-C7 level with the cervical hemilaminoplasty technique is described. A 47-year-old female patient presented to our clinic with increasing complaints of neck and left arm pain, left arm numbness and searing pain for the last 10 years. On examination, hypoesthesia at the C4-7 dermatomes in the left upper extremity, an increase in deep tendon reflexes, and bilateral positive Hoffmann reflexes were observed. C3-C7 laminae were opened unilaterally on the right side with a midline skin incision. The laminae were drilled with a high-speed drill to provide a wide opening, both on the midline obliquely and from the border of the lamina-facet joint. After the tumor was totally excised, hemilaminae were placed into the previous position and reconstructed with mini-plates and screws. Cervical hemilaminoplasty provides a wide field of vision in tumor surgery of this region. Besides, the reconstruction of hemilaminae is important for stability. As the integrity of the spinal canal is preserved during reoperations of this region, the risk of complications is decreased.

  4. Ginsenoside Rg1 improves bone marrow haematopoietic activity via extramedullary haematopoiesis of the spleen.

    Science.gov (United States)

    Liu, Hua-Hsing; Chen, Fei-Peng; Liu, Rong-Kai; Lin, Chun-Lin; Chang, Ko-Tung

    2015-11-01

    Cyclophosphamide (CY) is a chemotherapeutic agent used for cancer and immunological diseases. It induces cytotoxicity of bone marrow and causes myelosuppression and extramedullary haematopoiesis (EMH) in treated patients. EMH is characterized with the emergence of multipotent haematopoietic progenitors most likely in the spleen and liver. Previous studies indicated that a Chinese medicine, ginsenoside Rg1, confers a significant effect to elevate the number of lineage (Lin(-) ) Sca-1(+) c-Kit(+) haematopoietic stem and progenitor cells (HSPCs) and restore the function of bone marrow in CY-treated myelosuppressed mice. However, whether the amelioration of bone marrow by Rg1 accompanies an alleviation of EMH in the spleen was still unknown. In our study, the cellularity and weight of the spleen were significantly reduced after Rg1 treatment in CY-treated mice. Moreover, the number of c-Kit(+) HSPCs was significantly decreased but not as a result of apoptosis, indicating that Rg1 alleviated EMH of the spleen induced by CY. Unexpectedly, the proliferation activity of c-Kit(+) HSPCs was only up-regulated in the spleen, but not in the bone marrow, after Rg1 treatment in CY-treated mice. We also found that a fraction of c-Kit(+) /CD45(+) HSPCs was simultaneously increased in the circulation after Rg1 treatment. Interestingly, the effects of Rg1 on the elevation of HSPCs in bone marrow and in the peripheral blood were suppressed in CY-treated splenectomized mice. These results demonstrated that Rg1 improves myelosuppression induced by CY through its action on the proliferation of HSPCs in EMH of the spleen and migration of HSPCs from the spleen to the bone marrow. © 2015 The Authors. Journal of Cellular and Molecular Medicine published by John Wiley & Sons Ltd and Foundation for Cellular and Molecular Medicine.

  5. Extramedullary Relapse Following Total Marrow and Lymphoid Irradiation in Patients Undergoing Allogeneic Hematopoietic Cell Transplantation

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Ji Hyun [Department of Radiation Oncology, City of Hope National Medical Center, Duarte, California (United States); Stein, Anthony [Department of Hematology/Hematopoietic Cell Transplantation, City of Hope National Medical Center, Duarte, California (United States); Tsai, Nicole [Department of Biostatistics, City of Hope National Medical Center, Duarte, California (United States); Schultheiss, Timothy E. [Department of Radiation Oncology, City of Hope National Medical Center, Duarte, California (United States); Palmer, Joycelynne [Department of Biostatistics, City of Hope National Medical Center, Duarte, California (United States); Liu, An [Department of Radiation Oncology, City of Hope National Medical Center, Duarte, California (United States); Rosenthal, Joseph [Department of Hematology/Hematopoietic Cell Transplantation, City of Hope National Medical Center, Duarte, California (United States); Department of Pediatrics, City of Hope National Medical Center, Duarte, California (United States); Forman, Stephen J. [Department of Hematology/Hematopoietic Cell Transplantation, City of Hope National Medical Center, Duarte, California (United States); Wong, Jeffrey Y.C., E-mail: jwong@coh.org [Department of Radiation Oncology, City of Hope National Medical Center, Duarte, California (United States)

    2014-05-01

    Purpose: Approximately 5% to 20% of patients who undergo total body irradiation (TBI) in preparation for hematopoietic cell transplantation (HCT) can develop extramedullary (EM) relapse. Whereas total marrow and lymphoid irradiation (TMLI) provides a more conformally targeted radiation therapy for patients, organ sparing has the potential to place the patient at a higher risk for EM relapse than TBI. This study evaluated EM relapse in patients treated with TMLI at our institution. Methods and Materials: Patients eligible for analysis had been enrolled in 1 of 3 prospective TMLI trials between 2006 and 2012. The TMLI targeted bones, major lymph node chains, liver, spleen, testes, and brain, using image-guided tomotherapy with total dose ranging from 12 to 15 Gy. Results: A total of 101 patients with a median age of 47 years were studied. The median follow-up was 12.8 months. Incidence of EM relapse and bone marrow (BM) relapse were 12.9% and 25.7%, respectively. Of the 13 patients who had EM relapse, 4 also had BM relapse, and 7 had EM disease prior to HCT. There were a total of 19 EM relapse sites as the site of initial recurrence: 11 soft tissue, 6 lymph node, 2 skin. Nine of these sites were within the target region and received ≥12 Gy. Ten initial EM relapse sites were outside of the target region: 5 sites received 10.1 to 11.4 Gy while 5 sites received <10 Gy. Pretransplantation EM was the only significant predictor of subsequent EM relapse. The cumulative incidence of EM relapse was 4% at 1 year and 11.4% at 2 years. Conclusions: EM relapse incidence was as frequent in regions receiving ≥10 Gy as those receiving <10 Gy. EM relapse rates following TMLI that included HCT regimens were comparable to published results with regimens including TBI and suggest that TMLI is not associated with an increased EM relapse risk.

  6. Extramedullary Relapse Following Total Marrow and Lymphoid Irradiation in Patients Undergoing Allogeneic Hematopoietic Cell Transplantation

    International Nuclear Information System (INIS)

    Kim, Ji Hyun; Stein, Anthony; Tsai, Nicole; Schultheiss, Timothy E.; Palmer, Joycelynne; Liu, An; Rosenthal, Joseph; Forman, Stephen J.; Wong, Jeffrey Y.C.

    2014-01-01

    Purpose: Approximately 5% to 20% of patients who undergo total body irradiation (TBI) in preparation for hematopoietic cell transplantation (HCT) can develop extramedullary (EM) relapse. Whereas total marrow and lymphoid irradiation (TMLI) provides a more conformally targeted radiation therapy for patients, organ sparing has the potential to place the patient at a higher risk for EM relapse than TBI. This study evaluated EM relapse in patients treated with TMLI at our institution. Methods and Materials: Patients eligible for analysis had been enrolled in 1 of 3 prospective TMLI trials between 2006 and 2012. The TMLI targeted bones, major lymph node chains, liver, spleen, testes, and brain, using image-guided tomotherapy with total dose ranging from 12 to 15 Gy. Results: A total of 101 patients with a median age of 47 years were studied. The median follow-up was 12.8 months. Incidence of EM relapse and bone marrow (BM) relapse were 12.9% and 25.7%, respectively. Of the 13 patients who had EM relapse, 4 also had BM relapse, and 7 had EM disease prior to HCT. There were a total of 19 EM relapse sites as the site of initial recurrence: 11 soft tissue, 6 lymph node, 2 skin. Nine of these sites were within the target region and received ≥12 Gy. Ten initial EM relapse sites were outside of the target region: 5 sites received 10.1 to 11.4 Gy while 5 sites received <10 Gy. Pretransplantation EM was the only significant predictor of subsequent EM relapse. The cumulative incidence of EM relapse was 4% at 1 year and 11.4% at 2 years. Conclusions: EM relapse incidence was as frequent in regions receiving ≥10 Gy as those receiving <10 Gy. EM relapse rates following TMLI that included HCT regimens were comparable to published results with regimens including TBI and suggest that TMLI is not associated with an increased EM relapse risk

  7. Management of extramedullary plasmacytoma: Role of radiotherapy and prognostic factor analysis in 55 patients.

    Science.gov (United States)

    Wen, Ge; Wang, Weihu; Zhang, Yujing; Niu, Shaoqing; Li, Qiwen; Li, Yexiong

    2017-10-01

    To investigate potential prognostic factors affecting patient outcomes and to evaluate the optimal methods and effects of radiotherapy (RT) in the management of extramedullary plasmacytoma (EMP). Data from 55 patients with EMP between November 1999 and August 2015 were collected. The median age was 51 (range, 22-77) years. The median tumor size was 3.5 (range, 1.0-15.0) cm. The median applied dose was 50.0 (range, 30.0-70.0) Gy. Thirty-nine patients (70.9%) presented with disease in the head or neck region. Twelve patients received RT alone, 9 received surgery (S) alone, 3 received chemotherapy (CT) alone, and 3 patients did not receive any treatment. Combination therapies were applied in 28 patients. The median follow-up duration was 56 months. The 5-year local recurrence-free survival (LRFS), multiple myeloma-free survival (MMFS), progression-free survival (PFS) and overall survival (OS) rates were 79.8%, 78.6%, 65.2% and 76.0%, respectively. Univariate analysis revealed that RT was a favourable factor for all examined endpoints. Furthermore, head and neck EMPs were associated with superior LRFS, MMFS and PFS. Tumor size <4 cm was associated with superior MMFS, PFS and OS; serum M protein negativity was associated with superior MMFS and PFS; age ≥50 years and local recurrence were associated with poor MMFS. The dose ≥45 Gy group exhibited superior 5-year LRFS, MMFS and PFS rates (94.7%, 94.4%, 90.0%, respectively), while the corresponding values for the dose <45 Gy group were 62.5% (P=0.008), 53.3% (P=0.036) and 41.7% (P<0.001). Involved-site RT of at least 45 Gy should be considered for EMP. Furthermore, patients with head and neck EMP, tumor size <4 cm, age <50 years and serum M protein negativity had better outcomes.

  8. Wnt signaling controls the specification of definitive and primitive hematopoiesis from human pluripotent stem cells.

    Science.gov (United States)

    Sturgeon, Christopher M; Ditadi, Andrea; Awong, Geneve; Kennedy, Marion; Keller, Gordon

    2014-06-01

    Efforts to derive hematopoietic stem cells (HSCs) from human pluripotent stem cells (hPSCs) are complicated by the fact that embryonic hematopoiesis consists of two programs, primitive and definitive, that differ in developmental potential. As only definitive hematopoiesis generates HSCs, understanding how this program develops is essential for being able to produce this cell population in vitro. Here we show that both hematopoietic programs transition through hemogenic endothelial intermediates and develop from KDR(+)CD34(-)CD144(-) progenitors that are distinguished by CD235a expression. Generation of primitive progenitors (KDR(+)CD235a(+)) depends on stage-specific activin-nodal signaling and inhibition of the Wnt-β-catenin pathway, whereas specification of definitive progenitors (KDR(+)CD235a(-)) requires Wnt-β-catenin signaling during this same time frame. Together, these findings establish simple selective differentiation strategies for the generation of primitive or definitive hematopoietic progenitors by Wnt-β-catenin manipulation, and in doing so provide access to enriched populations for future studies on hPSC-derived hematopoietic development.

  9. Role of Vitamin A/Retinoic Acid in Regulation of Embryonic and Adult Hematopoiesis

    Directory of Open Access Journals (Sweden)

    Ana Cañete

    2017-02-01

    Full Text Available Vitamin A is an essential micronutrient throughout life. Its physiologically active metabolite retinoic acid (RA, acting through nuclear retinoic acid receptors (RARs, is a potent regulator of patterning during embryonic development, as well as being necessary for adult tissue homeostasis. Vitamin A deficiency during pregnancy increases risk of maternal night blindness and anemia and may be a cause of congenital malformations. Childhood Vitamin A deficiency can cause xerophthalmia, lower resistance to infection and increased risk of mortality. RA signaling appears to be essential for expression of genes involved in developmental hematopoiesis, regulating the endothelial/blood cells balance in the yolk sac, promoting the hemogenic program in the aorta-gonad-mesonephros area and stimulating eryrthropoiesis in fetal liver by activating the expression of erythropoietin. In adults, RA signaling regulates differentiation of granulocytes and enhances erythropoiesis. Vitamin A may facilitate iron absorption and metabolism to prevent anemia and plays a key role in mucosal immune responses, modulating the function of regulatory T cells. Furthermore, defective RA/RARα signaling is involved in the pathogenesis of acute promyelocytic leukemia due to a failure in differentiation of promyelocytes. This review focuses on the different roles played by vitamin A/RA signaling in physiological and pathological mouse hematopoiesis duddurring both, embryonic and adult life, and the consequences of vitamin A deficiency for the blood system.

  10. Vascular Platform to Define Hematopoietic Stem Cell Factors and Enhance Regenerative Hematopoiesis

    Directory of Open Access Journals (Sweden)

    Michael G. Poulos

    2015-11-01

    Full Text Available Hematopoietic stem cells (HSCs inhabit distinct microenvironments within the adult bone marrow (BM, which govern the delicate balance between HSC quiescence, self-renewal, and differentiation. Previous reports have proposed that HSCs localize to the vascular niche, comprised of endothelium and tightly associated perivascular cells. Herein, we examine the capacity of BM endothelial cells (BMECs to support ex vivo and in vivo hematopoiesis. We demonstrate that AKT1-activated BMECs (BMEC-Akt1 have a unique transcription factor/cytokine profile that supports functional HSCs in lieu of complex serum and cytokine supplementation. Additionally, transplantation of BMEC-Akt1 cells enhanced regenerative hematopoiesis following myeloablative irradiation. These data demonstrate that BMEC-Akt1 cultures can be used as a platform for the discovery of pro-HSC factors and justify the utility of BMECs as a cellular therapy. This technical advance may lead to the development of therapies designed to decrease pancytopenias associated with myeloablative regimens used to treat a wide array of disease states.

  11. Hematopoietic Stem Cells Are the Major Source of Multilineage Hematopoiesis in Adult Animals.

    Science.gov (United States)

    Sawai, Catherine M; Babovic, Sonja; Upadhaya, Samik; Knapp, David J H F; Lavin, Yonit; Lau, Colleen M; Goloborodko, Anton; Feng, Jue; Fujisaki, Joji; Ding, Lei; Mirny, Leonid A; Merad, Miriam; Eaves, Connie J; Reizis, Boris

    2016-09-20

    Hematopoietic stem cells (HSCs) sustain long-term reconstitution of hematopoiesis in transplantation recipients, yet their role in the endogenous steady-state hematopoiesis remains unclear. In particular, recent studies suggested that HSCs provide a relatively minor contribution to immune cell development in adults. We directed transgene expression in a fraction of HSCs that maintained reconstituting activity during serial transplantations. Inducible genetic labeling showed that transgene-expressing HSCs gave rise to other phenotypic HSCs, confirming their top position in the differentiation hierarchy. The labeled HSCs rapidly contributed to committed progenitors of all lineages and to mature myeloid cells and lymphocytes, but not to B-1a cells or tissue macrophages. Importantly, labeled HSCs gave rise to more than two-thirds of all myeloid cells and platelets in adult mice, and this contribution could be accelerated by an induced interferon response. Thus, classically defined HSCs maintain immune cell development in the steady state and during systemic cytokine responses. Copyright © 2016 Elsevier Inc. All rights reserved.

  12. Impaired Hematopoiesis and Disrupted Monocyte/Macrophage Homeostasis in Mucopolysaccharidosis Type I Mice.

    Science.gov (United States)

    Viana, Gustavo Monteiro; Buri, Marcus Vinícius; Paredes-Gamero, Edgar Julian; Martins, Ana Maria; D'Almeida, Vânia

    2016-03-01

    Mucopolysaccharidosis type I (MPS I) is a rare autosomal recessive disease caused by alpha-L-iduronidase deficiency in which heparan and dermatan sulfate degradation is compromised. Besides primary lysosomal glycosaminoglycan accumulation, further changes in cellular functions have also been described in several murine MPS models. Herein, we evaluated alterations in hematopoiesis and its implications on the production of mature progeny in a MPS I murine model. Despite the significant increase in hematopoietic stem cells, a reduction in common myeloid progenitors and granulocyte-macrophage progenitor cells was observed in Idua -/- mice bone marrow. Furthermore, no alterations in number, viability nor activation of cell death mechanisms were observed in Idua -/- mice mature macrophages but they presented higher sensitivity to apoptotic induction after staurosporine treatment. In addition, changes in Ca(2+) signaling and a reduction in phagocytosis ability were also found. In summary, our results revealed significant intracellular changes in mature Idua -/- macrophages related to alterations in Idua -/- mice hematopoiesis, revealing a disruption in cell homeostasis. These results provide new insights into physiopathology of MPS I. © 2015 Wiley Periodicals, Inc.

  13. Effects of irradiation on the production of factors regulating humoral hematopoiesis

    International Nuclear Information System (INIS)

    Seki, Masatoshi; Yoshida, Kazuko

    1974-01-01

    Changes in factors regulating humoral hematopoiesis after irradiation were studied by intraperitoneal insertion of cellulose acetate membrane (CA membrane). There is little possibility that regulation factors is produced by macrophage itself. The growth of the colony depends mainly on increase or decrease of regulation factors in the host. However, there is a possibility that the macrophage interferes the action of these regulation factors. From the fact that there is no action of EPO (erythropoiesis) in Sl/slsup(d) mice having abnormal function of microenvironment, it is suggested that the action of EPO is brought about through the function of the microenvironment. In the analysis of the colony formation by intraperitoneal insertion of CA membrane, it is considered that the erythroblastic colony can be formed by the macrophage through the action of EPO. In short, it is concluded that factors regulating humoral hematopoiesis increased in the body of the mouse which have been irradiated to the whole body, and the increase in these factors is not constant, but remarkably changes with time. (Namekawa, K.)

  14. Engraftment and reconstitution of hematopoiesis is dependent on VEGFR2 mediated regeneration of sinusoidal endothelial cells

    Science.gov (United States)

    Hooper, Andrea T.; Butler, Jason M.; Nolan, Daniel J; Kranz, Andrea; Iida, Kaoruko; Kobayashi, Mariko; Kopp, Hans-Georg; Shido, Koji; Petit, Isabelle; Yanger, Kilangsungla; James, Daylon; Witte, Larry; Zhu, Zhenping; Wu, Yan; Pytowski, Bronislaw; Rosenwaks, Zev; Mittal, Vivek; Sato, Thomas N.; Rafii, Shahin

    2011-01-01

    SUMMARY The phenotypic attributes and molecular determinants for the regeneration of bone marrow (BM) sinusoidal endothelial cells (SECs) and their contribution to hematopoiesis are unknown. We show that after myelosuppression VEGFR2 activation promotes reassembly of regressed SECs, reconstituting hematopoietic stem and progenitor cells (HSPCs). VEGFR2 and VEGFR3 expression are restricted to BM vasculature, demarcating a continuous network of VEGFR2+VEGFR3+Sca1− SECs and VEGFR2+VEGFR3−Sca1+ arterioles. While chemotherapy (5FU) and sublethal irradiation (650 rad) induce minor SEC regression, lethal irradiation (950 rad) induces severe regression of SECs requiring BM transplantation (BMT) for regeneration. Conditional deletion of VEGFR2 in adult mice blocks regeneration of SECs in sublethally irradiated animals, preventing hematopoietic reconstitution. Inhibition of VEGFR2 signaling in lethally irradiated wild type mice rescued with BMT severely impairs SEC reconstruction, preventing engraftment and reconstitution of HSPCs. Therefore, activation of VEGFR2 is critical for regeneration of VEGFR3+Sca1− SECs that are essential for engraftment and restoration of HSPCs and hematopoiesis. PMID:19265665

  15. Histopathology of a benign bile duct lesion in the liver: Morphologic mimicker or precursor of intrahepatic cholangiocarcinoma.

    Science.gov (United States)

    Lee, Kyoung-Bun

    2016-09-01

    A bile duct lesion originating from intrahepatic bile ducts is generally regarded as an incidental pathologic finding in liver specimens. However, a recent study on the molecular classification of intrahepatic cholangiocarcinoma has focused on the heterogeneity of this carcinoma and has suggested that the cells of different origins present in the biliary tree may have a major role in the mechanism of oncogenesis. In this review, benign intrahepatic bile duct lesions-regarded in the past as reactive changes or remnant developmental anomalies and now noted to have potential for developing precursor lesions of intrahepatic cholangiocarcinoma-are discussed by focusing on the histopathologic features and its implications in clinical practice.

  16. Application of adiponectin, TNF-α and ferrtin in type 2 diabetes with intrahepatic lipid infiltration

    International Nuclear Information System (INIS)

    Zhu Cuiying; Zhou Huan; Han Yuan; Ling Liqun; Huang Gang

    2008-01-01

    The aim of this study was to find and establish the serum marker which can reflect the degree of intrahepatic lipid infiltration in Type 2 diabetes patients and assess the therapeutic effect. It helps us to observe the improvement of intrahepatic lipid deposit under controlled serum glucose metabolism. Twenty-three Type 2 diabetes patients with obvious intrahepatic lipid infiltration diagnosed by CT scan were divided into two groups, one group took rosiglitazone orally (male: female 5:6), and the combined treatment group took rosiglitazone and metformin simultaneously (male: female 6:6), in daily therapeutic dose of 4 mg rosiglitazone and 2 g metformin for 24 weeks. Before and after treatment, we measured fasting serum glucose, glycated hemoglobin (GHb), insulin, insulin resistant index, plasma adiponectin, leptin, tumor necrotic factor α (TNF-α), ferrtin respectively. After the treatment, fasting serum glucose and GHb decreased obvious, especially the combined treatment group. Intrahepatic lipid content percent decreased, too, in both groups (the rosiglitazone group: 43.3±25.8 vs 29.1±18.7, P<0.01, the combined group: 43.4±21.8 vs 22.0±16.7, P<0.01). Plasma adiponectin and TNF-α had correlation to intrahepatic lipid contend percent change. Plasma adiponectin and TNF-α was obviously improved in the rosiglitazone group and the combined treatment group (Adiponectin: 11.96±7.3 vs 20.61±12.0 ng/mL, 12.76±6.7 vs 25.81±12.8 ng/mL; TNFα: 6.92±2.5 vs 5.89±1.9 pg/mL, 6.81±2.14 vs 5.45±2.0 pg/mL; P<0.01). In addition, serum ferrtin concentration decreased obviously, especially the combined treatment group (rosiglitazone group: 345±116 vs 288±71ng/mL, P<0.05, combined treatment group: 362±194 vs 258±109 ng/mL, P<0.01). It can be concluded that 1) rosiglitazone and metformin controls serum glucose metabolism and improves intrahepatic lipid infiltration by especially the combined treatment, 2) plasma adiponectic and TNF-α are effective markers to reflect

  17. The spleen as an extramedullary source of inflammatory cells responding to acetaminophen-induced liver injury

    International Nuclear Information System (INIS)

    Mandal, Mili; Gardner, Carol R.; Sun, Richard; Choi, Hyejeong; Lad, Sonali; Mishin, Vladimir; Laskin, Jeffrey D.; Laskin, Debra L.

    2016-01-01

    Macrophages have been shown to play a role in acetaminophen (APAP)-induced hepatotoxicity, contributing to both pro- and anti-inflammatory processes. In these studies, we analyzed the role of the spleen as an extramedullary source of hepatic macrophages. APAP administration (300 mg/kg, i.p.) to control mice resulted in an increase in CD11b + infiltrating Ly6G + granulocytic and Ly6G − monocytic cells in the spleen and the liver. The majority of the Ly6G + cells were also positive for the monocyte/macrophage activation marker, Ly6C, suggesting a myeloid derived suppressor cell (MDSC) phenotype. By comparison, Ly6G − cells consisted of 3 subpopulations expressing high, intermediate, and low levels of Ly6C. Splenectomy was associated with increases in mature (F4/80 + ) and immature (F4/80 − ) pro-inflammatory Ly6C hi macrophages and mature anti-inflammatory (Ly6C lo ) macrophages in the liver after APAP; increases in MDSCs were also noted in the livers of splenectomized (SPX) mice after APAP. This was associated with increases in APAP-induced expression of chemokine receptors regulating pro-inflammatory (CCR2) and anti-inflammatory (CX3CR1) macrophage trafficking. In contrast, APAP-induced increases in pro-inflammatory galectin-3 + macrophages were blunted in livers of SPX mice relative to control mice, along with hepatic expression of TNF-α, as well as the anti-inflammatory macrophage markers, FIZZ-1 and YM-1. These data demonstrate that multiple subpopulations of pro- and anti-inflammatory cells respond to APAP-induced injury, and that these cells originate from distinct hematopoietic reservoirs. - Highlights: • Multiple inflammatory cell subpopulations accumulate in the spleen and liver following acetaminophen (APAP) intoxication. • Splenectomy alters liver inflammatory cell populations responding to APAP. • Inflammatory cells accumulating in the liver in response to APAP originate from the spleen and the bone marrow. • Hepatotoxicity is reduced in

  18. The spleen as an extramedullary source of inflammatory cells responding to acetaminophen-induced liver injury

    Energy Technology Data Exchange (ETDEWEB)

    Mandal, Mili, E-mail: milimandal@gmail.com [Department of Pharmacology and Toxicology, Ernest Mario School of Pharmacy, Rutgers University, Piscataway, NJ 08854 (United States); Gardner, Carol R., E-mail: cgardner@pharmacy.rutgers.edu [Department of Pharmacology and Toxicology, Ernest Mario School of Pharmacy, Rutgers University, Piscataway, NJ 08854 (United States); Sun, Richard, E-mail: fishpower52@gmail.com [Department of Pharmacology and Toxicology, Ernest Mario School of Pharmacy, Rutgers University, Piscataway, NJ 08854 (United States); Choi, Hyejeong, E-mail: choi@eohsi.rutgers.edu [Department of Pharmacology and Toxicology, Ernest Mario School of Pharmacy, Rutgers University, Piscataway, NJ 08854 (United States); Lad, Sonali, E-mail: sonurose92@gmail.com [Department of Pharmacology and Toxicology, Ernest Mario School of Pharmacy, Rutgers University, Piscataway, NJ 08854 (United States); Mishin, Vladimir, E-mail: mishinv@eohsi.rutgers.edu [Department of Pharmacology and Toxicology, Ernest Mario School of Pharmacy, Rutgers University, Piscataway, NJ 08854 (United States); Laskin, Jeffrey D., E-mail: jlaskin@eohsi.rutgers.edu [Department of Environmental and Occupational Health, School of Public Health, Rutgers University, Piscataway, NJ 08854 (United States); Laskin, Debra L., E-mail: laskin@eohsi.rutgers.edu [Department of Pharmacology and Toxicology, Ernest Mario School of Pharmacy, Rutgers University, Piscataway, NJ 08854 (United States)

    2016-08-01

    Macrophages have been shown to play a role in acetaminophen (APAP)-induced hepatotoxicity, contributing to both pro- and anti-inflammatory processes. In these studies, we analyzed the role of the spleen as an extramedullary source of hepatic macrophages. APAP administration (300 mg/kg, i.p.) to control mice resulted in an increase in CD11b{sup +} infiltrating Ly6G{sup +} granulocytic and Ly6G{sup −} monocytic cells in the spleen and the liver. The majority of the Ly6G{sup +} cells were also positive for the monocyte/macrophage activation marker, Ly6C, suggesting a myeloid derived suppressor cell (MDSC) phenotype. By comparison, Ly6G{sup −} cells consisted of 3 subpopulations expressing high, intermediate, and low levels of Ly6C. Splenectomy was associated with increases in mature (F4/80{sup +}) and immature (F4/80{sup −}) pro-inflammatory Ly6C{sup hi} macrophages and mature anti-inflammatory (Ly6C{sup lo}) macrophages in the liver after APAP; increases in MDSCs were also noted in the livers of splenectomized (SPX) mice after APAP. This was associated with increases in APAP-induced expression of chemokine receptors regulating pro-inflammatory (CCR2) and anti-inflammatory (CX3CR1) macrophage trafficking. In contrast, APAP-induced increases in pro-inflammatory galectin-3{sup +} macrophages were blunted in livers of SPX mice relative to control mice, along with hepatic expression of TNF-α, as well as the anti-inflammatory macrophage markers, FIZZ-1 and YM-1. These data demonstrate that multiple subpopulations of pro- and anti-inflammatory cells respond to APAP-induced injury, and that these cells originate from distinct hematopoietic reservoirs. - Highlights: • Multiple inflammatory cell subpopulations accumulate in the spleen and liver following acetaminophen (APAP) intoxication. • Splenectomy alters liver inflammatory cell populations responding to APAP. • Inflammatory cells accumulating in the liver in response to APAP originate from the spleen and the

  19. Transcaval transjugular intrahepatic portosystemic shunt: preliminary clinical results

    Energy Technology Data Exchange (ETDEWEB)

    Lee, Kwang Hun; Lee, Do Yun; Won, Jong Yoon [Yonsei University College of Medicine, Seoul (Korea, Republic of); Park, Sang Joon [Hallym University College of Medicine, Seoul (Korea, Republic of); Kim, Jae Kyu; Yoon, Woong [Chonnam National University Hospital, Gwangju (Korea, Republic of)

    2003-03-01

    To determine the feasibility of transcaval transjugular intrahepatic portosystemic shunt (TIPS) creation in patients with unusual anatomy between the hepatic veins and portal bifurcation, and inaccessible or inadequate hepatic veins. Transcaval TIPS, performed in six patients, was indicated by active variceal bleeding (n=2), recurrent variceal bleeding (n=2), intractable ascites (n=1), and as a bridge to liver transplantation (n=1). The main reasons for transcaval rather than classic TIPS were the presence of an unusually acute angle between the hepatic veins and the level of the portal bifurcation (n=3), hepatic venous occlusion (n=2), and inadequate small hepatic veins (n=1). Technical and functional success was achieved in all patients. The entry site into liver parenchyma from the inferior vena cava was within 2 cm of the atriocaval junction. Procedure-related complications included the death of one patient due to hemoperitoneum despite the absence of contrast media spillage at tractography, and another suffered reversible hepatic encephalopathy. In patients with unusual anatomy between the hepatic veins and portal bifurcation, and inaccessible or inadequate hepatic veins, transcaval TIPS creation is feasible.

  20. Transcaval transjugular intrahepatic portosystemic shunt: preliminary clinical results

    International Nuclear Information System (INIS)

    Lee, Kwang Hun; Lee, Do Yun; Won, Jong Yoon; Park, Sang Joon; Kim, Jae Kyu; Yoon, Woong

    2003-01-01

    To determine the feasibility of transcaval transjugular intrahepatic portosystemic shunt (TIPS) creation in patients with unusual anatomy between the hepatic veins and portal bifurcation, and inaccessible or inadequate hepatic veins. Transcaval TIPS, performed in six patients, was indicated by active variceal bleeding (n=2), recurrent variceal bleeding (n=2), intractable ascites (n=1), and as a bridge to liver transplantation (n=1). The main reasons for transcaval rather than classic TIPS were the presence of an unusually acute angle between the hepatic veins and the level of the portal bifurcation (n=3), hepatic venous occlusion (n=2), and inadequate small hepatic veins (n=1). Technical and functional success was achieved in all patients. The entry site into liver parenchyma from the inferior vena cava was within 2 cm of the atriocaval junction. Procedure-related complications included the death of one patient due to hemoperitoneum despite the absence of contrast media spillage at tractography, and another suffered reversible hepatic encephalopathy. In patients with unusual anatomy between the hepatic veins and portal bifurcation, and inaccessible or inadequate hepatic veins, transcaval TIPS creation is feasible

  1. Intrahepatic porto-hepatic venous shunts in Rendu-Osler-Weber disease: imaging demonstration

    International Nuclear Information System (INIS)

    Matsumoto, Shunro; Mori, Hiromu; Yamada, Yasunari; Hayashida, Tomoko; Hori, Yuzo; Kiyosue, Hiro

    2004-01-01

    This study describes the imaging features of the intrahepatic portohepatic venous (PHV) shunt, which is a potential cause of portosystemic encephalopathy in Rendu-Osler-Weber disease. Six patients with Rendu-Osler-Weber disease (two men, four women; age range 42-73 years) were retrospectively studied. There were two from one family and three from another family. Of these patients, one was diagnosed with definitive portosystemic encephalopathy because of a psychiatric disorder. We retrospectively reviewed the radiological examinations, including abdominal angiography (n=6), three-phase dynamic helical computed tomography (CT; n=3), and conventional enhanced CT (n=1). In one patient, CT during angiography and CT angioportography were also performed. Evaluation was placed on the imaging features of intrahepatic PHV shunts. On angiography, intrahepatic PHV shunts showing multiple and small shunts <5 mm in diameter in an apparent network were detected in all patents. In two patients, a large shunt with a size of either 7 or 10 mm was associated. These intrahepatic PHV shunts were predominantly distributed in the peripheral parenchyma. Intrahepatic PHV shunts would be characterized by small and multiple shunts in an apparent network on the periphery with or without a large shunt. (orig.)

  2. Anatomy of the Portal Vein Bifurcation: Implication for Transjugular Intrahepatic Portal Systemic Shunts

    International Nuclear Information System (INIS)

    Kwok, Philip Chong-hei; Ng, Wai Fu; Lam, Christine Suk-yee; Tsui, Polly Po; Faruqi, Asma

    2003-01-01

    Purpose: The relationship of the portalvein bifurcation to the liver capsule in Asians, which is an important landmark for transjugular intrahepatic portosystemic shunt, has not previously been described. Methods: The anatomy of the portal vein bifurcation was studied in 70 adult Chinese cadavers; it was characterized as intrahepatic or extrahepatic. The length of the exposed portion of the right and left portal veins was measured when the bifurcation was extrahepatic. Results: The portal vein bifurcation was intrahepatic in 37 cadavers (53%) and extrahepatic in 33 cadavers (47%). The mean length of the right and left extrahepatic portal veins was 0.96 cm and 0.85 cm respectively.Both were less than or equal to 2 cm in 94% of the cadavers with extrahepatic bifurcation. There was no correlation between the presence of cirrhosis and the location of the portal vein bifurcation(p 1.0). There was no statistically significant difference in liver mass in cadavers with either extrahepatic or intrahepatic bifurcation (p =0.40). Conclusions: These findings suggest that fortransjugular intrahepatic portosystemic shunt placement, a portal vein puncture 2 cm from the bifurcation will be safe in most cases

  3. The human protooncogene product p33pim is expressed during fetal hematopoiesis and in diverse leukemias

    International Nuclear Information System (INIS)

    Amson, R.; Przedborski, S.; Telerman, A.; Sigaux, F.; Flandrin, G.; Givol, D.

    1989-01-01

    The authors measured the human pim-1 protooncogene (PIM) expression during fetal development and in hematopoietic malignancies. The data indicate that during human fetal hematopoiesis the 33-kDa pim product, p33pim, is highly expressed in the liver and the spleen. In contrast, a the adult stage it is only slightly expressed in circulating granulocytes. Out of 70 hematopoietic malignancies analyzed, 51 patients and 19 cell lines, p33pim was overexpressed in ∼ 30% of the samples, particularly in myeloid and lymphoid acute leukemias. This overexpression was unrelated to any stage of cellular differentiation and was not due to gene rearrangement or amplification. These results imply a physiological role of the pim-1 protooncogene during hematopoietic development and a deregulation in various leukemias

  4. COMP-Ang1 Recovers Hematopoiesis after Gamma-ray Irradiation in Mice

    International Nuclear Information System (INIS)

    Lee, Hae-June; Lee, Yoon-Jin; Kwon, Hee-Choong; Lee, Su-Jae; Bae, Sang-Woo; Lee, Yun-Sil; Kim, Sung-Ho

    2006-01-01

    It has been reported that angiopoietin (Ang1) enhanced the ability of hematopoietic stem cells (HSCs) to become quiescent and induced adhesion to bone, resulting in protection of the HSC compartment from myleosuppressive stress. Ang1 activates Tie2 receptors expressed on the vascular endothelial cells and HSCs expressing the receptor tyrosine kinase Tie2 are quiescent and antiapoptotic, comprise a sidepopulation of HSCs. COMP-Ang1 is generated protein, is a soluble, stable, and potent Ang1 variant by replacing the N-terminal portion of Ang1 with short coiled-coil domain of cartilage oligomeric matrix protein (COMP). Radio- and chemotherapy induces considerable damage of bone marrow. Herein, by using COMP-Ang1, we investigated the effect of COMP-Ang1 on restoration of hematopoiesis in bone marrow against radiation-induced myelosuppression

  5. Effect of intra-hepatic arterial infusion chemotherapy for patients with liver metastasis from breast cancer

    International Nuclear Information System (INIS)

    Liu Dezhong; Li Huai; Zeng Huiying; Yang Ling

    2001-01-01

    Objective: To evaluate the efficacy of intra-hepatic arterial infusion chemotherapy for patients with liver metastasis from breast cancer. Methods: 1993-1998 years, Thirty four patients with liver metastasis from breast cancer had received epi-adriamycin, cisplatin, mitomycin and 5-fluorouracil by intrahepatic arterial infusion chemotherapy. Twelve patients had received embolization. Results: Six patients (17.65%) had a complete response, 12 patients (35.29%) had a partial response. The overall response rate was 52.94%. Cumulative survival rates at 1, 2, 3 and 4 years were 56.90%, 25.00%, 5.00% and 5.00% respectively (Kaplan-Meier method). The median overall survival time was 11.5 months. Conclusion: Intra-hepatic arterial infusion chemotherapy is safe and effective for liver metastasis from breast cancer and should be the first choice of treatment for these patients

  6. Clinicopathological and prognostic significance of epithelial mesenchymal transition-related protein expression in intrahepatic cholangiocarcinoma

    Directory of Open Access Journals (Sweden)

    Yao X

    2012-10-01

    Full Text Available Xing Yao,1,* Xiang Wang,1,* Zishu Wang,2,* Licheng Dai,1 Guolei Zhang,1 Qiang Yan,1 Weimin Zhou11Huzhou Central Hospital, Zhejiang Huzhou, 2Department of Medical Oncology, First Affiliated Hospital, Bengbu Medical College, Anhui, People’s Republic of China *These authors contributed equally to this workBackground: The aim of this study was to examine the patterns of expression of epithelial-mesenchymal transition (EMT-related proteins in intrahepatic cholangiocarcinoma. The clinicopathological and prognostic value of these markers was also evaluated.Methods: We detected the expression status of three EMT-related proteins, ie, E-cadherin, vimentin, and N-cadherin, by immunohistochemistry in consecutive intrahepatic cholangiocarcinoma specimens from 96 patients.Results: The frequency of loss of the epithelial marker E-cadherin, and acquisition of mesenchymal markers, vimentin and N-cadherin, in intrahepatic cholangiocarcinoma was 43.8%, 37.5% and 57.3%, respectively. Altered expression of EMT markers was associated with aggressive tumor behavior, including lymph node metastasis, undifferentiated-type histology, advanced tumor stage, venous invasion, and shorter overall survival. Moreover, loss of E-cadherin was retained as an independent prognostic factor for patients with intrahepatic cholangiocarcinoma in multivariate analysis.Conclusion: Our results suggest that the EMT process is associated with tumor progression and a poor outcome in patients with intrahepatic cholangiocarcinoma, and inhibition of EMT might offer novel promising molecular targets for the treatment of affected patients.Keywords: intrahepatic cholangiocarcinoma, epithelial-mesenchymal transition, expression, prognosis, immunohistochemistry

  7. Intrahepatic cholangiocarcinoma prognostic determination using pre-operative serum C-reactive protein levels

    International Nuclear Information System (INIS)

    Lin, Zi-Ying; Liang, Zhen-Xing; Zhuang, Pei-Lin; Chen, Jie-Wei; Cao, Yun; Yan, Li-Xu; Yun, Jing-Ping; Xie, Dan; Cai, Mu-Yan

    2016-01-01

    Serum C-reactive protein (CRP), an acute inflammatory response biomarker, has been recognized as an indicator of malignant disease progression. However, the prognostic significance of CRP levels collected before tumor removal in intrahepatic cholangiocarcinoma requires further investigation. We sampled the CRP levels in 140 patients with intrahepatic cholangiocarcinoma who underwent hepatectomies with regional lymphadenectomies between 2006 and 2013. A retrospective analysis of the clinicopathological data was performed. We focused on the impact of serum CRP on the patients’ cancer-specific survival and recurrence-free survival rates. High levels of preoperative serum CRP were significantly associated with well-established clinicopathologic features, including gender, advanced tumor stage, and elevated carcinoembryonic antigen and carbohydrate antigen 19-9 levels (P < 0.05). Univariate analysis demonstrated a significant association between high levels of serum CRP and adverse cancer-specific survival (P = 0.001) and recurrence-free survival (P < 0.001). In patients with stage I/II intrahepatic cholangiocarcinoma, the serum CRP level was a prognostic indicator for cancer-specific survival. In patients with stage I/II or stage III/IV, the serum CRP level was a prognostic indicator for recurrence-free survival (P < 0.05). Additionally, multivariate analysis identified serum CRP level in intrahepatic cholangiocarcinoma as an independent prognostic factor (P < 0.05). We confirmed a significant association of elevated pre-operative CRP levels with poor clinical outcomes for the tested patients with intrahepatic cholangiocarcinoma. Our results indicate that the serum CRP level may represent a useful factor for patient stratification in intrahepatic cholangiocarcinoma management

  8. Intrahepatic cholangiocarcinoma--a rare indication for liver transplantation. Case report and review of the literature.

    Science.gov (United States)

    Hrehoreţ, D; Alexandrescu, S; Grigorie, R; Herlea, V; Anghel, R; Popescu, I

    2012-01-01

    While hepatocellular carcinoma is a common indication for liver transplantation, intrahepatic cholangiocarcinoma represents a controversial indication for this procedure, due to lower disease-free and overall survival rates achieved by liver transplantation in such patients. Hence, in the last years, few centers reported satisfactory survival rates after liver transplantation for cholangiocarcinoma, in highly selected groups of patients. Herein we present the clinicopathological characteristics, the pre- and postoperative management and the favorable outcome of a patient undergoing liver transplantation for an unresectable intrahepatic cholangiocarcinoma. We consider that reporting the patients with such favorable outcomes is useful, since collecting the data presented by different centers may contribute to identification of a selected group of patients with cholangiocarcinoma who may benefit from liver transplantation. A 62-year old female patient with a primary liver tumor developed on HBV liver cirrhosis, was admitted in our center for therapeutical management. Since preoperative work-up suggested that the tumor is an unresectable hepatocellular carcinoma (due to its location and underlying liver disease), we decided to perform liver transplantation. The pathological examination of the explanted liver revealed that the tumor was a stage I intrahepatic cholangiocarcinoma. The postoperative course was uneventful, and in present, 15 months after transplantation, the patient is alive, without recurrence. Liver transplantation may represent a valid therapeutical option in selected patients with intrahepatic cholangiocarcinoma. Patients with early stage intrahepatic cholangiocarcinomas unresectable due to the underlying liver cirrhosis seem to benefit mostly by liver transplantation. Further studies are needed to identify the favorable prognostic factors in order to select the most appropriate candidates for liver transplantation. The most suitable immunosuppressive

  9. Congenital hepatic arteriovenous fistula with intrahepatic portosystemic shunt and aortic stenosis in a dog

    International Nuclear Information System (INIS)

    Koide, K.; Koide, Y.; Wada, Y.; Nakaniwa, S.; Yamane, Y.

    2004-01-01

    Examination of a 2-month-old male golden retriever presented to the hospital revealed malnutrition, ascites, cardiac murmur and hyperammonemia. Identification of subaortic stenosis and hepatic arteriovenous fistula was made through ultrasonography and angiocardiography. In addition, intrasurgical mesenteric portography showed an intrahepatic portosystemic shunt. The dog did not show portal hypertension and secondary multiple extrahepatic portosystemic shunts. Surgical correction was attempted after medical treatment. The hepatic artery branch which was connected to the hepatic arteriovenous fistula was separated, and completely ligated using silk ligature. However, the separation of the intrahepatic shunt blood vessel was unsuccessful and the dog died 15 hr postoperatively

  10. Bile Duct Leaks from the Intrahepatic Biliary Tree: A Review of Its Etiology, Incidence, and Management

    Directory of Open Access Journals (Sweden)

    Sorabh Kapoor

    2012-01-01

    Full Text Available Bile leaks from the intrahepatic biliary tree are an important cause of morbidity following hepatic surgery and trauma. Despite reduction in mortality for hepatic surgery in the last 2 decades, bile leaks rates have not changed significantly. In addition to posted operative bile leaks, leaks may occur following drainage of liver abscess and tumor ablation. Most bile leaks from the intrahepatic biliary tree are transient and managed conservatively by drainage alone or endoscopic biliary decompression. Selected cases may require reoperation and enteric drainage or liver resection for management.

  11. Preoperative MR angiography evaluation of transjugular intrahepatic portosystemic shunt

    International Nuclear Information System (INIS)

    Guo Li; Yang Dakuan; Yuan Shuguang; Yan Dong; Wang Jiaping; Li Yingchun

    2010-01-01

    Objective: To discuss the application of MR angiography (MRA) in deciding the puncture points of transjugular intrahepatic portosystemic shunt (TIPS). Methods: Preoperative MRA was performed in 59 patients with portal hypertension (study group) in order to search for the causes of portal hypertension, to observe the patterns and route of the hepatic and portal veins and to measure the vascular diameter at the scheduled puncture site. MRA was also performed in 50 healthy subjects, which was served as the control group. The results were compared between two groups. Results: The diseases in the study group included simple cirrhosis (n=49), cirrhosis accompanied with hepatocellular carcinoma (n=4), pure portal vein thrombosis (n=3), splenic vein stenosis (n=1) and Budd-Chiari syndrome (n=2). In study group the type I, II and III of the hepatic vein classification were seen in 14, 39 and 12 cases respectively,while in control group in 12, 34 and 14 cases respectively. In study group, the right, middle and left hepatic vein which had the diameter larger enough for puncturing existed in 52, 40 and 28 cases respectively, while in control group in 46, 34 and 23 cases respectively. The safe point for puncture via the right and the left branch of the portal vein was located beyond the distance of (16.2 ± 3.1) mm and (14.2 ± 3.8) mm respectively. Conclusion: MRA is a valuable non-invasive examination, which is of great value in determining the causes of portal hypertension and in planning the puncturing sites before performing TIPS. (authors)

  12. Clinical results of the transjugular intrahepatic portosystemic shunt

    Energy Technology Data Exchange (ETDEWEB)

    Park, Auh Whan; Sim, Jae In; Ryeom, Hun Kyu; Kim, Yong Joo [Kyungpook National University Hospital, Daegu (Korea, Republic of)

    1994-04-15

    To evaluate the clinical results of transjugular intrahepatic portosystemic shunt(TIPS) for the control of variceal bleeding. TIPS creation was attempted in 23 patients with endoscopically confirmed variceal bleeding. Most patients had multiple episodes of bleeding in the past and have been treated with multiple endoscopic sclerotherapies. Pre-and post-procedural hepatic and portal vein pressures were measured. After creation of TIPS patients were followed up at regular intervals. TIPS has been successfully accomplished in 22 of 23 patients using Wallstent(n = 21) and Strecker stent(n = 1). Immediate bleeding control was achieved in all patients with shunt creation. No procedure-related complication was noted. Portal vein pressure was reduced from 30.7 {+-} 5.8 mmHg to 20.8 {+-} 4.7 mmHg. The mean pressure gradient of portosystemic shunt dropped from 22.8 {+-} 6.0 prior to TIPS to 12.2 {+-} 4.1 immediately after. During the follow-up period (6-556 days, mean; 10 months), seven patients died; progressive hepatic failure (n 4), variceal rebleeding (n = 2), and respiratory failure(n = 1). Hepatic encephalopathy after TIPS was noted in 7 patients(31.8%). Variceal rebleeding occurred in 3 patients(13.6%). The remaining 15 patients have survived an average of 11 months. This results suggest that TIPS is a safe and effective method for lowering portal pressure and controlling variceal bleeding. Furthermore if these initial results are encouraged by further long-term observation, TIPS could replace endoscopic and risky surgical intervention.

  13. Differential intrahepatic phospholipid zonation in simple steatosis and nonalcoholic steatohepatitis.

    Directory of Open Access Journals (Sweden)

    Julia Wattacheril

    Full Text Available Nonalcoholic fatty liver disease (NAFLD occurs frequently in a setting of obesity, dyslipidemia and insulin resistance, but the etiology of the disease, particularly the events favoring progression to nonalcoholic steatohepatitis (NASH as opposed to simple steatosis (SS, are not fully understood. Based on known zonation patterns in protein, glucose and lipid metabolism, coupled with evidence that phosphatidylcholine may play a role in NASH pathogenesis, we hypothesized that phospholipid zonation exists in liver and that specific phospholipid abundance and distribution may be associated with histologic disease. A survey of normal hepatic protein expression profiles in the Human Protein Atlas revealed pronounced zonation of enzymes involved in lipid utilization and storage, particularly those facilitating phosphatidylcholine (PC metabolism. Immunohistochemistry of obese normal, SS and NASH liver specimens with anti-phosphatidylethanomine N-methyltransferase (PEMT antibodies showed a progressive decrease in the zonal distribution of this PC biosynthetic enzyme. Phospholipid quantitation by liquid chromatography mass spectrometry (LC-MS in hepatic extracts of Class III obese patients with increasing NAFLD severity revealed that most PC species with 32, 34 and 36 carbons as well as total PC abundance was decreased with SS and NASH. Matrix assisted laser desorption ionization-imaging mass spectrometry (MALDI-IMS imaging revealed strong zonal distributions for 32, 34 and 36 carbon PCs in controls (minimal histologic findings and SS that was lost in NASH specimens. Specific lipid species such as PC 34:1 and PC 36:2 best illustrated this phenomenon. These findings suggest that phospholipid zonation may be associated with the presence of an intrahepatic proinflammatory phenotype and thus have broad implications in the etiopathogenesis of NASH.

  14. Flow cytometric minimal residual disease assessment of peripheral blood in acute lymphoblastic leukaemia patients has potential for early detection of relapsed extramedullary disease.

    Science.gov (United States)

    Keegan, Alissa; Charest, Karry; Schmidt, Ryan; Briggs, Debra; Deangelo, Daniel J; Li, Betty; Morgan, Elizabeth A; Pozdnyakova, Olga

    2018-03-27

    To evaluate peripheral blood (PB) for minimal residual disease (MRD) assessment in adults with acute lymphoblastic leukaemia (ALL). We analysed 76 matched bone marrow (BM) aspirate and PB specimens independently for the presence of ALL MRD by six-colour flow cytometry (FC). The overall rate of BM MRD-positivity was 24% (18/76) and PB was also MRD-positive in 22% (4/18) of BM-positive cases. We identified two cases with evidence of leukaemic cells in PB at the time of the extramedullary relapse that were interpreted as MRD-negative in BM. The use of PB MRD as a non-invasive method for monitoring of systemic relapse may have added clinical and diagnostic value in patients with high risk of extramedullary disease. © Article author(s) (or their employer(s) unless otherwise stated in the text of the article) 2018. All rights reserved. No commercial use is permitted unless otherwise expressly granted.

  15. Hematopoiesis on cellulose ester membranes (CEM). X. Effects of in vitro irradiation of stromal cells prior to application on CEM

    International Nuclear Information System (INIS)

    Knospe, W.H.; Husseini, S.G.

    1986-01-01

    Cellulose ester membranes (CEM) were coated with stromal cells from murine bone or bone marrow irradiated in vitro with 1000, 2000, or 4000 rad and then implanted i.p. in CAF1 mice for periods of six and 12 months. CEM coated with stromal cells from bone showed excellent regeneration of bone and hematopoiesis after 1000 rad in vitro irradiation. After 2000 rad, hematopoietic and bone regeneration was reduced by about 50%, and after 4000 rad it was completely absent in CEM coated with stromal cells from bone. CEM coated with stromal cells from bone marrow showed no regeneration of hematopoiesis or bone after 1000, 2000, and 4000 rad in vitro irradiation and residence i.p. for six and 12 months. These results indicate that regeneration of the hematopoietic microenvironment is dependent upon living stromal cells. A difference in radiation sensitivity is demonstrated between stromal cells from bone and from bone marrow

  16. Parallels between immune driven-hematopoiesis and T cell activation: 3 signals that relay inflammatory stress to the bone marrow

    Energy Technology Data Exchange (ETDEWEB)

    Libregts, Sten F.W.M.; Nolte, Martijn A., E-mail: m.nolte@sanquin.nl

    2014-12-10

    Quiescence, self-renewal, lineage commitment and differentiation of hematopoietic stem cells (HSCs) towards fully mature blood cells are a complex process that involves both intrinsic and extrinsic signals. During steady-state conditions, most hematopoietic signals are provided by various resident cells inside the bone marrow (BM), which establish the HSC micro-environment. However, upon infection, the hematopoietic process is also affected by pathogens and activated immune cells, which illustrates an effective feedback mechanism to hematopoietic stem and progenitor cells (HSPCs) via immune-mediated signals. Here, we review the impact of pathogen-associated molecular patterns (PAMPs), damage-associated molecular patterns (DAMPs), costimulatory molecules and pro-inflammatory cytokines on the quiescence, proliferation and differentiation of HSCs and more committed progenitors. As modulation of HSPC function via these immune-mediated signals holds an interesting parallel with the “three-signal-model” described for the activation and differentiation of naïve T-cells, we propose a novel “three-signal” concept for immune-driven hematopoiesis. In this model, the recognition of PAMPs and DAMPs will activate HSCs and induce proliferation, while costimulatory molecules and pro-inflammatory cytokines confer a second and third signal, respectively, which further regulate expansion, lineage commitment and differentiation of HSPCs. We review the impact of inflammatory stress on hematopoiesis along these three signals and we discuss whether they act independently from each other or that concurrence of these signals is important for an adequate response of HSPCs upon infection. - Highlights: • Inflammation and infection have a direct impact on hematopoiesis in the bone marrow. • We draw a striking parallel between immune-driven hematopoiesis and T cell activation. • We review how PAMPs and DAMPs, costimulation and cytokines influence HSPC function.

  17. Activation of adenosine receptors and inhibition of cyclooxygenases: two recent pharmacological approaches to modulation of radiation suppressed hematopoiesis

    International Nuclear Information System (INIS)

    Hofer, M.; Pospisil, M.; Vacek, A.; Hola, J.; Weiterova, L.; Streitova, D.; Znojil, V.

    2008-01-01

    Searching for drugs conforming to requirements for protection and/or treatment of radiation-induced damage belongs to the most important tasks of current radiobiology. In the Laboratory of Experimental Hematology, Institute of Biophysics, v.v.i., Academy of Sciences of the Czech Republic, Brno, Czech Republic, two original approaches for stimulation of radiation-suppressed hematopoiesis have been tested in recent years, namely activation of adenosine receptors and inhibition of cyclooxygenases. Non-selective activation of adenosine receptors, induced by combined administration of dipyridamole, a drug preventing adenosine uptake and supporting thus its extracellular receptor-mediated action, and adenosine monophosphate, an adenosine prodrug, has been found to stimulate hematopoiesis when the drugs were given either pre- or post-irradiation. When synthetic adenosine receptor agonists selective for individual adenosine receptor subtypes were tested, stimulatory effects in myelosuppressed mice have been found after administration of IB-MECA, a selective adenosine A3 receptor agonist. Non-selective cyclooxygenase inhibitors, inhibiting both cyclooxygenase-1 (COX-1) and cyclooxygenase-2 (COX-2), indomethacin, diclofenac, or flurbiprofen, have been observed to act positively on radiation-perturbed hematopoiesis in sublethally irradiated mice. However, their undesirable gastrointestinal side effects have been found to negatively influence survival of lethally irradiated animals. Recently tested selective COX-2 inhibitor meloxicam, preserving protective action of COX-1-synthesized prostaglandins in the gastrointestinal tissues, has been observed to retain the hematopoiesis-stimulating effects of non-selective cyclooxygenase inhibitors and to improve the survival of animals exposed to lethal radiation doses. These findings bear evidence for the possibility to use selective adenosine A3 receptor agonists and selective COX-2 inhibitors in human practice for treatment of

  18. Parallels between immune driven-hematopoiesis and T cell activation: 3 signals that relay inflammatory stress to the bone marrow

    International Nuclear Information System (INIS)

    Libregts, Sten F.W.M.; Nolte, Martijn A.

    2014-01-01

    Quiescence, self-renewal, lineage commitment and differentiation of hematopoietic stem cells (HSCs) towards fully mature blood cells are a complex process that involves both intrinsic and extrinsic signals. During steady-state conditions, most hematopoietic signals are provided by various resident cells inside the bone marrow (BM), which establish the HSC micro-environment. However, upon infection, the hematopoietic process is also affected by pathogens and activated immune cells, which illustrates an effective feedback mechanism to hematopoietic stem and progenitor cells (HSPCs) via immune-mediated signals. Here, we review the impact of pathogen-associated molecular patterns (PAMPs), damage-associated molecular patterns (DAMPs), costimulatory molecules and pro-inflammatory cytokines on the quiescence, proliferation and differentiation of HSCs and more committed progenitors. As modulation of HSPC function via these immune-mediated signals holds an interesting parallel with the “three-signal-model” described for the activation and differentiation of naïve T-cells, we propose a novel “three-signal” concept for immune-driven hematopoiesis. In this model, the recognition of PAMPs and DAMPs will activate HSCs and induce proliferation, while costimulatory molecules and pro-inflammatory cytokines confer a second and third signal, respectively, which further regulate expansion, lineage commitment and differentiation of HSPCs. We review the impact of inflammatory stress on hematopoiesis along these three signals and we discuss whether they act independently from each other or that concurrence of these signals is important for an adequate response of HSPCs upon infection. - Highlights: • Inflammation and infection have a direct impact on hematopoiesis in the bone marrow. • We draw a striking parallel between immune-driven hematopoiesis and T cell activation. • We review how PAMPs and DAMPs, costimulation and cytokines influence HSPC function

  19. Intrahepatic upregulation of MRTF-A signaling contributes to increased hepatic vascular resistance in cirrhotic rats with portal hypertension.

    Science.gov (United States)

    Zheng, Lei; Qin, Jun; Sun, Longci; Gui, Liang; Zhang, Chihao; Huang, Yijun; Deng, Wensheng; Huang, An; Sun, Dong; Luo, Meng

    2017-06-01

    Portal hypertension in cirrhosis is mediated, in part, by increased intrahepatic resistance, reflecting massive structural changes associated with fibrosis and intrahepatic vasoconstriction. Activation of the Rho/MRTF/SRF signaling pathway is essential for the cellular regulatory network of fibrogenesis. The aim of this study was to investigate MRTF-A-mediated regulation of intrahepatic fibrogenesis in cirrhotic rats. Portal hypertension was induced in rats via an injection of CCl 4 oil. Hemodynamic measurements were obtained using a polyethylene PE-50 catheter and pressure transducers. Expression of hepatic fibrogenesis was measured using histological staining. Expression of protein was measured using western blotting. Upregulation of MRTF-A protein expression in the livers of rats with CCl 4 -induced cirrhosis was relevant to intrahepatic resistance and hepatic fibrogenesis in portal hypertensive rats with increased modeling time. Inhibition of MRTF-A by CCG-1423 decelerated hepatic fibrosis, decreased intrahepatic resistance and portal pressure, and alleviated portal hypertension. Increased intrahepatic resistance in rats with CCl 4 -induced portal hypertension is associated with an upregulation of MRTF-A signaling. Inhibition of this pathway in the liver can decrease hepatic fibrosis and intrahepatic resistance, as well as reduce portal pressure in cirrhotic rats with CCl 4 -induced portal hypertension. Copyright © 2016 Elsevier Masson SAS. All rights reserved.

  20. Expression profile analysis of aorta-gonad-mesonephros region-derived stromal cells reveals genes that regulate hematopoiesis

    International Nuclear Information System (INIS)

    Nagao, Kenji; Ohta, Takayuki; Hinohara, Atsushi; Tahara, Tomoyuki; Hagiwara, Tetsuya; Maeda, Yoshitake; Yoneya, Takashi; Sohma, Yoshiaki; Heike, Toshio; Nakahata, Tatsutoshi; Inagaki, Yoshimasa; Nishikawa, Mitsuo

    2008-01-01

    The aorta-gonad-mesonephros (AGM) region is involved in the generation and maintenance of the first definitive hematopoietic stem cells (HSCs). A mouse AGM-derived cell line, AGM-S3, was shown to support the development of HSCs. To elucidate the molecular mechanisms regulating early hematopoiesis, we obtained subclones from AGM-S3, one of which was hematopoiesis supportive (S3-A9) and the other one of which was non-supportive (S3-A7), and we analyzed their gene expression profiles by gene chip analysis. In the present study, we found that Glypican-1 (GPC1) was highly expressed in the supportive subclone AGM-S3-A9. Over-expression of GPC1 in non-supportive cells led to the proliferation of progenitor cells in human cord blood when cocultured with the transfected-stromal cells. Thus, GPC1 may have an important role in the establishment of a microenvironment that supports early events in hematopoiesis

  1. Many layers of embryonic hematopoiesis: new insights into B-cell ontogeny and the origin of hematopoietic stem cells.

    Science.gov (United States)

    Hadland, Brandon; Yoshimoto, Momoko

    2018-04-01

    In adult hematopoiesis, the hematopoietic stem cell (HSC) sits at the top of a hierarchy of hematopoietic progenitors responsible for generating the diverse repertoire of blood and immune cells. During embryonic development, however, the initial waves of hematopoiesis provide the first functioning blood cells of the developing embryo, such as primitive erythrocytes arising in the yolk sac, independently of HSCs. In the field of developmental immunology, it has been recognized that some components of the immune system, such as B-1a lymphocytes, are uniquely produced during the embryonic and neonatal period, suggesting a "layered" development of immunity. Several recent studies have shed new light on the developmental origin of the layered immune system, suggesting complex and sometimes multiple contributions to unique populations of innate-like immune cells from both fetal HSCs and earlier HSC-independent progenitors. In this review, we will attempt to synthesize these studies to provide an integrated model of developmental hematopoiesis and layered immunity that may offer new insights into the origin of HSCs. Copyright © 2018 ISEH – Society for Hematology and Stem Cells. Published by Elsevier Inc. All rights reserved.

  2. Vascular Endothelial Growth Factor and Angiopoietin-1 Stimulate Postnatal Hematopoiesis by Recruitment of Vasculogenic and Hematopoietic Stem Cells

    Science.gov (United States)

    Hattori, Koichi; Dias, Sergio; Heissig, Beate; Hackett, Neil R.; Lyden, David; Tateno, Masatoshi; Hicklin, Daniel J.; Zhu, Zhenping; Witte, Larry; Crystal, Ronald G.; Moore, Malcolm A.S.; Rafii, Shahin

    2001-01-01

    Tyrosine kinase receptors for angiogenic factors vascular endothelial growth factor (VEGF) and angiopoietin-1 (Ang-1) are expressed not only by endothelial cells but also by subsets of hematopoietic stem cells (HSCs). To further define their role in the regulation of postnatal hematopoiesis and vasculogenesis, VEGF and Ang-1 plasma levels were elevated by injecting recombinant protein or adenoviral vectors expressing soluble VEGF165, matrix-bound VEGF189, or Ang-1 into mice. VEGF165, but not VEGF189, induced a rapid mobilization of HSCs and VEGF receptor (VEGFR)2+ circulating endothelial precursor cells (CEPs). In contrast, Ang-1 induced delayed mobilization of CEPs and HSCs. Combined sustained elevation of Ang-1 and VEGF165 was associated with an induction of hematopoiesis and increased marrow cellularity followed by proliferation of capillaries and expansion of sinusoidal space. Concomitant to this vascular remodeling, there was a transient depletion of hematopoietic activity in the marrow, which was compensated by an increase in mobilization and recruitment of HSCs and CEPs to the spleen resulting in splenomegaly. Neutralizing monoclonal antibody to VEGFR2 completely inhibited VEGF165, but not Ang-1–induced mobilization and splenomegaly. These data suggest that temporal and regional activation of VEGF/VEGFR2 and Ang-1/Tie-2 signaling pathways are critical for mobilization and recruitment of HSCs and CEPs and may play a role in the physiology of postnatal angiogenesis and hematopoiesis. PMID:11342585

  3. SELENIUM EFFECT UPON THE RATS' HEMATOPOIESIS IN THE SUBACUTE BENZENE INTOXICATION

    Directory of Open Access Journals (Sweden)

    Pavle Randjelovic

    2001-03-01

    Full Text Available The antioxidants (selenium, vitamins C and E stabilize the cell membrane andprotect the cells from the action of free radicals. On the other hand, the antioxidantsreduce the effects of chemical and physical agenls. Bcsidcs, selenium has animportant role in Transporting electrons in the mitochondria and il is necessary for iheglulathione peroxidase function in the protection from apoplhosis. Benzene is auniversal solvent and has a wide application in chemical industry. Its toxicity ismanifested in the damages done to the central nervous syslem, liver, kidneys andhematopoiesis system. Tn this experiment the Wistar rats were used that wereclassified in three experimental groups regarding the quantity of the receivedselenium. Each group comprised ten animals of both sexes and after two weeks'treatment by selenium of 4,8 and 16 mcg, the animals had received benzene byinlraperiloneal administration in the dose of 1,2 ml/kg of the body weight. Thecounting of the shaped blood elements was done after the selenium pretreatment andafter the benzene intoxication. The obtained results poinl to increased number of alithe blood elements after the selenium pretreatment while after benzene adminislrationthere was a drastic drop of the number of erylhrocyles and leukocytes alongwith moderate lhrombocylopenia. After the sacrifice, Ihe hematopoiesis organs weretaken. The hislological findings of the bone marrow show the emergence ofdisturbances, especially of the red sort cells as well as an obvious fat degeneration which is particularly conspicuous in the second and third groups of animals. Therewas also some damage done to the spleen, especially of its red pulp along with thepresence of a greater number of fresh erythrocytes in the second and third groups.Only the changes were more drastic in the third group. The obtained results show thatselenium in higher concentrations increases the number of erytrocytes andleukocytes which proves that it stimulates highly

  4. Spontaneous perforation of gallbladder with intrahepatic biloma formation: sonographic signs and correlation with computed tomography

    Energy Technology Data Exchange (ETDEWEB)

    Hollanda, Erick Sabbagh de; Torres, Ulysses dos Santos; Gual, Fabiana; Oliveira, Eduardo Portela de; Cardoso, Luciana Vargas; Criado, Divanei Aparecida Bottaro, E-mail: usantor@yahoo.com.br [Faculdade de Medicina de Sao Jose do Rio Preto (Famerp), SP (Brazil). Hospital de Base

    2013-09-15

    Spontaneous perforation of gallbladder is a severe and infrequent complication of acute cholecystitis that requires early and accurate diagnosis. Concomitant development of intrahepatic collections is rarely observed in such cases. The present report emphasizes the relevance of imaging studies in this setting, describing the typical sonographic and tomographic findings for the diagnosis of such condition. (author)

  5. Intrahepatic hematoma: hepatic lesion in a newborn with high {alpha}-fetoprotein level

    Energy Technology Data Exchange (ETDEWEB)

    Lam, Chiu Ying Flora; Chan, Kui Fai; Fan, Tsz Wo; Kwok, Chong Hei Philip; Chan, Chi Hum Susan; Tsang, Tsz Kan [Queen Elizabeth Hospital, Department of Radiology and Imaging, Hong Kong (China)

    2005-11-01

    Hepatic hematomas are relatively common in fetuses and neonates; most are subcapsular in location. Sometimes their imaging features can be non-specific, so differentiation from other aggressive lesions like hepatoblastoma can be difficult, especially if there is a concurrent high {alpha}-fetoprotein level. We report a case of intrahepatic hematoma with a rising {alpha}-fetoprotein level. (orig.)

  6. Association between biliary complications and technique of hilar division (extrahepatic vs. intrahepatic in major liver resections

    Directory of Open Access Journals (Sweden)

    Gamaletsos Evangelos

    2006-08-01

    Full Text Available Abstract Background Division of major vascular and biliary structures during major hepatectomies can be carried out either extrahepatically at the porta hepatic or intrahepatically during the parenchymal transection. In this retrospective study we test the hypothesis that the intrahepatic technique is associated with less early biliary complications. Methods 150 patients who underwent major hepatectomies were retrospectively allocated into an intrahepatic group (n = 100 and an extrahepatic group (n = 50 based on the technique of hilar division. The two groups were operated by two different surgical teams, each one favoring one of the two approaches for hilar dissection. Operative data (warm ischemic time, operative time, blood loss, biliary complications, morbidity and mortality rates were analyzed. Results In extrahepatic patients, operative time was longer (245 ± 50 vs 214 ± 38 min, p Conclusion Intrahepatic hilar division is as safe as extrahepatic hilar division in terms of intraoperative blood requirements, morbidity and mortality. The extrahepatic technique is associated with more severe bile leaks and biliary injuries.

  7. Canine intrahepatic vasculature: is a functional anatomic model relevant to the dog?

    Science.gov (United States)

    Hall, Jon L; Mannion, Paddy; Ladlow, Jane F

    2015-01-01

    To clarify canine intrahepatic portal and hepatic venous system anatomy using corrosion casting and advanced imaging and to devise a novel functional anatomic model of the canine liver to investigate whether this could help guide the planning and surgical procedure of partial hepatic lobectomy and interventional radiological procedures. Prospective experimental study. Adult Greyhound cadavers (n = 8). Portal and hepatic vein corrosion casts of healthy livers were assessed using computed tomography (CT). The hepatic lobes have a consistent hilar hepatic and portal vein supply with some variation in the number of intrahepatic branches. For all specimens, 3 surgically resectable areas were identified in the left lateral lobe and 2 surgically resectable areas were identified in the right medial lobe as defined by a functional anatomic model. CT of detailed acrylic casts allowed complex intrahepatic vascular relationships to be investigated and compared with previous studies. Improving understanding of the intrahepatic vascular supply facilitates interpretation of advanced images in clinical patients, the planning and performance of surgical procedures, and may facilitate interventional vascular procedures, such as intravenous embolization of portosystemic shunts. Functional division of the canine liver similar to human models is possible. The left lateral and right medial lobes can be consistently divided into surgically resectable functional areas and partial lobectomies can be performed following a functional model; further study in clinically affected animals would be required to investigate the relevance of this functional model in the dog. © Copyright 2014 by The American College of Veterinary Surgeons.

  8. Efficacy and safety of ursodeoxycholic acid versus cholestyramine in intrahepatic cholestasis of pregnancy

    NARCIS (Netherlands)

    Kondrackiene, Jurate; Beuers, Ulrich; Kupcinskas, Limas

    2005-01-01

    BACKGROUND & AIMS: Treatment of intrahepatic cholestasis of pregnancy with ursodeoxycholic acid appears promising, but data are limited so far. The aim of this randomized study was to evaluate the efficacy and safety of ursodeoxycholic acid in comparison with cholestyramine. METHODS: Eighty-four

  9. Autotaxin activity has a high accuracy to diagnose intrahepatic cholestasis of pregnancy

    NARCIS (Netherlands)

    Kremer, Andreas E.; Bolier, Ruth; Dixon, Peter H.; Geenes, Victoria; Chambers, Jenny; Tolenaars, Dagmar; Ris-Stalpers, Carrie; Kaess, Bernhard M.; Rust, Christian; van der Post, Joris A.; Williamson, Catherine; Beuers, Ulrich; Oude Elferink, Ronald P. J.

    2015-01-01

    Intrahepatic cholestasis of pregnancy (ICP) is defined by pruritus, elevated total fasting serum bile salts (TBS) and transaminases, and an increased risk of adverse fetal outcome. An accurate diagnostic marker is needed. Increased serum autotaxin correlates with cholestasis-associated pruritus. We

  10. Calorie restricted high protein diets downregulate lipogenesis and lower intrahepatic triglyceride concentrations in male rats

    Science.gov (United States)

    The purpose of this investigation was to assess the influence of calorie restriction (CR) alone, higher-protein/lower-carbohydrate intake alone, and combined CR higher-protein/lower-carbohydrate intake on glucose homeostasis, hepatic de novo lipogenesis (DNL), and intrahepatic triglycerides. Twelve-...

  11. Complications of percutaneous transhepatic biliary drainage in patients with dilated and nondilated intrahepatic bile ducts

    International Nuclear Information System (INIS)

    Weber, Andreas; Gaa, Jochen; Rosca, Bogdan; Born, Peter; Neu, Bruno; Schmid, Roland M.; Prinz, Christian

    2009-01-01

    Percutaneous transhepatic biliary drainage (PTBD) have been described as an effective technique to obtain biliary access. Between January 1996 and December 2006, a total of 419 consecutive patients with endoscopically inaccessible bile ducts underwent PTBD. The current retrospective study evaluated success and complication rates of this invasive technique. PTBD was successful in 410/419 patients (97%). The success rate was equal in patients with dilated and nondilated bile ducts (p = 0.820). In 39/419 patients (9%) procedure related complications could be observed. Major complications occurred in 17/419 patients (4%). Patients with nondilated intrahepatic bile ducts had significantly higher complication rates compared to patients with dilated intrahepatic bile ducts (14.5% vs. 6.9%, respectively [p = 0.022]). Procedure related deaths were observed in 3 patients (0.7%). In conclusion, percutaneous transhepatic biliary drainage is an effective procedure in patients with dilated and nondilated intrahepatic bile ducts. However, patients with nondilated intrahepatic bile ducts showed a higher risk for procedure related complications.

  12. Deep venous thrombosis and agenesis of the intrahepatic segment of inferior vena cava

    International Nuclear Information System (INIS)

    Velasco, J.; Fernandez, M.M.; Manzanares, R.; Hernando, A.

    1997-01-01

    We present a case of agenesis of the intrahepatic segment of inferior vena cava (IVC) with drainage through the azygos and hemiazygos systems. The presenting sign was deep venous thrombosis (DVT) in both lower extremities. The different imaging studies led to the diagnosis of both the congenital and acquired venous abnormalities, which are discussed. (Author) 14 refs

  13. Predictors of premature delivery in patients with intrahepatic cholestasis of pregnancy

    NARCIS (Netherlands)

    Kondrackiene, Jurate; Beuers, Ulrich; Zalinkevicius, Rimantas; Tauschel, Horst-Dietmar; Gintautas, Vladas; Kupcinskas, Limas

    2007-01-01

    AIM: To evaluate the predictive value of clinical symptoms and biochemical parameters for prematurity in intrahepatic cholestasis of pregnancy (ICP). METHODS: Sixty symptomatic patients with ICP were included in this retrospective analysis. Preterm delivery was defined as delivery before 37 wk

  14. Usefulness of 18F-FDG PET in intrahepatic cholangiocarcinoma

    International Nuclear Information System (INIS)

    Kim, Young-Jin; Yun, Mijin; Lee, Jong Doo; Lee, Woo Jung; Kim, Kyung Sik

    2003-01-01

    Surgical resection is the only curative treatment strategy for intrahepatic cholangiocarcinoma (CC). Therefore, accurate staging is essential for appropriate management of patients with CC. We assessed the usefulness of 2-[ 18 F]fluoro-2-deoxy-d-glucose (FDG) positron emission tomography (PET) in the staging of CC. We undertook a retrospective review of FDG PET images in 21 patients (10 female, 11 male; mean age 57 years) diagnosed with CC. Ten patients had hilar CC and 11, peripheral CC. Patients underwent abdominal magnetic resonance imaging (MRI) (n=20) and computed tomography (CT) (n=12) for the evaluation of primary tumours, and chest radiography and whole-body bone scintigraphy for work-up of distant metastases. For semi-quantitative analysis, the maximum voxel standardised uptake value (SUV max ) was obtained from the primary tumour. All peripheral CCs showed intensely increased FDG uptake, and some demonstrated ring-shaped uptake corresponding to peripheral rim enhancement on CT and/or MRI. In nine of the ten patients, hilar CCs demonstrated increased FDG uptake of a focal nodular or linear branching appearance. The remaining case was false negative on FDG PET. One patient with a false negative result on MRI demonstrated increased uptake on FDG PET. Among the ten hilar CCs, FDG uptake was intense in only two patients and was slightly higher than that of the hepatic parenchyma in the remaining patients. For the detection of lymph node metastasis, FDG PET and CT/MRI were concordant in 16 patients, and discordant in five (FDG PET was positive in three, and CT and MRI in two). FDG PET identified unsuspected distant metastases in four of the 21 patients; all of these patients had peripheral CC. FDG PET is useful in detecting the primary lesion in both hilar and peripheral CC and is of value in discovering unsuspected distant metastases in patients with peripheral CC. FDG PET could be useful in cases of suspected hilar CC with non-confirmatory biopsy and

  15. Radiation Exposure in Transjugular Intrahepatic Portosystemic Shunt Creation

    Energy Technology Data Exchange (ETDEWEB)

    Miraglia, Roberto, E-mail: rmiraglia@ismett.edu; Maruzzelli, Luigi, E-mail: lmaruzzelli@ismett.edu; Cortis, Kelvin, E-mail: kelvincortis@ismett.edu [Mediterranean Institute for Transplantation and Advanced Specialized Therapies (ISMETT), Radiology Service, Department of Diagnostic and Therapeutic Services (Italy); D’Amico, Mario, E-mail: mdamico@ismett.edu [University of Palermo, Department of Radiology (Italy); Floridia, Gaetano, E-mail: gfloridia@ismett.edu; Gallo, Giuseppe, E-mail: ggallo@ismett.edu; Tafaro, Corrado, E-mail: ctafaro@ismett.edu; Luca, Angelo, E-mail: aluca@ismett.edu [Mediterranean Institute for Transplantation and Advanced Specialized Therapies (ISMETT), Radiology Service, Department of Diagnostic and Therapeutic Services (Italy)

    2016-02-15

    PurposeTransjugular intrahepatic portosystemic shunt (TIPS) creation is considered as being one of the most complex procedures in abdominal interventional radiology. Our aim was twofold: quantification of TIPS-related patient radiation exposure in our center and identification of factors leading to reduced radiation exposure.Materials and methodsThree hundred and forty seven consecutive patients underwent TIPS in our center between 2007 and 2014. Three main procedure categories were identified: Group I (n = 88)—fluoroscopic-guided portal vein targeting, procedure done in an image intensifier-based angiographic system (IIDS); Group II (n = 48)—ultrasound-guided portal vein puncture, procedure done in an IIDS; and Group III (n = 211)—ultrasound-guided portal vein puncture, procedure done in a flat panel detector-based system (FPDS). Radiation exposure (dose-area product [DAP], in Gy cm{sup 2} and fluoroscopy time [FT] in minutes) was retrospectively analyzed.ResultsDAP was significantly higher in Group I (mean ± SD 360 ± 298; median 287; 75th percentile 389 Gy cm{sup 2}) as compared to Group II (217 ± 130; 178; 276 Gy cm{sup 2}; p = 0.002) and Group III (129 ± 117; 70; 150 Gy cm{sup 2}p < 0.001). The difference in DAP between Groups II and III was also significant (p < 0.001). Group I had significantly longer FT (25.78 ± 13.52 min) as compared to Group II (20.45 ± 10.87 min; p = 0.02) and Group III (19.76 ± 13.34; p < 0.001). FT was not significantly different between Groups II and III (p = 0.73).ConclusionsReal-time ultrasound-guided targeting of the portal venous system during TIPS creation results in a significantly lower radiation exposure and reduced FT. Further reduction in radiation exposure can be achieved through the use of modern angiographic units with FPDS.

  16. Gelatin sponge particle embolization of spontaneously putured intrahepatic arterial aneurysms in a patient with polyarteritis nodosa: A case report

    Energy Technology Data Exchange (ETDEWEB)

    Seol, Myung Jin; Noh, Kyung Hee; Kim, Young Jun; Jeon, Doo Sung [Dept. of Radiology, Presbyterian Medical Center, Jeonju (Korea, Republic of)

    2017-01-15

    Multiple intrahepatic arterial aneurysms and spontaneous aneurysmal rupture associated with polyarteritis nodosa leading to hemoperitoneum are extremely rare occurrences, but the conditions can be life-threatening if left untreated because of the risk of massive hemorrhage. We report a case of a high-risk surgical patient with polyarteritis nodosa complicated by spontaneous rupture of multiple intrahepatic arterial aneurysms. He was initially treated with emergency gelatin sponge particle embolization, followed by maintenance steroid treatment. Complete resolution of intrahepatic arterial aneurysms was observed at follow-up.

  17. Clinical experience in treatment of complex intrahepatic bile duct stones by regular hepatectomy

    Directory of Open Access Journals (Sweden)

    WU Xiao

    2016-09-01

    Full Text Available Objective To investigate the clinical effect of regular hepatectomy in patients with complex intrahepatic bile duct stones. Methods A retrospective analysis was performed for the clinical data of 98 patients with complex intrahepatic bile duct stones who were treated in our hospital from January to December, 2013. The distribution characteristics of intrahepatic bile duct stones, clinical manifestations, extent of operation, time of operation, complications, and surgical outcome were analyzed. Results All the 98 patients completed regular hepatectomy. Of all patients, 37 underwent choledocholithotomy with T-tube drainage and segmental hepatectomy, 6 underwent resection of the left lateral lobe of the liver, 1 underwent left hemihepatectomy, 7 underwent resection of a single hepatic segment in the right lobe of the liver combined with segmental hepatectomy, 45 underwent combined segmental hepatectomy of the left and right lobes of the liver, and 2 underwent biliary-enteric basin anastomosis after hilar bile duct reconstruction. No patients died during the perioperative period. The mean time of operation was 65.0±5.0 min, and the mean intraoperative blood loss was 83.0±6.2 ml. No patients experienced residual stones after surgery. Of all patients, 5 (5.1% experienced complications, among whom 3 experienced bile leakage and 2 experienced blood exudation on the surface of the wound in the liver; 3 patients were diagnosed with intrahepatic cholangiocarcinoma by postoperative pathological examination. All the patients for followed up for 5 years, and 11 (11.2% experienced recurrence of stones after surgery. Conclusion Regular hepatectomy is safe and effective in the treatment of complex intrahepatic bile duct stones, with few complications and a low recurrence rate of stones. Therefore, it holds promise for wide clinical application.

  18. Metformin improves defective hematopoiesis and delays tumor formation in Fanconi anemia mice.

    Science.gov (United States)

    Zhang, Qing-Shuo; Tang, Weiliang; Deater, Matthew; Phan, Ngoc; Marcogliese, Andrea N; Li, Hui; Al-Dhalimy, Muhsen; Major, Angela; Olson, Susan; Monnat, Raymond J; Grompe, Markus

    2016-12-15

    Fanconi anemia (FA) is an inherited bone marrow failure disorder associated with a high incidence of leukemia and solid tumors. Bone marrow transplantation is currently the only curative therapy for the hematopoietic complications of this disorder. However, long-term morbidity and mortality remain very high, and new therapeutics are badly needed. Here we show that the widely used diabetes drug metformin improves hematopoiesis and delays tumor formation in Fancd2 -/- mice. Metformin is the first compound reported to improve both of these FA phenotypes. Importantly, the beneficial effects are specific to FA mice and are not seen in the wild-type controls. In this preclinical model of FA, metformin outperformed the current standard of care, oxymetholone, by improving peripheral blood counts in Fancd2 -/- mice significantly faster. Metformin increased the size of the hematopoietic stem cell compartment and enhanced quiescence in hematopoietic stem and progenitor cells. In tumor-prone Fancd2 -/- Trp53 +/- mice, metformin delayed the onset of tumors and significantly extended the tumor-free survival time. In addition, we found that metformin and the structurally related compound aminoguanidine reduced DNA damage and ameliorated spontaneous chromosome breakage and radials in human FA patient-derived cells. Our results also indicate that aldehyde detoxification might be one of the mechanisms by which metformin reduces DNA damage in FA cells. © 2016 by The American Society of Hematology.

  19. Regulation of embryonic hematopoiesis by a cytokine-inducible SH2 domain homolog in zebrafish.

    Science.gov (United States)

    Lewis, Rowena S; Noor, Suzita M; Fraser, Fiona W; Sertori, Robert; Liongue, Clifford; Ward, Alister C

    2014-06-15

    Cytokine-inducible SH2 domain-containing protein (CISH), a member of the suppressor of cytokine signaling family of negative feedback regulators, is induced by cytokines that activate STAT5 and can inhibit STAT5 signaling in vitro. However, demonstration of a definitive in vivo role for CISH during development has remained elusive. This study employed expression analysis and morpholino-mediated knockdown in zebrafish in concert with bioinformatics and biochemical approaches to investigate CISH function. Two zebrafish CISH paralogs were identified, cish.a and cish.b, with high overall conservation (43-46% identity) with their mammalian counterparts. The cish.a gene was maternally derived, with transcripts present throughout embryogenesis, and increasing at 4-5 d after fertilization, whereas cish.b expression commenced at 8 h after fertilization. Expression of cish.a was regulated by the JAK2/STAT5 pathway via conserved tetrameric STAT5 binding sites (TTCN3GAA) in its promoter. Injection of morpholinos targeting cish.a, but not cish.b or control morpholinos, resulted in enhanced embryonic erythropoiesis, myelopoiesis, and lymphopoiesis, including a 2- 3-fold increase in erythrocytic markers. This occurred concomitantly with increased activation of STAT5. This study indicates that CISH functions as a conserved in vivo target and regulator of STAT5 in the control of embryonic hematopoiesis. Copyright © 2014 by The American Association of Immunologists, Inc.

  20. Engineered Murine HSCs Reconstitute Multi-lineage Hematopoiesis and Adaptive Immunity

    Directory of Open Access Journals (Sweden)

    Yi-Fen Lu

    2016-12-01

    Full Text Available Hematopoietic stem cell (HSC transplantation is curative for malignant and genetic blood disorders, but is limited by donor availability and immune-mismatch. Deriving HSCs from patient-matched embryonic/induced-pluripotent stem cells (ESCs/iPSCs could address these limitations. Prior efforts in murine models exploited ectopic HoxB4 expression to drive self-renewal and enable multi-lineage reconstitution, yet fell short in delivering robust lymphoid engraftment. Here, by titrating exposure of HoxB4-ESC-HSC to Notch ligands, we report derivation of engineered HSCs that self-renew, repopulate multi-lineage hematopoiesis in primary and secondary engrafted mice, and endow adaptive immunity in immune-deficient recipients. Single-cell analysis shows that following engraftment in the bone marrow niche, these engineered HSCs further specify to a hybrid cell type, in which distinct gene regulatory networks of hematopoietic stem/progenitors and differentiated hematopoietic lineages are co-expressed. Our work demonstrates engineering of fully functional HSCs via modulation of genetic programs that govern self-renewal and lineage priming.

  1. The impact of Mir-9 regulation in normal and malignant hematopoiesis

    Directory of Open Access Journals (Sweden)

    Abbas Khosravi

    2018-03-01

    Full Text Available MicroRNA-9 (MiR-9 dysregulation has been observed in various cancers. Recently, MiR-9 is considered to have a part in hematopoiesis and hematologic malignancies. However, its importance in blood neoplasms is not yet well defined. Thus, this study was conducted in order to assess the significance of MiR-9 role in the development of hematologic neoplasia, prognosis, and treatment approaches. We have shown that a large number of MiR-9 targets (such as FOXOs, SIRT1, CCND1, ID2, CCNG1, Ets, and NFkB play essential roles in leukemogenesis and that it is overexpressed in different leukemias. Our findings indicated MiR-9 downregulation in a majority of leukemias. However, its overexpression was reported in patients with dysregulated MiR-9 controlling factors (such as MLLr. Additionally, prognostic value of MiR-9 has been reported in some types of leukemia. This study generally emphasizes on the critical role of MiR-9 in hematologic malignancies as a prognostic factor and a therapeutic target.

  2. Protective effects of bacterial endotoxin in reconstruction of hematopoiesis after irradiation

    Energy Technology Data Exchange (ETDEWEB)

    Wenzhong, Zhao; Renqian, Zhong [Second Military medical Coll., Shanghai, SH (China). Dept. of Radiation Medicine

    1989-06-01

    The mechanism of protective effects of bacterial endotoxin (ET) in reconstruction of hemopoiesis post-irradiation has been studied. The results showed that (1) in normal men, dogs, rabbits and rats, after im injection of typhobacterial endotoxin (ETt), the peripheral blood leucocytes (PBL) increased in number remarkably; (2) the injection of microdoses of ETt into hypothalamus of the rabbits and rats caused no obvious change in number of PBL, suggesting that there was neither regulation on the granulomacrophagopoiesis of the vegetative nervous center in hypothalamus nor the regulative effect of the hypothalamo-pituitary endocrine system. The curves of the stimulating factor (CSF) levels in serum after injection of bacillus coli endotoxin (ETc) were compared between the mouse bone marrow cell (BMC) membrane {sup 3}H-galactose incorporation method and the agar cellular colony culture technique. It was shown that they had obvious relationships with the dose-response curve of purified CSF-1. There was no effect on the incorporation value and colony number when ETc was added to culture well directly. The protective effects of the ETc-serum on the 8 Gy irradiated mice was realized by inducing GM-CSF and promoting the reconstruction of hematopoiesis.

  3. Itga2b regulation at the onset of definitive hematopoiesis and commitment to differentiation.

    Directory of Open Access Journals (Sweden)

    Stephanie Dumon

    Full Text Available Product of the Itga2b gene, CD41 contributes to hematopoietic stem cell (HSC and megakaryocyte/platelet functions. CD41 expression marks the onset of definitive hematopoiesis in the embryo where it participates in regulating the numbers of multipotential progenitors. Key to platelet aggregation, CD41 expression also characterises their precursor, the megakaryocyte, and is specifically up regulated during megakaryopoiesis. Though phenotypically unique, megakaryocytes and HSC share numerous features, including key transcription factors, which could indicate common sub-regulatory networks. In these respects, Itga2b can serve as a paradigm to study features of both developmental-stage and HSC- versus megakaryocyte-specific regulations. By comparing different cellular contexts, we highlight a mechanism by which internal promoters participate in Itga2b regulation. A developmental process connects epigenetic regulation and promoter switching leading to CD41 expression in HSC. Interestingly, a similar process can be observed at the Mpl locus, which codes for another receptor that defines both HSC and megakaryocyte identities. Our study shows that Itga2b expression is controlled by lineage-specific networks and associates with H4K8ac in megakaryocyte or H3K27me3 in the multipotential hematopoietic cell line HPC7. Correlating with the decrease in H3K27me3 at the Itga2b Iocus, we find that following commitment to megakaryocyte differentiation, the H3K27 demethylase Jmjd3 up-regulation influences both Itga2b and Mpl expression.

  4. Single-Cell Network Analysis Identifies DDIT3 as a Nodal Lineage Regulator in Hematopoiesis

    Directory of Open Access Journals (Sweden)

    Cristina Pina

    2015-06-01

    Full Text Available We explore cell heterogeneity during spontaneous and transcription-factor-driven commitment for network inference in hematopoiesis. Since individual genes display discrete OFF states or a distribution of ON levels, we compute and combine pairwise gene associations from binary and continuous components of gene expression in single cells. Ddit3 emerges as a regulatory node with positive linkage to erythroid regulators and negative association with myeloid determinants. Ddit3 loss impairs erythroid colony output from multipotent cells, while forcing Ddit3 in granulo-monocytic progenitors (GMPs enhances self-renewal and impedes differentiation. Network analysis of Ddit3-transduced GMPs reveals uncoupling of myeloid networks and strengthening of erythroid linkages. RNA sequencing suggests that Ddit3 acts through development or stabilization of a precursor upstream of GMPs with inherent Meg-E potential. The enrichment of Gata2 target genes in Ddit3-dependent transcriptional responses suggests that Ddit3 functions in an erythroid transcriptional network nucleated by Gata2.

  5. The Effect of Patient-Specific Instrumentation Incorporating an Extramedullary Tibial Guide on Operative Efficiency for Total Knee Arthroplasty

    Directory of Open Access Journals (Sweden)

    Oh-Ryong Kwon

    2017-01-01

    Full Text Available This retrospective study was to determine if patient-specific instrumentation (PSI for total knee arthroplasty (TKA leads to shortened surgical time through increased operating room efficiency according to different tibial PSI designs. 166 patients underwent primary TKA and were categorized into three groups as follows: PSI without extramedullary (EM tibial guide (group 1, n=48, PSI with EM tibial guide (group 2, n=68, and conventional instrumentation (CI group (group 3, n=50. Four factors were compared between groups, namely, operative room time, thickness of bone resection, tibial slope, and rotation of the component. The mean surgical time was significantly shorter in the PSI with EM tibial guide group (group 2, 63.9±13.6 min compared to the CI group (group 3, 82.8±24.9 min (P<0.001. However, there was no significant difference in the PSI without EM tibial guide group (group 1, 75.3±18.8 min. This study suggests that PSI incorporating an EM tibial guide may lead to high operative efficiency in TKA compared to CI. This trial is registered with KCT0002384.

  6. MicroRNA-125 family members exert a similar role in the regulation of murine hematopoiesis.

    Science.gov (United States)

    Wojtowicz, Edyta E; Walasek, Marta A; Broekhuis, Mathilde J C; Weersing, Ellen; Ritsema, Martha; Ausema, Albertina; Bystrykh, Leonid V; de Haan, Gerald

    2014-10-01

    MicroRNAs (miRNAs) are crucial for proper functioning of hematopoietic stem and progenitor cells (HSPCs). Members of the miRNA-125 family (consisting of miR-125a, miR-125b1, and miR-125b2) are known to confer a proliferative advantage on cells upon overexpression, to decrease the rate of apoptosis by targeting proapoptotic genes, and to promote differentiation toward the myeloid lineage in mice. However, many distinct biological effects of the three miR-125 species have been reported as well. In the current study, we set out to assess whether the three miRNA-125s that carry identical seed sequences could be functionally different. Our data show that overexpression of each of the three miR-125 family members preserves HSPCs in a primitive state in vitro, results in a competitive advantage upon serial transplantation, and promotes skewing toward the myeloid lineage. All miR-125 family members decreased the pool of phenotypically defined Lin(-)Sca(+)Kit(+)CD48(-)CD150(+) long-term hematopoietic stem cells, simultaneously increasing the self-renewal activity upon secondary transplantation. The downregulation of miR-125s in hematopoietic stem cells abolishes these effects and impairs long-term contribution to blood cell production. The introduction of a point mutation within the miRNA-125 seed sequence abolishes all abovementioned effects and leads to the restoration of normal hematopoiesis. Our results show that all miR-125 family members are similar in function, they likely operate in a seed-sequence-dependent manner, and they induce a highly comparable hematopoietic phenotype. Copyright © 2014 ISEH - International Society for Experimental Hematology. Published by Elsevier Inc. All rights reserved.

  7. MDS-associated somatic mutations and clonal hematopoiesis are common in idiopathic cytopenias of undetermined significance.

    Science.gov (United States)

    Kwok, Brian; Hall, Jeff M; Witte, John S; Xu, Yin; Reddy, Prashanti; Lin, Keming; Flamholz, Rachel; Dabbas, Bashar; Yung, Aine; Al-Hafidh, Jenan; Balmert, Emily; Vaupel, Christine; El Hader, Carlos; McGinniss, Matthew J; Nahas, Shareef A; Kines, Julie; Bejar, Rafael

    2015-11-19

    Establishing a diagnosis in patients suspected of having a myelodysplastic syndrome (MDS) can be challenging and could be informed by the identification of somatic mutations. We performed a prospective study to examine the frequency and types of mutations encountered in 144 patients with unexplained cytopenias. Based on bone marrow findings, 17% were diagnosed with MDS, 15% with idiopathic cytopenias of undetermined significance (ICUS) and some evidence of dysplasia, and 69% with ICUS and no dysplasia. Bone marrow DNA was sequenced for mutations in 22 frequently mutated myeloid malignancy genes. Somatic mutations were identified in 71% of MDS patients, 62% of patients with ICUS and some dysplasia, and 20% of ICUS patients and no dysplasia. In total, 35% of ICUS patients carried a somatic mutation or chromosomal abnormality indicative of clonal hematopoiesis. We validated these results in a cohort of 91 lower-risk MDS and 249 ICUS cases identified over a 6-month interval. Mutations were found in 79% of those with MDS, in 45% of those with ICUS with dysplasia, and in 17% of those with ICUS without dysplasia. The spectrum of mutated genes was similar with the exception of SF3B1 which was rarely mutated in patients without dysplasia. Variant allele fractions were comparable between clonal ICUS (CCUS) and MDS as were mean age and blood counts. We demonstrate that CCUS is a more frequent diagnosis than MDS in cytopenic patients. Clinical and mutational features are similar in these groups and may have diagnostic utility once outcomes in CCUS patients are better understood. © 2015 by The American Society of Hematology.

  8. Oral feeding with polyunsaturated fatty acids fosters hematopoiesis and thrombopoiesis in healthy and bone marrow-transplanted mice.

    Science.gov (United States)

    Limbkar, Kedar; Dhenge, Ankita; Jadhav, Dipesh D; Thulasiram, Hirekodathakallu V; Kale, Vaijayanti; Limaye, Lalita

    2017-09-01

    Hematopoietic stem cells play the vital role of maintaining appropriate levels of cells in blood. Therefore, regulation of their fate is essential for their effective therapeutic use. Here we report the role of polyunsaturated fatty acids (PUFAs) in regulating hematopoiesis which has not been explored well so far. Mice were fed daily for 10 days with n-6/n-3 PUFAs, viz. linoleic acid (LA), arachidonic acid (AA), alpha-linolenic acid and docosahexanoic acid (DHA) in four separate test groups with phosphate-buffered saline fed mice as control set. The bone marrow cells of PUFA-fed mice showed a significantly higher hematopoiesis as assessed using side population, Lin-Sca-1 + ckit+, colony-forming unit (CFU), long-term culture, CFU-spleen assay and engraftment potential as compared to the control set. Thrombopoiesis was also stimulated in PUFA-fed mice. A combination of DHA and AA was found to be more effective than when either was fed individually. Higher incorporation of PUFAs as well as products of their metabolism was observed in the bone marrow cells of PUFA-fed mice. A stimulation of the Wnt, CXCR4 and Notch1 pathways was observed in PUFA-fed mice. The clinical relevance of this study was evident when bone marrow-transplanted recipient mice, which were fed with PUFAs, showed higher engraftment of donor cells, suggesting that the bone marrow microenvironment may also be stimulated by feeding with PUFAs. These data indicate that oral administration of PUFAs in mice stimulates hematopoiesis and thrombopoiesis and could serve as a valuable supplemental therapy in situations of hematopoietic failure. Copyright © 2017 Elsevier Inc. All rights reserved.

  9. Biochemical Characterization of P4-ATPase Mutations Associated with Intrahepatic Cholestatic Disease

    DEFF Research Database (Denmark)

    Gantzel, Rasmus; Vestergaard, Anna Lindeløv; Mikkelsen, Stine

    The cholestatic disorders progressive familial intrahepatic cholestasis type 1 (PFIC1, also referred to as Byler’s disease) and benign recurrent intrahepatic cholestasis type 1 (BRIC1) are caused by mutation of the P4-ATPase ATP8B1. The substrate of ATP8B1 is very likely to be phosphatidylserine...... families have been investigated, and more than 50 distinct disease mutations have been identified, with roughly half being missense mutations. In this project we try to answer the question whether PFIC1 mutations are generally more disturbing than BRIC1 mutations with respect to expression, structural...... stability and function. We investigate the mutations in our well functioning system of ATP8A2, being expressed in mammalian HEK293T cells, affinity-purified, and reconstituted in lipid vesicles. Well-known mutations from both groups of patients have been selected for study. I91P in ATP8A2 (L127P in ATP8B1...

  10. Sonographic differentiation of pneumobilia from intrahepatic stone: U.F.O. sign

    International Nuclear Information System (INIS)

    Han, Sang Suk; Kim, S. S.; Kwon, D. Y.; Kim, C. D.; Cha, S. S.; Jeon, J. R.; Jung, D. H.

    1990-01-01

    Pneumobilia is one of the most troublesome problem in the sonographic examination of the hepatobiliary system, and the differentiation of air from intrahepatic stone is very important. We have analyzed the sonographic characteristics of 42 cases of pneumobilia dn 56 cases of intrahepatic stones over a period of 4 years. New findings of pneumobilia we observed were U.F.O. sign, wing like artefact, and nonvisualization of the lumen of the bile filled duct. Findings of pneumobilia only were U.F.O. sign, nng down and wing like artefact. Findings of stone only were ecdhogenic foci larger than 1.5cm, heterogeneous echo, wedge, shell, or sandwich like contour, and visible lumen of the bile duct

  11. Biochemical characterization of P4-ATPase mutations associated with Intrahepatic Cholestatic Disease

    DEFF Research Database (Denmark)

    Gantzel, Rasmus; Vestergaard, Anna Lindeløv; Mikkelsen, Stine

    Progressive familial intrahepatic cholestasis type 1 (PFIC1) and benign recurrent intrahepatic cholestasis type 1 (BRIC1) are caused by mutation of the P4-ATPase ATP8B1 that flips phospholipid from the exoplasmic leaflet to the cytoplasmic leaflet of canalicular membranes. It is hypothesized...... that PFIC1 mutations are the most disturbing with respect to expression, structural stability and/or function. Although recent data indicates that the specific phospholipid substrate of ATP8B1 is phosphatidylcholine (PC) [1] whereas ATP8A2 flips phosphatidylserine (PS) and phosphatidylethanolamine (PE......), there may be several mechanistic similarities between ATP8B1 and ATP8A2, and here we investigate known disease mutations using our well-functioning methodology for expression, affinity purification and assay of the partial reactions of ATP8A2. Mutations I91P (L127P in ATP8B1) and L308F (I344F) are located...

  12. [A case of fascioliasis in the intrahepatic duct with concurrent clonochiasis].

    Science.gov (United States)

    Kim, Seung-Hyun; Jung, Chang-Kil; Her, Jin; Hur, Ki-Hwan; Choi, Jae-Hyuc; Kang, Kee-Hoon; Hwang, Chan-Hee

    2014-11-01

    The main causes of biliary obstruction are stones and cancers. Fascioliasis is a very rare case which causes biliary obstruction. Fascioliasis is a zoonosis caused by Fasciola hepatica which infects herbivores like sheep and cattle. F. hepatica lives in the biliary system or the liver parenchyma of a host. In Korea, the occurrence of this infection in human is very rare and only few cases have been reported. A 32-year-old male presented with upper abdominal pain and jaundice. His laboratory finding revealed elevated liver transaminases. Abdomen CT scan showed mild left intrahepatic bile duct dilatation. On ERCP, adult F. hepatica worms were found and were thus removed. Concurrently, clonorchiasis was diagnosed by stool exam and serologic enzyme-linked immunosorbent assay test. Clonorchiasis was treated with praziquantel. Herein, we report a case of intrahepatic bile duct dilatation due to F. hepatica infection with concurrent Clonorchis sinensis infestation.

  13. Congenital, solitary, large, intrahepatic arterioportal fistula in a child: management and review of the literature

    Energy Technology Data Exchange (ETDEWEB)

    Kumar, Nagappan; de Ville de Goyet, Jean; Sharif, Khalid; McKiernan, Patrick [Liver Unit, Birmingham Children' s Hospital NHS Trust, Steelhouse Lane, Birmingham B4 6NH (United Kingdom); John, Philip [Department of Radiology, Birmingham Children' s Hospital NHS Trust, Birmingham (United Kingdom)

    2003-01-01

    Congenital intrahepatic arterioportal fistula (APF) is a rare condition. In most cases, the symptoms and complications develop during infancy. We report here the incidental finding of a large and solitary congenital APF in a 13-year-old boy, with subsequent related clinical complications. At angiography, an APF connecting the left hepatic artery and the left branch of the portal vein (PV) was demonstrated with reversed flow in the left and main PV. The fistula was successfully occluded, in a single embolisation session, using an Amplatzer occlusion device. This was associated with immediate restoration of normal hepatopetal flow in the PV and followed by resolution of the clinical signs of portal hypertension. This patient is the oldest child with congenital intrahepatic APF to be reported. We emphasise the interest of using a large device (Amplatzer) to occlude a solitary large APF in a single session and, more importantly, to avoid other possible complications related to embolisation. (orig.)

  14. Minimally invasive treatment of intrahepatic cholangiolithiasis after stricture of hepaticojejunal anastomosis.

    Science.gov (United States)

    Varabei, Aliaksandr; Arlouski, Yury; Lagodich, Natalli; Arehay, Vitaly

    2018-03-01

    The aim of the study was to improve the results of treatment of patients with intrahepatic cholangiolithiasis for hepaticojejunostomy stricture with use of miniinvasive methods. In our centre during the period from 2002 till 2016 were treated in 58 patients with hepaticojejunostomy strictures. Thirteen patients from their was coexistant intrahepatic cholangiolithiasis. Forty-six (79.3%) patients was performed rehepaticojejunostomy. Twelve patients was performed a minimally invasive intervention such as laser recanalisation using double balloon enteroscopy (7 patients) and lithoextraction with double balloon enteroscopy (1), transhepatic cholangioscopy (2 patients) with laser lithotripsy (1), balloon dilatation of the stricture rehepaticojejunostomosis (4), lithoextraction (4), including with double balloon enteroscopy ("randevoux" procedure) (1), stenting (2). We observed several complication such as cholangitis (5); recurrent cholangiolithiasis (1); restricture of rehepaticojejunostomosis (2). Miniinvasive endoscopic techniques treatment and endobiliary correction of rehepaticojejunostomosis strictures and cholangiolithiasis have shown good results.

  15. Treatment of Portosystemic Shunt Myelopathy with a Stent Graft Deployed through a Transjugular Intrahepatic Route

    International Nuclear Information System (INIS)

    Jain, Deepak; Arora, Ankur; Deka, Pranjal; Mukund, Amar; Bhatnagar, Shorav; Jindal, Deepti; Kumar, Niteen; Pamecha, Viniyendra

    2013-01-01

    A case of surgically created splenorenal shunt complicated with shunt myelopathy was successfully managed by placement of a stent graft within the splenic vein to close the portosystemic shunt and alleviate myelopathy. To our knowledge, this is the first report of a case of shunt myelopathy in a patient with noncirrhotic portal fibrosis without cirrhosis treated by a novel technique wherein a transjugular intrahepatic route was adopted to deploy the stent graft

  16. Comparative Proteomics Analysis of Placenta from Pregnant Women with Intrahepatic Cholestasis of Pregnancy

    OpenAIRE

    Zhang, Ting; Guo, Yueshuai; Guo, Xuejiang; Zhou, Tao; Chen, Daozhen; Xiang, Jingying; Zhou, Zuomin

    2013-01-01

    INTRODUCTION: Intrahepatic cholestasis of pregnancy (ICP) usually occurs in the third trimester and associated with increased risks in fetal complications. Currently, the exact cause of this disease is unknown. In this study we aim to investigate the potential proteins in placenta, which may participate in the molecular mechanisms of ICP-related fetal complications using iTRAQ-based proteomics approach. METHODS: The iTRAQ analysis combined with liquid chromatography-tandem mass spectrometry (...

  17. A rare intrahepatic portacaval tubular shunt in a patient with spastic paraparesis

    Energy Technology Data Exchange (ETDEWEB)

    Ardic, S.; Deniz, E.; Ardic, F.A. [Ankara State Hospital, Ankara, (Turkey). Department of Radiology; Arbatli, M. [Bayindir Tip Hospital, Ankara, (Turkey). Department of Radiology; Aysun, A. [Ankara Oncology Center, Ankara, (Turkey). Department of Radiology

    1997-05-01

    Being a non-invasive and less costly method of imaging, colour Doppler with spectral analysis has an ever-increasing role in the diagnosis and follow-up of portosystemic collaterals in chronic liver disease. A case is presented a case with characteristic cranial MR and CT findings of portosystemic encephalopathy and an intrahepatic portacaval shunt demonstrated with colour Doppler and abdominal MR imaging. 17 refs.

  18. Calorie Restricted High Protein Diets Downregulate Lipogenesis and Lower Intrahepatic Triglyceride Concentrations in Male Rats

    Directory of Open Access Journals (Sweden)

    Lee M. Margolis

    2016-09-01

    Full Text Available The purpose of this investigation was to assess the influence of calorie restriction (CR alone, higher-protein/lower-carbohydrate intake alone, and combined CR higher-protein/lower-carbohydrate intake on glucose homeostasis, hepatic de novo lipogenesis (DNL, and intrahepatic triglycerides. Twelve-week old male Sprague Dawley rats consumed ad libitum (AL or CR (40% restriction, adequate (10%, or high (32% protein (PRO milk-based diets for 16 weeks. Metabolic profiles were assessed in serum, and intrahepatic triglyceride concentrations and molecular markers of de novo lipogenesis were determined in liver. Independent of calorie intake, 32% PRO tended to result in lower homeostatic model assessment of insulin resistance (HOMA-IR values compared to 10% PRO, while insulin and homeostatic model assessment of β-cell function (HOMA-β values were lower in CR than AL, regardless of protein intake. Intrahepatic triglyceride concentrations were 27.4 ± 4.5 and 11.7 ± 4.5 µmol·g−1 lower (p < 0.05 in CR and 32% PRO compared to AL and 10% PRO, respectively. Gene expression of fatty acid synthase (FASN, stearoyl-CoA destaurase-1 (SCD1 and pyruvate dehydrogenase kinase, isozyme 4 (PDK4 were 45% ± 1%, 23% ± 1%, and 57% ± 1% lower (p < 0.05, respectively, in CR than AL, regardless of protein intake. Total protein of FASN and SCD were 50% ± 1% and 26% ± 1% lower (p < 0.05 in 32% PRO compared to 10% PRO, independent of calorie intake. Results from this investigation provide evidence that the metabolic health benefits associated with CR—specifically reduction in intrahepatic triglyceride content—may be enhanced by consuming a higher-protein/lower-carbohydrate diet.

  19. A rare intrahepatic portacaval tubular shunt in a patient with spastic paraparesis

    International Nuclear Information System (INIS)

    Ardic, S.; Deniz, E.; Ardic, F.A.; Arbatli, M.; Aysun, A.

    1997-01-01

    Being a non-invasive and less costly method of imaging, colour Doppler with spectral analysis has an ever-increasing role in the diagnosis and follow-up of portosystemic collaterals in chronic liver disease. A case is presented a case with characteristic cranial MR and CT findings of portosystemic encephalopathy and an intrahepatic portacaval shunt demonstrated with colour Doppler and abdominal MR imaging

  20. Intrahepatic injection of adenovirus reduces inflammation and increases gene transfer and therapeutic effect in mice

    OpenAIRE

    Crettaz, J. (Julien); Berraondo, P. (Pedro); Mauleon, I. (Itsaso); Ochoa, L. (Laura); Shankar, V. (Vijay); Barajas, M. (Miguel); Rooijen, N. (Nico) van; Kochanek, S. (Stefan); Qian, C. (Cheng); Prieto, J. (Jesús); Hernandez-Alcoceba, R. (Rubén); Gonzalez-Aseguinolaza, G. (Gloria)

    2006-01-01

    Recombinant adenoviruses (Ad) are among the most extensively used vectors for liver gene transfer. One of the major limitations for the clinical application of these vectors is the inflammatory immune response associated with systemic administration of high dose of virus. We evaluated the effect of Ad administration route on the inflammatory immune response and liver transgene expression. We compared direct intrahepatic injection (IH) with the systemic administration via tail vein (IV). IH in...

  1. Effects of transjugular intrahepatic portosystemic shunt (TIPS) on blood volume distribution in patients with cirrhosis

    DEFF Research Database (Denmark)

    Busk, Troels M; Bendtsen, Flemming; Henriksen, Jens H

    2017-01-01

    increased (+22%, prestores central hypovolaemia......BACKGROUND: Cirrhosis is accompanied by portal hypertension with splanchnic and systemic arterial vasodilation, and central hypovolaemia. A transjugular intrahepatic portosystemic shunt (TIPS) alleviates portal hypertension, but also causes major haemodynamic changes. AIMS: To investigate effects...... catheterization. Central and arterial blood volume (CBV) and cardiac output (CO) were determined with indicator dilution technique. RESULTS: After TIPS, the thoracic blood volume increased (+10.4% of total blood volume (TBV), p

  2. Trousseau's Syndrome Caused by Intrahepatic Cholangiocarcinoma: An Autopsy Case Report and Literature Review

    Directory of Open Access Journals (Sweden)

    Takashi Yuri

    2014-05-01

    Full Text Available An autopsy case report of Trousseau's syndrome caused by intrahepatic cholangiocarcinoma is presented, and seven previously reported cases are reviewed. A 73-year-old woman experiencing light-headedness and dementia of unknown cause for 6 months developed severe hypotonia. A hypointense lesion compatible with acute cerebral infarction was detected by magnetic resonance imaging. Abdominal computed tomography revealed an ill-defined large liver mass in the right lobe. The mass was not further investigated because of the patient's poor condition. She died of multiple organ failure, and an autopsy was conducted. Postmortem examination revealed intrahepatic cholangiocarcinoma, fibrous vegetations on the mitral valves and multiple thromboemboli in the cerebrum, spleen and rectum. Trousseau's syndrome is defined as an idiopathic thromboembolism in patients with undiagnosed or concomitantly diagnosed malignancy. This syndrome is encountered frequently in patients with mucin-producing carcinomas, while the incidence in patients with intrahepatic cholangiocarcinoma is uncommon. We found that tissue factor and mucin tumor marker (CA19-9, CA15-3 and CA-125 expression in cancer cells may be involved in the pathogenesis of thromboembolism. A patient with unexplained thromboembolism may have occult visceral malignancy; thus, mucin tumor markers may indicate the origin of a mucin-producing carcinoma, and postmortem examination may play an important role in revealing the hidden malignancy.

  3. The Hippo signaling functions through the Notch signaling to regulate intrahepatic bile duct development in mammals

    Science.gov (United States)

    Wu, Nan; Nguyen, Quy; Wan, Ying; Zhou, Tiaohao; Venter, Julie; Frampton, Gabriel A; DeMorrow, Sharon; Pan, Duojia; Meng, Fanyin; Glaser, Shannon; Alpini, Gianfranco; Bai, Haibo

    2018-01-01

    The Hippo signaling pathway and the Notch signaling pathway are evolutionary conserved signaling cascades that have important roles in embryonic development of many organs. In murine liver, disruption of either pathway impairs intrahepatic bile duct development. Recent studies suggested that the Notch signaling receptor Notch2 is a direct transcriptional target of the Hippo signaling pathway effector YAP, and the Notch signaling is a major mediator of the Hippo signaling in maintaining biliary cell characteristics in adult mice. However, it remains to be determined whether the Hippo signaling pathway functions through the Notch signaling in intrahepatic bile duct development. We found that loss of the Hippo signaling pathway tumor suppressor Nf2 resulted in increased expression levels of the Notch signaling pathway receptor Notch2 in cholangiocytes but not in hepatocytes. When knocking down Notch2 on the background of Nf2 deficiency in mouse livers, the excessive bile duct development induced by Nf2 deficiency was suppressed by heterozygous and homozygous deletion of Notch2 in a dose-dependent manner. These results implicated that Notch signaling is one of the downstream effectors of the Hippo signaling pathway in regulating intrahepatic bile duct development. PMID:28581486

  4. Intrahepatic peribiliary perivascular epithelioid cell tumor (PEComa) associated with heterotopic pancreas: A case report.

    Science.gov (United States)

    Kiriyama, Yuka; Tsukamoto, Tetsuya; Mizoguchi, Yoshikazu; Ishihara, Shin; Horiguchi, Akihiko; Tokoro, Takamasa; Kato, Yutaro; Sugioka, Atsushi; Kuroda, Makoto

    2016-08-20

    Perivascular epithelioid-cell tumor (PEComa) is a group of rare mesenchymal neoplasms that express myomelanocytic-cell markers and exhibit a wide variety of histopathological features. Although heterotopic pancreas has been reported to occur in the gastrointestinal tract, intrahepatic heterotopic pancreas has been reported only rarely. We present a case of intrahepatic PEComa that showed a strong regional correlation with the presence of heterotopic pancreas. An intrahepatic tumor and biliary dilatation was incidentally discovered during a diagnostic evaluation to investigate low-back pain in a 47-year-old Japanese male. Cholangiocarcinoma was suspected and a left hemihepatectomy performed. Histological examination revealed a 3 × 3.8-mm tumor in the neighboring B2 bile duct. Histological and immunohistochemical investigations revealed the presence of a PEComa and pancreatic acini within the tumor mass. PEComa in the hepatobiliary and pancreatic regions are extremely rare. The presence of heterotopic pancreas is also relatively uncommon. The strong regional association of these 2 lesions raises the possibility of a PEComa originating from heterotopic pancreas or from an irritable response caused by heterotopic pancreas.

  5. Clinical and pathological significance of ROS1 expression in intrahepatic cholangiocarcinoma

    International Nuclear Information System (INIS)

    Lee, Kyung-Hun; Lee, Kyoung-Bun; Kim, Tae-Yong; Han, Sae-Won; Oh, Do-Youn; Im, Seock-Ah; Kim, Tae-You; Yi, Nam-Joon; Lee, Kwang-Woong; Suh, Kyung-Suk; Jang, Ja-June; Bang, Yung-Jue

    2015-01-01

    More knowledge about genetic and molecular features of cholangiocarcinoma is needed to develop effective therapeutic strategies. We investigated the clinical and pathological significance of ROS1 expression in intrahepatic cholangiocarcinoma. One hundred ninety-four patients with curatively resected intrahepatic cholangiocarcinoma were included in this study. Tumor tissue specimens were collected and analyzed for ROS1 gene rearrangement using fluorescence in situ hybridization (FISH) and ROS1 protein expression using immunohistochemistry (IHC). ROS1 immunohistochemistry was positive (moderate or strong staining) in 72 tumors (37.1 %). ROS1 protein expression was significantly correlated with well differentiated tumors, papillary or mucinous histology, oncocytic/hepatoid or intestinal type tumors, and periductal infiltrating or intraductal growing tumors (vs. mass-forming cholangiocarcinoma). ROS-expressing tumors were associated with better disease-free survival (30.1 months for ROS1 expression (+) tumors vs. 9.0 months for ROS1 (−) tumors, p = 0.006). Moreover, ROS1 expression was an independent predictor of better disease-free survival in a multivariate analysis (HR 0.607, 95 % CI 0.377–0.976; p = 0.039). Although break-apart FISH was successfully performed in 102 samples, a split pattern indicative of ROS1 gene rearrangement was not found in the examined samples. ROS1 protein expression was associated with well-differentiated histology and better survival in our patients with resected intrahepatic cholangiocarcinoma. ROS1 gene rearrangement by break-apart FISH was not found in the examined samples

  6. Transcatheter embolization of a congenital intrahepatic arterioportal venous malformation: A case report

    International Nuclear Information System (INIS)

    Sing, T.M.Y.S.; Wong, K.P.; Young, N.; Le, S.D.V.

    1997-01-01

    Congenital intrahepatic arterioportal venous malformations (APVM) are uncommon lesions. A congenital intrahepatic APVM found incidentally in a 51 -year-old man during pre-operative aortography for an abdominal aortic aneurysm is reported here. This was successfully treated by transcatheter embolization of the involved hepatic artery prior to surgical repair of the aortic aneurysm. A 51-year-old smoker was admitted for pre-operative aortography of an abdominal aortic aneurysm (AAA). Liver function tests showed a mildly elevated alkaline phosphatase. There was no previous history of liver disease or trauma. Aortography demonstrated a large infra-renal AAA measuring 10 cm in diameter and 20 cm in length. The coeliac axis was noted to be grossly dilated with tortuous veins seen to the right side of the lower thoracic spine on delayed images. Coeliac angiography revealed a dilated intrahepatic vascular abnormality in the left lobe of the liver with late opacification of the portal vein. Contrast abdominal CT demonstrated the AAA and the dilated coeliac axis feeding a large vascular malformation in the lateral aspect of the left lobe of the liver. The arterial inflow was via the left hepatic artery and a large vein was seen leading into the left portal vein. Endoscopy showed no oesophageal varices. (authors)

  7. The value of ultrasonographic examination in the evaluation of liver size and intrahepatic vessels

    International Nuclear Information System (INIS)

    Narojek, T.

    1995-01-01

    The objective of the investigations was to comparatively evaluate the size and shape of the liver on the basis of x-ray and ultrasonographic examinations and to estimate the liver parenchyma and intrahepatic vessels on the basis of ultrasonographic examinations. The studies were done on 64 dogs and 13 cats of different breed and sex, aged from 1 day to 18 years. The ultrasonographic examinations were done with the use of Bruel and Kjaer apparatus type 1849 and Concept 2000 by Dynamic Imaging. In 64 animals the ultrasonographic image of the liver was normal. In 7 cases an enlargement of the liver without any changes in the liver parenchyma was noted. An enlargement of intrahepatic veins was found in 8 cases (4 with circulatory insufficiency, 2 with ascites). The assessment of the size and shape of the liver done on that basis of ultrasound examinations agreed with that based on x-ray examinations. The ultrasonic examinations also allowed the evaluation of the liver parenchyma and intrahepatic veins. (author). 9 refs, figs, 3 tabs

  8. Angiographic embolization in the treatment of intrahepatic arterial bleeding in patients with blunt abdominal trauma.

    Science.gov (United States)

    Kong, Ya-Lin; Zhang, Hong-Yi; He, Xiao-Jun; Zhao, Gang; Liu, Cheng-Li; Xiao, Mei; Zhen, Yu-Ying

    2014-04-01

    Angiographic embolization (AE) as an adjunct non-operative treatment of intrahepatic arterial bleeding has been widely used. The present study aimed to evaluate the efficacy of selective AE in patients with hepatic trauma. Seventy patients with intrahepatic arterial bleeding after blunt abdominal trauma who had undergone selective AE in 10 years at this institution were retrospectively reviewed. The criteria for selective AE included active extravasation on contrast-enhanced CT, an episode of hypotension or a decrease in hemoglobin level during the non-operative treatment. The data of the patients included demographics, grade of liver injuries, mechanism of blunt abdominal trauma, associated intra-abdominal injuries, indications for AE, angiographic findings, type of AE, and AE-related hepatobiliary complications. In the 70 patients, 32 (45.71%) had high-grade liver injuries. Extravazation during the early arterial phase mainly involved the right hepatic segments. Thirteen (18.57%) patients underwent embolization of intrahepatic branches and the extrahepatic trunk and these patients all developed AE-related hepatobiliary complications. In 19 patients with AE-related complications, 14 received minimally invasive treatment and recovered without severe sequelae. AE is an adjunct treatment for liver injuries. Selective and/or super-selective AE should be advocated to decrease the incidence and severity of AE-related hepatobiliary complications.

  9. Transcatheter embolization of a congenital intrahepatic arterioportal venous malformation: A case report

    Energy Technology Data Exchange (ETDEWEB)

    Sing, T.M.Y.S.; Wong, K.P.; Young, N. [Westmead Hospital, Westmead, NSW, (Australia). Department of Radiaology; Le, S.D.V. [Bankstown-Lidcombe Hospital, Bankstown, NSW, (Australia). Department of Nuclear Medicine and Ultrasound

    1997-08-01

    Congenital intrahepatic arterioportal venous malformations (APVM) are uncommon lesions. A congenital intrahepatic APVM found incidentally in a 51 -year-old man during pre-operative aortography for an abdominal aortic aneurysm is reported here. This was successfully treated by transcatheter embolization of the involved hepatic artery prior to surgical repair of the aortic aneurysm. A 51-year-old smoker was admitted for pre-operative aortography of an abdominal aortic aneurysm (AAA). Liver function tests showed a mildly elevated alkaline phosphatase. There was no previous history of liver disease or trauma. Aortography demonstrated a large infra-renal AAA measuring 10 cm in diameter and 20 cm in length. The coeliac axis was noted to be grossly dilated with tortuous veins seen to the right side of the lower thoracic spine on delayed images. Coeliac angiography revealed a dilated intrahepatic vascular abnormality in the left lobe of the liver with late opacification of the portal vein. Contrast abdominal CT demonstrated the AAA and the dilated coeliac axis feeding a large vascular malformation in the lateral aspect of the left lobe of the liver. The arterial inflow was via the left hepatic artery and a large vein was seen leading into the left portal vein. Endoscopy showed no oesophageal varices. (authors). 11 refs., 7 figs.

  10. MRI Findings of Early-Stage Hyperacute Hemorrhage Causing Extramedullary Compression of the Cervical Spinal Cord in a Dog with Suspected Steroid-Responsive Meningitis-Arteritis

    Directory of Open Access Journals (Sweden)

    Adriano Wang-Leandro

    2017-09-01

    Full Text Available A 9-month-old female Weimaraner was presented to the emergency service due to episodes of fever and neck pain. Physical examination revealed a stiff neck posture and elevated body temperature. Shortly after clinical examination was performed, the dog developed peracute onset of non-ambulatory tetraparesis compatible with a C1–C5 spinal cord (SC lesion. Immediately thereafter (<1 h, MRI of the cervical SC was performed with a 3-T scanner. A left ventrolateral intradural-extramedullary SC compression caused by a round-shaped structure at the level of C3––C4 was evidenced. The structure was iso- to slightly hyperintense in T1-weighted (T1W sequences compared to SC parenchyma and hyperintense in T2-weighted, gradient echo, and fluid-attenuated inversion recovery. Moreover, the structure showed a strong homogeneous contrast uptake in T1W sequences. Cerebrospinal fluid (CSF analysis revealed a mixed pleocytosis, as well as elevated protein and erythrocyte count. Early-stage hyperacute extramedullary hemorrhage was suspected due to immune mediated vasculitis. The dog was maintained under general anesthesia and artificial ventilation for 24 h and long-term therapy with corticosteroids and physiotherapy was initiated. Eight weeks after initial presentation, the dog was ambulatory, slightly tetraparetic. Follow-up MRI showed a regression of the round-shaped structure and pleocytosis was not evident in CSF analysis. This report describes an early-stage hyperacute extramedullary hemorrhage, a condition rarely recorded in dogs even in experimental settings.

  11. Multi-Institutional Analysis of Solitary Extramedullary Plasmacytoma of the Head and Neck Treated With Curative Radiotherapy

    International Nuclear Information System (INIS)

    Sasaki, Ryohei; Yasuda, Koichi; Abe, Eisuke; Uchida, Nobue; Kawashima, Mitsuhiko; Uno, Takashi; Fujiwara, Masayuki; Shioyama, Yoshiyuki; Kagami, Yoshikazu; Shibamoto, Yuta; Nakata, Kensei; Takada, Yoshie; Kawabe, Tetsuya; Uehara, Kazuyuki; Nibu, Kenichi; Yamada, Syogo

    2012-01-01

    Purpose: The purpose of this study was to elucidate the efficacy and optimal method of radiotherapy in the management of solitary extramedullary plasmacytoma occurring in the head and neck regions (EMPHN). Methods and Materials: Sixty-seven patients (43 male and 24 female) diagnosed with EMPHN between 1983 and 2008 at 23 Japanese institutions were reviewed. The median patient age was 64 years (range, 12–83). The median dose administered was 50 Gy (range, 30–64 Gy). Survival data were calculated by the Kaplan-Meier method. Results: The median follow-up duration was 63 months. Major tumor sites were nasal or paranasal cavities in 36 (54%) patients, oropharynx or nasopharynx in 16 (23%) patients, orbita in 6 (9%) patients, and larynx in 3 (5%) patients. The 5- and 10-year local control rates were 95% and 87%, whereas the 5- and 10-year disease-free survival rates were 56% and 54%, respectively. There were 5 (7.5%), 12 (18%), and 8 (12%) patients who experienced local failure, distant metastasis, and progression to multiple myeloma, respectively. In total, 18 patients died, including 10 (15%) patients who died due to complications from EMPHN. The 5- and 10-year overall survival (OS) rates were 73% and 56%, respectively. Radiotherapy combined with surgery was identified as the lone significant prognostic factor for OS (p = 0.04), whereas age, gender, radiation dose, tumor size, and chemotherapy were not predictive. No patient experienced any severe acute morbidity. Conclusions: Radiotherapy was quite effective and safe for patients with EMPHN. Radiotherapy combined with surgery produced a better outcome according to survival rates. These findings require confirmation by further studies with larger numbers of patients with EMPHN.

  12. Randomized comparison of reduced fat and reduced carbohydrate hypocaloric diets on intrahepatic fat in overweight and obese human subjects.

    Science.gov (United States)

    Haufe, Sven; Engeli, Stefan; Kast, Petra; Böhnke, Jana; Utz, Wolfgang; Haas, Verena; Hermsdorf, Mario; Mähler, Anja; Wiesner, Susanne; Birkenfeld, Andreas L; Sell, Henrike; Otto, Christoph; Mehling, Heidrun; Luft, Friedrich C; Eckel, Juergen; Schulz-Menger, Jeanette; Boschmann, Michael; Jordan, Jens

    2011-05-01

    Obesity-related hepatic steatosis is a major risk factor for metabolic and cardiovascular disease. Fat reduced hypocaloric diets are able to relieve the liver from ectopically stored lipids. We hypothesized that the widely used low carbohydrate hypocaloric diets are similarly effective in this regard. A total of 170 overweight and obese, otherwise healthy subjects were randomized to either reduced carbohydrate (n = 84) or reduced fat (n = 86), total energy restricted diet (-30% of energy intake before diet) for 6 months. Body composition was estimated by bioimpedance analyses and abdominal fat distribution by magnetic resonance tomography. Subjects were also submitted to fat spectroscopy of liver and oral glucose tolerance testing. In all, 102 subjects completed the diet intervention with measurements of intrahepatic lipid content. Both hypocaloric diets decreased body weight, total body fat, visceral fat, and intrahepatic lipid content. Subjects with high baseline intrahepatic lipids (>5.56%) lost ≈7-fold more intrahepatic lipids compared with those with low baseline values (diet composition. In contrast, changes in visceral fat mass and insulin sensitivity were similar between subgroups, with low and high baseline intrahepatic lipids. A prolonged hypocaloric diet low in carbohydrates and high in fat has the same beneficial effects on intrahepatic lipid accumulation as the traditional low-fat hypocaloric diet. The decrease in intrahepatic lipids appears to be independent of visceral fat loss and is not tightly coupled with changes in whole body insulin sensitivity during 6 months of an energy restricted diet. Copyright © 2011 American Association for the Study of Liver Diseases.

  13. Zebrafish hoxd4a acts upstream of meis1.1 to direct vasculogenesis, angiogenesis and hematopoiesis.

    Directory of Open Access Journals (Sweden)

    Aseervatham Anusha Amali

    Full Text Available Mice lacking the 4th-group paralog Hoxd4 display malformations of the anterior vertebral column, but are viable and fertile. Here, we report that zebrafish embryos having decreased function of the orthologous hoxd4a gene manifest striking perturbations in vasculogenesis, angiogenesis and primitive and definitive hematopoiesis. These defects are preceded by reduced expression of the hemangioblast markers scl1, lmo2 and fli1 within the posterior lateral plate mesoderm (PLM at 13 hours post fertilization (hpf. Epistasis analysis revealed that hoxd4a acts upstream of meis1.1 but downstream of cdx4 as early as the shield stage in ventral-most mesoderm fated to give rise to hemangioblasts, leading us to propose that loss of hoxd4a function disrupts hemangioblast specification. These findings place hoxd4a high in a genetic hierarchy directing hemangioblast formation downstream of cdx1/cdx4 and upstream of meis1.1. An additional consequence of impaired hoxd4a and meis1.1 expression is the deregulation of multiple Hox genes implicated in vasculogenesis and hematopoiesis which may further contribute to the defects described here. Our results add to evidence implicating key roles for Hox genes in their initial phase of expression early in gastrulation.

  14. ZIP8 zinc transporter: indispensable role for both multiple-organ organogenesis and hematopoiesis in utero.

    Directory of Open Access Journals (Sweden)

    Marina Gálvez-Peralta

    Full Text Available Previously this laboratory characterized Slc39a8-encoded ZIP8 as a Zn(2+/(HCO(3(-(2 symporter; yet, the overall physiological importance of ZIP8 at the whole-organism level remains unclear. Herein we describe the phenotype of the hypomorphic Slc39a8(neo/neo mouse which has retained the neomycin-resistance gene in intron 3, hence causing significantly decreased ZIP8 mRNA and protein levels in embryo, fetus, placenta, yolk sac, and several tissues of neonates. The Slc39a8(neo allele is associated with diminished zinc and iron uptake in mouse fetal fibroblast and liver-derived cultures; consequently, Slc39a8(neo/neo newborns exhibit diminished zinc and iron levels in several tissues. Slc39a8(neo/neo homozygotes from gestational day(GD-11.5 onward are pale, growth-stunted, and die between GD18.5 and 48 h postnatally. Defects include: severely hypoplastic spleen; hypoplasia of liver, kidney, lung, and lower limbs. Histologically, Slc39a8(neo/neo neonates show decreased numbers of hematopoietic islands in yolk sac and liver. Low hemoglobin, hematocrit, red cell count, serum iron, and total iron-binding capacity confirmed severe anemia. Flow cytometry of fetal liver cells revealed the erythroid series strikingly affected in the hypomorph. Zinc-dependent 5-aminolevulinic acid dehydratase, required for heme synthesis, was not different between Slc39a8(+/+ and Slc39a8(neo/neo offspring. To demonstrate further that the mouse phenotype is due to ZIP8 deficiency, we bred Slc39a8(+/neo with BAC-transgenic BTZIP8-3 line (carrying three extra copies of the Slc39a8 allele; this cross generated viable Slc39a8(neo/neo_BTZIP8-3(+/+ pups showing none of the above-mentioned congenital defects-proving Slc39a8(neo/neo causes the described phenotype. Our study demonstrates that ZIP8-mediated zinc transport plays an unappreciated critical role during in utero and neonatal growth, organ morphogenesis, and hematopoiesis.

  15. Localized intrahepatic bile duct dilatation without a visible mass or stone as depicted on CT images: findings of malignancy prediction

    Energy Technology Data Exchange (ETDEWEB)

    Choi, Ju Wan; Kim, Gab Chul; Jeong, Han Young; Lee, Hui Joong; Lee, Jae Hyuck; Ryeom, Hun Kyu [Kyungpook National University Hospital, Daegu (Korea, Republic of); Kim, Jong Yeol [Kumi Cha Medical Center, Collge of Medicine, Pochon CHA University, Kumi (Korea, Republic of)

    2008-09-15

    This study was preformed to evaluate factors that can predict the presence of a malignancy for localized intrahepatic bile duct dilatation without a visible mass or stone as depicted on CT images. A total of 29 patients (male: 16, female: 13) who had localized intrahepatic bile duct dilatation without a visible mass, stone or injury as depicted on CT images were included in the study. A history of extrahepatic malignancy and biliary stone disease, tumor marker levels, CT findings of the intrahepatic bile duct and associated findings were reviewed. The findings were analyzed between two groups (patients with a malignancy and patients with benign disease) on follow-up. In 29 patient, 11 patients had malignant lesions (four metastases and seven cholangiocarcinomas). The history of an extrahepatic malignancy and the shape of an intrahepatic duct obstruction or stenosis as seen on CT were significantly correlated with the results between the benign and malignant group of patients. The follow-up results of the malignant group of patients indicated that for six patients who had developed a new mass, one patient each showed aggravation of ductal dilatation and thickening of the ductal wall. When a patient with localized intrahepatic bile duct dilatation without a definite cause has a history of an extrahepatic malignancy or shows abrupt tapering or irregular narrowing on CT images, short-term follow-up should be performed. The patient should be investigated carefully for mass formation or a change of the dilated bile duct due to a possibility of malignant ductal dilatation.

  16. Quantitation of the intrahepatic shunt index by injection of /sup 99m/Tc-macroaggregated albumin into the spleen

    Energy Technology Data Exchange (ETDEWEB)

    Saito, Masaaki; Ohnishi, Kunihiko; Katsurai, Hiroshi; Tanaka, Hideo; Chin, Nobuyoshi; Iida, Shinji; Nomura, Fumio; Okuda, Kunio

    1987-11-01

    The accuracy of the intrahepatic shunt index measured by injection of /sup 99m/Tc-macroaggregated albumin into the spleen was assessed in comparison with the intrahepatic shunt index measured by injection of /sup 99m/Tc-macroaggregated albumin into the portal trunk in 28 patients with portal hypertension. /sup 99m/Tc-macroaggregated albumin was injected through the catheter into the portal trunk and the intrahepatic shunt index was calculated by counting over predetermined areas of the liver and lungs. Injection of /sup 99m/Tc-macroaggregated albumin into the spleen was performed between one or two weeks before portal vein catheterization, and the calculation of the intrahepatic shunt index was performed with a scinticamera and ROI-radiograms obtained by data-processing. There was a significant correlation between the intrahepatic shunt index calculated by injection of /sup 99m/Tc-macroaggregated albumin into the spleen and that by injection of /sup 99m/Tc-macroaggregated albumin into the portal trunk (r = 0.90, p < 0.001)

  17. Localized intrahepatic bile duct dilatation without a visible mass or stone as depicted on CT images: findings of malignancy prediction

    International Nuclear Information System (INIS)

    Choi, Ju Wan; Kim, Gab Chul; Jeong, Han Young; Lee, Hui Joong; Lee, Jae Hyuck; Ryeom, Hun Kyu; Kim, Jong Yeol

    2008-01-01

    This study was preformed to evaluate factors that can predict the presence of a malignancy for localized intrahepatic bile duct dilatation without a visible mass or stone as depicted on CT images. A total of 29 patients (male: 16, female: 13) who had localized intrahepatic bile duct dilatation without a visible mass, stone or injury as depicted on CT images were included in the study. A history of extrahepatic malignancy and biliary stone disease, tumor marker levels, CT findings of the intrahepatic bile duct and associated findings were reviewed. The findings were analyzed between two groups (patients with a malignancy and patients with benign disease) on follow-up. In 29 patient, 11 patients had malignant lesions (four metastases and seven cholangiocarcinomas). The history of an extrahepatic malignancy and the shape of an intrahepatic duct obstruction or stenosis as seen on CT were significantly correlated with the results between the benign and malignant group of patients. The follow-up results of the malignant group of patients indicated that for six patients who had developed a new mass, one patient each showed aggravation of ductal dilatation and thickening of the ductal wall. When a patient with localized intrahepatic bile duct dilatation without a definite cause has a history of an extrahepatic malignancy or shows abrupt tapering or irregular narrowing on CT images, short-term follow-up should be performed. The patient should be investigated carefully for mass formation or a change of the dilated bile duct due to a possibility of malignant ductal dilatation

  18. Quantitation of the intrahepatic shunt index by injection of 99mTc-macroaggregated albumin into the spleen

    International Nuclear Information System (INIS)

    Saito, Masaaki; Ohnishi, Kunihiko; Katsurai, Hiroshi; Tanaka, Hideo; Chin, Nobuyoshi; Iida, Shinji; Nomura, Fumio; Okuda, Kunio

    1987-01-01

    The accuracy of the intrahepatic shunt index measured by injection of 99m Tc-macroaggregated albumin into the spleen was assessed in comparison with the intrahepatic shunt index measured by injection of 99m Tc-macroaggregated albumin into the portal trunk in 28 patients with portal hypertension. 99m Tc-macroaggregated albumin was injected through the catheter into the portal trunk and the intrahepatic shunt index was calculated by counting over predetermined areas of the liver and lungs. Injection of 99m Tc-macroaggregated albumin into the spleen was performed between one or two weeks before portal vein catheterization, and the calculation of the intrahepatic shunt index was performed with a scinticamera and ROI-radiograms obtained by data-processing. There was a significant correlation between the intrahepatic shunt index calculated by injection of 99m Tc-macroaggregated albumin into the spleen and that by injection of 99m Tc-macroaggregated albumin into the portal trunk (r = 0.90, p < 0.001). (author)

  19. Activation of adenosine A3 receptors supports hematopoiesis-stimulating effects of granulocyte colony-stimulating factor in sublethally irradiated mice

    Czech Academy of Sciences Publication Activity Database

    Hofer, Michal; Pospíšil, Milan; Šefc, L.; Dušek, L.; Vacek, Antonín; Holá, Jiřina; Hoferová, Zuzana; Štreitová, Denisa

    2010-01-01

    Roč. 86, č. 8 (2010), s. 649-656 ISSN 0955-3002 R&D Projects: GA ČR(CZ) GA305/08/0158 Institutional research plan: CEZ:AV0Z50040507; CEZ:AV0Z50040702 Keywords : ionising radiation * hematopoiesis * adenosine A3 receptors Subject RIV: BO - Biophysics Impact factor: 1.861, year: 2010

  20. Combination therapy using PSE and TIO ameliorates hepatic encephalopathy due to intrahepatic portosystemic venous shunt in idiopathic portal hypertension

    International Nuclear Information System (INIS)

    Kojima, Seiichiro; Ito, Hiroyuki; Takashimizu, Shinji; Ichikawa, Hitoshi; Matsumoto, Tomohiro; Hasebe, Terumitsu; Watanabe, Norihito

    2016-01-01

    A 64-year-old woman treated for anemia and ascites exhibited hepatic encephalopathy. Abdominal ultrasonography and computed tomography (CT) showed communication between the portal vein and the middle hepatic vein, indicating an intrahepatic portosystemic venous shunt (PSS). Since hepatic encephalopathy of the patient was resistant to medical treatment, interventional radiology was performed for the treatment of shunt obliteration. Hepatic venography showed anastomosis between the hepatic vein branches, supporting the diagnosis of idiopathic portal hypertension (IPH). To minimize the increase in portal vein pressure after shunt obliteration, partial splenic artery embolization (PSE) was first performed to reduce portal vein blood flow. Transileocolic venous obliteration (TIO) was then performed, and intrahepatic PSS was successfully obliterated using coils with n-butyl-2-cyanoacrylate (NBCA). In the present case, hepatic encephalopathy due to intrahepatic PSS in the patient with IPH was successfully treated by combination therapy using PSE and TIO

  1. Increased intrahepatic apoptosis but reduced immune activation in HIV-HBV co-infected patients with advanced immunosuppression.

    Science.gov (United States)

    Iser, David M; Avihingsanon, Anchalee; Wisedopas, Naruemon; Thompson, Alexander J; Boyd, Alison; Matthews, Gail V; Locarnini, Stephen A; Slavin, John; Desmond, Paul V; Lewin, Sharon R

    2011-01-14

    to determine if intrahepatic immune activation is increased in HIV-hepatitis B virus (HBV) co-infected patients compared to HBV mono-infected patients and whether this reduced following HBV-active antiretroviral therapy (ART) in HIV-HBV co-infected patients. : Case-control observational study. we examined liver biopsies for markers of T-cell and monocyte infiltration and activation, natural killer cells, hepatic stellate cell (HSC) activation (staining for alpha smooth muscle actin) and apoptosis [using terminal dUTP nick-end labelling (TUNEL)] in treatment-naive Asian HIV-HBV co-infected (n = 16) and HBV mono-infected patients matched for age and HBV e-antigen status (n = 16). Liver biopsies from a subset of co-infected patients (n = 15) were also compared prior to and following 48 weeks of HBV-active ART. HIV-HBV co-infected patients had a median CD4 T-cell count of 25 cells/microl and lower alanine aminotransferase levels than HBV mono-infected patients (P = 0.03). In HIV-HBV co-infected patients, hepatocyte apoptosis was increased (P = 0.04) but there were fewer intrahepatic CD4 and CD8 T cells (P < 0.001), lower activation of intrahepatic T cells, Kupffer cells and HSC (P = 0.002, 0.008 and < 0.001, respectively). Following ART, there was a significant decrease in intrahepatic HBsAg staining (P = 0.04) and Kupffer cell activation (P = 0.003). we found no evidence of increased intrahepatic mononuclear and HSC activation in this cohort of HIV-HBV co-infected individuals with advanced immune suppression. An increase in intra-hepatic apoptosis in HIV-HBV co-infected individuals may potentially contribute to accelerated fibrosis in this setting. 2011 Wolters Kluwer Health | Lippincott Williams & Wilkins.

  2. Intrahepatic recurrence after percutaneous radiofrequency ablation of hepatocellular carcinoma: Analysis of the pattern and risk factors

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Young-sun [Department of Radiology and Center for Imaging Science, Samsung Medical Center, Sungkyunkwan University School of Medicine (Korea, Republic of); Department of Diagnostic Radiology, Hanyang University College of Medicine (Korea, Republic of); Rhim, Hyunchul [Department of Radiology and Center for Imaging Science, Samsung Medical Center, Sungkyunkwan University School of Medicine (Korea, Republic of) and Department of Diagnostic Radiology, Hanyang University College of Medicine (Korea, Republic of)]. E-mail: forest@smc.samsung.co.kr; Cho, On Koo [Department of Diagnostic Radiology, Hanyang University College of Medicine (Korea, Republic of); Koh, Byung Hee [Department of Diagnostic Radiology, Hanyang University College of Medicine (Korea, Republic of); Kim, Yongsoo [Department of Diagnostic Radiology, Hanyang University College of Medicine (Korea, Republic of)

    2006-09-15

    Purpose: To evaluate the pattern and risks for intrahepatic recurrence after percutaneous radiofrequency (RF) ablation for hepatocellular carcinoma (HCC). Materials and methods: We studied 62 patients with 72 HCCs ({<=}4 cm) who were treated with percutaneous RF ablation. The mean follow-up period was 19.1 months (6.0-49.1). We assessed the incidence and cumulative disease-free survival of local tumor progression (LTP) and intrahepatic distant recurrence (IDR). To analyze the risk factors, we examined the following, for the LTP: (1) tumor diameter, (2) contact with vessels, (3) degree of approximation to hepatic hilum, (4) contact with hepatic capsule, (5) presence of ablative safety margin, (6) degree of benign periablational enhancement and (7) serum alpha-fetoprotein; for the IDR: (1) severity of hepatic disease, (2) presence of HBsAg, (3) serum alpha-fetoprotein, (4) whether RF ablation was the initial treatment and (5) multiplicity of tumor for IDR. Results: The incidence of overall recurrence, LTP and IDR was 62.9%, 26.4% and 53.2%, respectively. The cumulative disease-free survival rates were 52%, 82% and 56% at 1 year, 26%, 63% and 30% at 2 years, respectively. Univariate analysis showed that the significant risk factors for LTP were: a tumor with a diameter >3 cm, contact of HCC with a vessel and an insufficient safety margin (p < 0.05). A multivariate stepwise Cox hazard model showed that the measurement of a tumor diameter >3 cm and insufficient safety margin were independent factors. Only the increased serum alpha-fetoprotein was a significant risk factor for IDR (p < 0.05). Conclusion: Intrahepatic recurrence after percutaneous RF ablation is common. Large HCC (>3 cm) with high serum alpha-fetoprotein should be treated more aggressively because of higher risk for recurrence.

  3. Potential efficacy of therapies targeting intrahepatic lesions after sorafenib treatment of patients with hepatocellular carcinoma

    International Nuclear Information System (INIS)

    Terashima, Takeshi; Yamashita, Tatsuya; Horii, Rika; Arai, Kuniaki; Kawaguchi, Kazunori; Kitamura, Kazuya; Yamashita, Taro; Sakai, Yoshio; Mizukoshi, Eishiro; Honda, Masao; Kaneko, Shuichi

    2016-01-01

    We investigated the contribution of subsequent therapy for advanced hepatocellular carcinoma refractory or intolerant to sorafenib. Further, we investigated the impact of sorafenib on overall survival using individual data. We reviewed the medical records of patients with advanced hepatocellular carcinoma treated with sorafenib. Survival after sorafenib treatment and overall survival were defined as the time when we discovered that patients were either refractory or intolerant to sorafenib and the period from the start of sorafenib treatment, respectively, until death during the study. We compared patients’ prognoses according to their subsequent treatment as follows: group A, therapies targeting intrahepatic lesions; group B, systemic therapies alone; group C, no subsequent therapy. We used linear regression analysis to determine whether there was an association with survival after sorafenib treatment and with overall survival. Of 79 patients, 63 (79.7 %) received one or more subsequent therapies (44 and 19 patients in groups A and B, respectively). The five patients who survived more than two years after sorafenib treatment was discontinued responded to therapies targeting intrahepatic lesions. The median survival times of groups A, B, and C were 11.9 months, 5.8 months, and 3.6 months, respectively. Multivariate analysis revealed that group A, Child-Pugh score, serum α-fetoprotein level, and cause of failure of sorafenib treatment were independent prognostic factors for survival after sorafenib treatment. Individual survival after sorafenib treatment correlated highly with overall survival. Targeting intrahepatic lesions may be useful for treating patients with advanced hepatocellular carcinoma patients after sorafenib treatment is discontinued. The online version of this article (doi:10.1186/s12885-016-2380-4) contains supplementary material, which is available to authorized users

  4. Is Local Radiotherapy Still Valuable for Patients With Multiple Intrahepatic Hepatocellular Carcinomas?

    International Nuclear Information System (INIS)

    Koom, Woong Sub; Seong, Jinsil; Han, Kwang Hyub; Lee, Do Yun; Lee, Jong Tae

    2010-01-01

    Purpose: To investigate whether local radiotherapy (RT) is valuable for patients with multiple hepatocellular carcinomas (HCCs). Methods and Materials: From July 1992 to August 2006, 107 patients with unresectable HCC were treated with local RT after incomplete transcatheter arterial chemoembolization (TACE). The RT field included a main tumor with or without other tumor nodules, depending on the effectiveness of TACE. The median RT dose was 50.4Gy in conventional fractionation. Patients were categorized into four groups: Group 1, single tumor (39 patients); Group 2, multiple tumors within the RT field (25 patients); Group 3, controlled tumors out of the RT field (19 patients); and Group 4, tumors that remained viable out of the RT field (24 patients). Results: Group 1 showed the best survival rate (MST, 35 months; 2-year OS, 60%) and Group 4 the worst (MST, 5 months; 2-year OS, 16%). Group 2 and Group 3 showed similar survival (MST, 13 vs. 19 months; 2-year OS, 35% vs. 46%; p = 0.698). Significantly worse intrahepatic control in Group 4 was observed. The survival in Groups 2 and 3 (MST, 16 months) was significantly different from that in Group 4 (p = 0.004), and was marginally significant compared with that in Group 1 (p = 0.051). Conclusions: Local RT to the main tumor could be applicable in well-controlled intrahepatic tumors out of the RT field. Patients with viable intrahepatic tumors out of the RT field showed worse survival. In future clinical trials, these patients need to be excluded.

  5. Intrahepatic Left to Right Portoportal Venous Collateral Vascular Formation in Patients Undergoing Right Portal Vein Ligation

    Energy Technology Data Exchange (ETDEWEB)

    Lienden, K. P. van, E-mail: k.p.vanlienden@amc.uva.nl [Academic Medical Center, University of Amsterdam, Department of Interventional Radiology (Netherlands); Hoekstra, L. T. [Academic Medical Center, University of Amsterdam, Department of Surgery (Netherlands); Bennink, R. J. [Academic Medical Center, University of Amsterdam, Department of Nuclear Medicine (Netherlands); Gulik, T. M. van [Academic Medical Center, University of Amsterdam, Department of Surgery (Netherlands)

    2013-12-15

    Purpose: We investigated intrahepatic vascular changes in patients undergoing right portal vein ligation (PVL) or portal vein embolization (PVE) in conjunction with the ensuing hypertrophic response and function of the left liver lobe. Methods: Between December 2008 and October 2011, 7 patients underwent right PVL and 14 patients PVE. Computed tomographic (CT) volumetry to assess future remnant liver (FRL) and functional hepatobiliary scintigraphy were performed in all patients before and 3 weeks after portal vein occlusion. In 18 patients an intraoperative portography was performed to assess perfusion through the occluded portal branches. Results: In all patients after initially successful PVL, reperfused portal veins were observed on CT scan 3 weeks after portal occlusion. This was confirmed in all cases during intraoperative portography. Intrahepatic portoportal collaterals were identified in all patients in the PVL group and in one patient in the PVE group. In all other PVE patients, complete occlusion of the embolized portal branches was observed on CT scan and on intraoperative portography. The median increase of FRL volume after PVE was 41.6 % (range 10-305 %), and after PVL was only 8.1 % (range 0-102 %) (p = 0.179). There were no differences in FRL function between both groups. Conclusion: Preoperative PVE and PVL are both methods to induce hypertrophy of the FRL in anticipation of major liver resection. Compared to PVE, PVL seems less efficient in inducing hypertrophy of the nonoccluded left lobe. This could be caused by the formation of intrahepatic portoportal neocollateral vessels, through which the ligated portal branches are reperfused within 3 weeks.

  6. Anabolic Androgen-induced Intrahepatic Cholestasis Presented With Normal AND#947;-Glutamyl-Transpeptidase

    Directory of Open Access Journals (Sweden)

    Savvoula Savvidou

    2014-04-01

    A case report of a young male with remarkable jaundice due to acute anabolic androgen-induced cholestasis is presented. Interestingly, and #947;-glutamyl transpeptidase remained normal throughout the patient's diagnostic workup. Histopathology was indicative of pure, and ldquo;bland and rdquo; intrahepatic cholestasis with minimal inflammation but significant fibrosis. The patient was successfully treated with ursodeoxycholic acid and glucocorticosteroids. The significance of normal and #947;-glutamyl transpeptidase along with the histopathological findings and the possible pathophysiological mechanisms are finally discussed. [J Interdiscipl Histopathol 2014; 2(2.000: 98-103

  7. Severe jaundice due to intrahepatic cholestasis after initiating anticoagulation with rivaroxaban.

    Science.gov (United States)

    Aslan, Abdullah N; Sari, Cenk; Baştuğ, Serdal; Sari, Sevil Ö; Akçay, Murat; Durmaz, Tahir; Bozkurt, Engin

    2016-03-01

    Rivaroxaban, a highly selective direct factor Xa inhibitor, is a new oral anticoagulant approved by the US Food and Drug Administration in November 2011 for stroke prophylaxis in patients with nonvalvular atrial fibrillation. Because of its efficacy and once-a-day dosing, it is commonly preferred in patients with nonvalvular atrial fibrillation and intolerance to warfarin in clinical practice. However, it can result in some adverse effects such as bleeding, rashes and liver injury. Here, we described a very rare adverse reaction of rivaroxaban, jaundice due to intrahepatic cholestasis, appeared in a 71-year-old male patient after taking rivaroxaban.

  8. A fatal case of primary basaloid squamous cell carcinoma in the intrahepatic bile ducts

    DEFF Research Database (Denmark)

    Kirkegaard, Johan; Grunnet, Mie; Hasselby, Jane Preuss

    2014-01-01

    of diagnosis but expired 20 months after surgery with epidural, lung, and spine metastasis. In addition to the unusual clinical presentation, the diagnosis of the liver tumor was that of a primary basaloid squamous cell carcinoma of the intrahepatic bile ducts, an entity with only one previous report......Primary squamous cholangiocellular carcinomas are very rare. We describe a case of a 67-year-old man, who underwent chemotherapy and surgery for a right-sided liver tumor with an unusual presentation of metastasis to a lymph node in the left armpit. The patient was asymptomatic at the time...

  9. Intrahepatic Portosystemic Venous Shunt: Successful Embolization Using the Amplatzer Vascular Plug II

    Energy Technology Data Exchange (ETDEWEB)

    Lee, Young Ju; Shin, Byung Seok; Lee, In Ho; Ohm, Joon Young; Lee, Byung Seok; Ahn, Moon Sang [Chungnam National University Hospital, Chungnam National University College of Medicine, Daejeon (Korea, Republic of); Kim, Ho Jun [Dept. of Radiology, Konyang University Hospital, Konyang University College of Medicine, Daejeon (Korea, Republic of)

    2012-11-15

    A 67-year-old woman presented with memory impairment and behavioral changes. Brain MRI indicated hepatic encephalopathy. Abdominal CT scans revealed an intrahepatic portosystemic venous shunt that consisted of two shunt tracts to the aneurysmal sac that communicated directly with the right hepatic vein. The large tract was successfully occluded by embolization using the newly available AMPLATZERTM Vascular Plug II and the small tract was occluded by using coils. The patient's symptoms disappeared after shunt closure and she remained free of recurrence at the 3-month follow-up evaluation.

  10. Pancreaticoportal Fistula and Disseminated Fat Necrosis After Revision of a Transjugular Intrahepatic Portosystemic Shunt

    International Nuclear Information System (INIS)

    Klein, Seth J.; Saad, Nael; Korenblat, Kevin; Darcy, Michael D.

    2013-01-01

    A 59-year old man with alcohol related cirrhosis and portal hypertension was referred for transjugular intrahepatic portosystemic shunt (TIPS) to treat his refractory ascites. Ten years later, two sequential TIPS revisions were performed for shunt stenosis and recurrent ascites. After these revisions, he returned with increased serum pancreatic enzyme levels and disseminated superficial fat necrosis; an iatrogenic pancreaticoportal vein fistula caused by disruption of the pancreatic duct was suspected. The bare area of the TIPS was subsequently lined with a covered stent-graft, and serum enzyme levels returned to baseline. In the interval follow-up period, the patient has clinically improved.

  11. Imaging Findings of Intrahepatic Bile Duct Adenoma (Peribiliary Gland Hamartoma): a Case Report and Literature Review

    Energy Technology Data Exchange (ETDEWEB)

    Kim, You Sung; Rha, Sung Eun; Oh, Soon Nam; Jung, Seung Eun; Shin, Yu Ri; Choi, Byung Gil; Byun, Jae Young; Jung, Eun Sun; Kim, Dong Goo [Catholic University of Korea, Seoul St.Mary' s Hospital, Seoul (Korea, Republic of)

    2010-10-15

    Intrahepatic bile duct adenoma is a rare benign epithelial hepatic tumor derived from bile duct cells. We report the imaging findings of a patient with bile duct adenoma, which appeared as a small heterogeneously enhancing mass with focal small cystic change on CT and MRI. Follow-up images at seven months showed a slight increase in tumor size, which could be partly explained by intratumoral hemorrhage on pathologic examination. Although rare, bile duct adenoma should be considered as a differential diagnosis of a small hypervascular tumor located in the periphery of liver. Focal cystic change and intratumoral hemorrhage may occur

  12. Diffusion weighted MRI in intrahepatic bile duct adenoma arising from the cirrhotic liver

    Energy Technology Data Exchange (ETDEWEB)

    An, Chansik [Research Institute of Radiological Science, Severance Hospital, Yonsei University College of Medicine, Seoul (Korea, Republic of); Park, Sumi; Choi, Yoon Jung [National Health Insurance Corporation Ilsan Hospital, Goyang (Korea, Republic of)

    2013-10-15

    A 64-year-old male patient with liver cirrhosis underwent a CT study for hepatocellular carcinoma surveillance, which demonstrated a 1.4-cm hypervascular subcapsular tumor in the liver. On gadoxetic acid-enhanced MRI, the tumor showed brisk arterial enhancement and persistent hyperenhancement in the portal phase, but hypointensity in the hepatobiliary phase. On diffusion-weighted MRI, the tumor showed an apparent diffusion coefficient twofold greater than that of the background liver parenchyma, which suggested that the lesion was benign. The histologic diagnosis was intrahepatic bile duct adenoma with alcoholic liver cirrhosis.

  13. Diffusion-weighted MRI in intrahepatic bile duct adenoma arising from the cirrhotic liver.

    Science.gov (United States)

    An, Chansik; Park, Sumi; Choi, Yoon Jung

    2013-01-01

    A 64-year-old male patient with liver cirrhosis underwent a CT study for hepatocellular carcinoma surveillance, which demonstrated a 1.4-cm hypervascular subcapsular tumor in the liver. On gadoxetic acid-enhanced MRI, the tumor showed brisk arterial enhancement and persistent hyperenhancement in the portal phase, but hypointensity in the hepatobiliary phase. On diffusion-weighted MRI, the tumor showed an apparent diffusion coefficient twofold greater than that of the background liver parenchyma, which suggested that the lesion was benign. The histologic diagnosis was intrahepatic bile duct adenoma with alcoholic liver cirrhosis.

  14. Cardiac and renal effects of a transjugular intrahepatic portosystemic shunt in cirrhosis

    DEFF Research Database (Denmark)

    Busk, Troels M; Bendtsen, Flemming; Møller, Søren

    2013-01-01

    Refractory ascites and recurrent variceal bleeding are among the serious complications of portal hypertension and cirrhosis for which a transjugular intrahepatic portosystemic shunt (TIPS) can be used. Cirrhotic patients have varying degrees of haemodynamic derangement, mainly characterized...... to improve in patients with the hepatorenal syndrome. The clinical and haemodynamic effects of TIPS have been studied intensively and will be reviewed in the present paper. Considerable knowledge on the effects of TIPS on the pathophysiology of cirrhosis has been gained, but studies on the central...

  15. Hepatic Veins and Inferior Vena Cava Thrombosis in a Child Treated by Transjugular Intrahepatic Portosystemic Shunt

    International Nuclear Information System (INIS)

    Carnevale, Francisco Cesar; Santos, Aline Cristine Barbosa; Tannuri, Uenis; Cerri, Giovanni Guido

    2010-01-01

    We report the case of a 9-year-old boy with portal hypertension, due to Budd-Chiari syndrome, and retrohepatic inferior vena cava thrombosis, submitted to a transjugular intrahepatic portosystemic shunt (TIPS) by connecting the suprahepatic segment of the inferior vena cava directly to the portal vein. After 3 months, the withdrawal of anticoagulants promoted the thrombosis of the TIPS. At TIPS revision, thrombosis of the TIPS and the main portal vein and clots at the splenic and the superior mesenteric veins were found. Successful angiography treatment was performed by thrombolysis and balloon angioplasty of a severe stenosis at the distal edge of the stent.

  16. RE: Endovascular Treatment of Congenital Intrahepatic Portosystemic Shunts with Amplatzer Plugs

    International Nuclear Information System (INIS)

    Sierre, Sergio; Alonso, Jose; Lipsich, Jose

    2012-01-01

    In our paper entitled 'Endovascular treatment of congenital portal vein fistulas with the Amplatzer occlusion device' published in the Journal of Vascular and Interventional Radiology in 2004, we already reported the use of the AVP in the treatment of an intrahepatic portosystemic venous shunt. This situation does not undervalue the quality of the reported case, but for didactic purposes, we believe it is important to state that the work of Dr. Lee confirms, as was previously reported, that these devices are useful and safe for these rare situations.

  17. RE: Endovascular Treatment of Congenital Intrahepatic Portosystemic Shunts with Amplatzer Plugs

    Energy Technology Data Exchange (ETDEWEB)

    Sierre, Sergio; Alonso, Jose; Lipsich, Jose [Hospital Nacional de Pediatria ' JP Garrahan' , Combate de los Pozos, Buenos (Argentina)

    2012-01-15

    In our paper entitled 'Endovascular treatment of congenital portal vein fistulas with the Amplatzer occlusion device' published in the Journal of Vascular and Interventional Radiology in 2004, we already reported the use of the AVP in the treatment of an intrahepatic portosystemic venous shunt. This situation does not undervalue the quality of the reported case, but for didactic purposes, we believe it is important to state that the work of Dr. Lee confirms, as was previously reported, that these devices are useful and safe for these rare situations.

  18. An intrahepatic calculus superimposed over the right renal shadow: a case of mistaken identity.

    Science.gov (United States)

    Learney, Robert M; Shrotri, Nitin

    2010-08-01

    A 36-year-old Caucasian British woman presented with a classic case of right renal colic. Initial plain abdominal radiography and intravenous urography identified an 8 x 5 mm calculus apparently lying within a right lower pole calyx. Following failed extracorporeal lithotripsy and flexible ureterorenoscopy, cross-sectional imaging revealed a misdiagnosis by superposition of an intrahepatic calculus over the right renal shadow. This case serves to support cross-sectional imaging in the diagnosis of renal calculi. Copyright 2010 Elsevier Inc. All rights reserved.

  19. Pancreaticoportal Fistula and Disseminated Fat Necrosis After Revision of a Transjugular Intrahepatic Portosystemic Shunt

    Energy Technology Data Exchange (ETDEWEB)

    Klein, Seth J., E-mail: kleins@mir.wustl.edu; Saad, Nael [Washington University School of Medicine, Interventional Radiology Section, Mallinckrodt Institute of Radiology (United States); Korenblat, Kevin [Washington University School of Medicine, Division of Gastroenterology, Department of Internal Medicine (United States); Darcy, Michael D. [Washington University School of Medicine, Interventional Radiology Section, Mallinckrodt Institute of Radiology (United States)

    2013-04-15

    A 59-year old man with alcohol related cirrhosis and portal hypertension was referred for transjugular intrahepatic portosystemic shunt (TIPS) to treat his refractory ascites. Ten years later, two sequential TIPS revisions were performed for shunt stenosis and recurrent ascites. After these revisions, he returned with increased serum pancreatic enzyme levels and disseminated superficial fat necrosis; an iatrogenic pancreaticoportal vein fistula caused by disruption of the pancreatic duct was suspected. The bare area of the TIPS was subsequently lined with a covered stent-graft, and serum enzyme levels returned to baseline. In the interval follow-up period, the patient has clinically improved.

  20. Recanalization of an Occluded Intrahepatic Portosystemic Covered Stent via the Percutaneous Transhepatic Approach

    Energy Technology Data Exchange (ETDEWEB)

    Chan, Chih Yang; Liang, Po Chin [National Taiwan University Hospital, Taipei (China)

    2010-08-15

    A 41-year-old woman with liver cirrhosis had recurrent portal hypertension and bleeding from esophageal varices due to complete occlusion of a previously inserted transjugular intrahepatic portosystemic shunt stent. Because recanalization of the stent by the transjugular approach was unsuccessful, ultrasound-guided entry to the splenic vein and portal vein was used. After catheter-directed intrathrombus thrombolysis, successful opening of the stent was achieved and a stent was placed. We herein report a rare case in which thrombolysis and recanalization of a TIPS stent were performed via a percutaneous transhepatic approach

  1. Blood morphology and the levels of selected cytokines related to hematopoiesis in occupational short-term exposure to lead

    Energy Technology Data Exchange (ETDEWEB)

    Dobrakowski, Michał, E-mail: michal.dobrakowski@poczta.fm [Department of Biochemistry, School of Medicine with the Division of Dentistry, Medical University of Silesia, ul. Jordana 19, 41-808 Zabrze (Poland); Boroń, Marta [Institute of Occupational Medicine and Environmental Health in Sosnowiec, ul. Kościelna 13, 41-200 Sosnowiec (Poland); Czuba, Zenon P. [Department of Microbiology and Immunology, School of Medicine with the Division of Dentistry, Medical University of Silesia, ul. Jordana 19, 41-808 Zabrze (Poland); Birkner, Ewa [Department of Biochemistry, School of Medicine with the Division of Dentistry, Medical University of Silesia, ul. Jordana 19, 41-808 Zabrze (Poland); Chwalba, Artur [SP ZOZ Municipal Hospital, Department of Internal Medicine, ul. Strzelców Bytomskich 11, 41-500 Chorzów (Poland); Hudziec, Edyta; Kasperczyk, Sławomir [Department of Biochemistry, School of Medicine with the Division of Dentistry, Medical University of Silesia, ul. Jordana 19, 41-808 Zabrze (Poland)

    2016-08-15

    The aim of the study was to investigate the influence of a short-term exposure to lead on the blood morphology and the levels of selected cytokines related to hematopoiesis in occupationally exposed workers. The study population included 37 males occupationally exposed to lead for 36 to 44 days. Their blood lead level raised from 10.7 ± 7.67 μg/dl at baseline to the level of 49.1 ± 14.1 μg/dl at the end of the study. The level of hemoglobin and values of MCH and MCHC were decreased due to a short-term exposure to lead by 2%, 2%, and 1%, respectively. The counts of WBC, LYM, and MXD increased significantly by 5%, 7%, and 35%. Similarly, the count of PLT increased by 7%, while PDW, MPV, and P-LCR decreased by 6%, 3%, and 9%, respectively. The levels of IL-7, G-CSF, HGF, PDGF AB/BB, SCF, and PECAM-1, decreased significantly by 30%, 33%, 8%, 30%, 25%, and 20%, respectively. A short-term occupational exposure to lead results in a decreased hemoglobin level and increased counts of WBC and PLT. Changes in counts and proportions of different types of leukocytes and decreased values of PLT indices, such as PDW, MPV, and P-LCR, due to the subacute lead-exposure may be associated with lead-induced decreased levels of cytokines related to hematopoiesis, including SCF, G-CSF, IL-7, and PDGF. - Highlights: • Subacute exposure to lead results in a decreased hemoglobin level. • Subacute exposure to lead results in increased counts of WBC and PLT. • Subacute exposure to lead decreases the levels of SCF, G-CSF, IL-7, and PDGF.

  2. Blood morphology and the levels of selected cytokines related to hematopoiesis in occupational short-term exposure to lead

    International Nuclear Information System (INIS)

    Dobrakowski, Michał; Boroń, Marta; Czuba, Zenon P.; Birkner, Ewa; Chwalba, Artur; Hudziec, Edyta; Kasperczyk, Sławomir

    2016-01-01

    The aim of the study was to investigate the influence of a short-term exposure to lead on the blood morphology and the levels of selected cytokines related to hematopoiesis in occupationally exposed workers. The study population included 37 males occupationally exposed to lead for 36 to 44 days. Their blood lead level raised from 10.7 ± 7.67 μg/dl at baseline to the level of 49.1 ± 14.1 μg/dl at the end of the study. The level of hemoglobin and values of MCH and MCHC were decreased due to a short-term exposure to lead by 2%, 2%, and 1%, respectively. The counts of WBC, LYM, and MXD increased significantly by 5%, 7%, and 35%. Similarly, the count of PLT increased by 7%, while PDW, MPV, and P-LCR decreased by 6%, 3%, and 9%, respectively. The levels of IL-7, G-CSF, HGF, PDGF AB/BB, SCF, and PECAM-1, decreased significantly by 30%, 33%, 8%, 30%, 25%, and 20%, respectively. A short-term occupational exposure to lead results in a decreased hemoglobin level and increased counts of WBC and PLT. Changes in counts and proportions of different types of leukocytes and decreased values of PLT indices, such as PDW, MPV, and P-LCR, due to the subacute lead-exposure may be associated with lead-induced decreased levels of cytokines related to hematopoiesis, including SCF, G-CSF, IL-7, and PDGF. - Highlights: • Subacute exposure to lead results in a decreased hemoglobin level. • Subacute exposure to lead results in increased counts of WBC and PLT. • Subacute exposure to lead decreases the levels of SCF, G-CSF, IL-7, and PDGF.

  3. Basic anatomic study of transjugular intrahepatic portosystemic shunt by direct transcaval approach

    International Nuclear Information System (INIS)

    Wu Xia; Xu Ke

    2007-01-01

    Objective: To investigate the indications and related anatomic foundation of transjugular intrahepatic portosystemic shunt (TIPS) through direct transcaval approach, and to evaluate the safety, feasibility and clinical significance. Methods: Sixty four patients diagnosed as hepatocirrhosis clinically were involved, including the function of liver Child B (n=40), Child C (n=24). After 2 phrase of hepatic CT enhancement scanning and postprocessing through multiple planner reconstruction (MPR) and curve planner reconstruction (CPR), the data were conjugated statistically by ANOVA. Results: The length of the intrahepatic segment of the inferior cava in Child B is longer than that in Child C (P<0.05). Referring the points of hepatic vein entrance to vena cava as A1, 2 cm away from right hepatic vein as A2, the crotch of portal vein as B1, 2 cm away from right portal vein as B2. The length of A1B1 is shorter than that of A2B1(P<0.05). The angle between A1B2 and right portal vein is smaller than that of A2B2 and right portal vein (P<0.05). Conclusion: Transcaval TIPS creation is safe and feasible, providing the direct transcaval approach as a favorable fluent patency way and less influence on hemodynamics in comparison with traditional TIPS. (authors)

  4. Dilatation of the intrahepatic bile ducts associated with benign liver lesions: an unusual finding

    International Nuclear Information System (INIS)

    Lapeyre, Matthieu; Mathieu, Didier; Rahmouni, Alain; Kobeiter, Hicham; Tailboux, Laurent

    2002-01-01

    In three patients presenting different types of liver lesions, including isolated cyst, focal nodular hyperplasia (FNH), and hemangioma, intrahepatic bile duct dilatation was observed on US and CT. Final diagnosis was obtained by surgery in two cases (cyst and FNH) and by 1-year follow-up in one patient presenting an isolated hemangioma. The only common characteristic in our three cases was that lesions were present in segment four according to Couinaud's classification, at the level of the transverse fissure, suggesting that a space-occupying lesion at this site may cause compression of the common hepatic duct and right or left intrahepatic bile ducts. Our report indicates that compression may occur even with lesion of moderate size (35-40 mm in diameter). A benign liver lesion may cause a bile duct dilatation, particularly if located in segment 4, close to the hilum. Awareness of this possibility is important to avoid unnecessary invasive diagnostic procedures, particularly when all imaging criteria are consistent with a benign lesion. (orig.)

  5. Tumors with intrahepatic bile duct differentiation in cirrhosis: implications on outcomes after liver transplantation.

    Science.gov (United States)

    Facciuto, Marcelo E; Singh, Manoj K; Lubezky, Nir; Selim, Motaz A; Robinson, Dorothy; Kim-Schluger, Leona; Florman, Sander; Ward, Stephen C; Thung, Swan N; Fiel, MariaIsabel; Schiano, Thomas D

    2015-01-01

    The role of liver transplantation (LT) in the management of cirrhotic patients with tumors exhibiting intrahepatic bile duct differentiation remains controversial. The objective of this study was to characterize the spectrum of these tumors and analyze post-LT outcomes. Retrospective pathology database search of explant histology analysis of liver transplants between April 1993 and November 2013. Thirty-two patients were analyzed, 75% were men with a mean age of 60 years. Seven patients had nodules demonstrating intrahepatic cholangiocarcinoma (I-CCA), nine had I-CCA nodules occurring concomitantly with hepatocellular carcinoma (HCC), and 16 had mixed HCC-CCA nodules. The median number of tumors was 1 and size was 2.5 cm. Overall patient survival post-LT at 1 and 5 years was 71% and 57%, respectively. Patients within Milan criteria, especially with I-CCA features, showed a 5-year tumor recurrence rate (10%) and 5-year survival rate (78%) comparable with other patients having HCC within Milan criteria. This series showed that patients with CCA within Milan criteria may be able to achieve acceptable long-term post-LT survival.

  6. An Imaging and Histological Study on Intrahepatic Microvascular Passage of Contrast Materials in Rat Liver

    Directory of Open Access Journals (Sweden)

    Qian Xia

    2017-01-01

    Full Text Available Background. Lipiodol has been applied for decades in transarterial chemoembolization to treat liver malignancies, but its intrahepatic pathway through arterioportal shunt (APS in the liver has not been histologically revealed. This rodent experiment was conducted to provide evidence for the pathway of Lipiodol delivered through the hepatic artery (HA but found in the portal vein (PV and to elucidate the observed unidirectional APS. Methods. Thirty rats were divided into 5 groups receiving systemic or local arterial infusion of red-stained iodized oil (RIO or its hydrosoluble substitute barium sulfate suspension (BSS, or infusion of BSS via the PV, monitored by real-time digital radiography. Histomorphology of serial frozen and paraffin sections was performed and quantified. Results. After HA infusion, RIO and BSS appeared extensively in PV lumens with peribiliary vascular plexus (PVP identified as the responsible anastomotic channel. After PV infusion, BSS appeared predominantly in the PV and surrounding sinusoids and to a much lesser extent in the PVP and HA (P<0.001. Fluid mechanics well explains the one-way-valve phenomenon of APS. Conclusions. Intravascularly injected rat livers provide histomorphologic evidences: (1 the PVP exists in between the HA and PV, which is responsible to the APS of Lipiodol; and (2 the intrahepatic vascular inflow appears HA-PVP-PV unidirectional without a physical one-way valve, which can be postulated by the fluid mechanics.

  7. Intrahepatic fat, abdominal adipose tissues, and metabolic state: magnetic resonance imaging study.

    Science.gov (United States)

    Yaskolka Meir, Anat; Tene, Lilac; Cohen, Noa; Shelef, Ilan; Schwarzfuchs, Dan; Gepner, Yftach; Zelicha, Hila; Rein, Michal; Bril, Nitzan; Serfaty, Dana; Kenigsbuch, Shira; Chassidim, Yoash; Sarusy, Benjamin; Dicker, Dror; Thiery, Joachim; Ceglarek, Uta; Stumvoll, Michael; Blüher, Matthias; Stampfer, Meir J; Rudich, Assaf; Shai, Iris

    2017-07-01

    Intrahepatic fat (IHF) is best known to associate with waist circumference (WC) and visceral adipose tissue (VAT), but its relation to abdominal subcutaneous adipose tissue is controversial. While IHF ≥ 5% dichotomously defines fatty liver, %IHF is rarely considered as a continuous variable that includes the normal range. In this study, we aimed to evaluate %IHF association with abdominal fat subdepots, pancreatic, and renal-sinus fats. We evaluated %IHF, abdominal fat subdepots, %pancreatic, and renal-sinus fats, among individuals with moderate abdominal obesity, using 3-Tesla magnetic resonance imaging. Among 275 participants, %IHF widely ranged (0.01%-50.4%) and was lower in women (1.6%) than men (7.3%; P fat was positively associated with %IHF (P = .005). In an age, sex, WC, and VAT-adjusted models, elevated liver enzymes, glycemic, lipid, and inflammatory biomarkers were associated with increased %IHF (P fat is differentially associated with abdominal fat subdepots. Intrahepatic-fat as a continuous variable could be predicted by specific traditional parameters, even within the current normal range, and partially independent of VAT. Copyright © 2017 John Wiley & Sons, Ltd.

  8. A novel method for murine intrahepatic islet transplantation via cecal vein.

    Science.gov (United States)

    Byun, Nari; Kim, Hyun-Je; Min, Byoung-Hoon; Shin, Jun-Seop; Yoon, Il-Hee; Kim, Jong-Min; Kim, Yong-Hee; Park, Chung-Gyu

    2015-12-01

    Islet transplantation is one of the most beneficial treatment modality to treat type 1 diabetic patients with frequent hypoglycemic unawareness. In clinical setting, human islets are infused via portal vein and are settled in the end-portal venules in the liver. However, mouse islets are transplanted into kidney subcapsule or liver through direct portal vein. These conventional transplantation methods have several drawbacks such as different physiological environments around the transplanted islets in kidney subcapsule from the liver and high mortality rate in direct portal vein approach. In this study, we introduced murine intrahepatic islet transplantation method via cecal vein to have the same surgical operation route in humans as well as guaranteeing low mortality rate after islet transplantation. With this protocol, consistent normoglycemia can be obtained in diabetic mice, while keeping operation-related mortality extremely low. This approach with easier accessibility and low mortality will make murine intrahepatic islet transplantation a useful model for studying immunological mechanisms such as strong innate and adaptive immune responses that occur in human islet transplantation. Copyright © 2015 Elsevier B.V. All rights reserved.

  9. Biological impact of geometric uncertainties: what margin is needed for intra-hepatic tumors?

    International Nuclear Information System (INIS)

    Kuo, Hsiang-Chi; Liu, Wen-Shan; Wu, Andrew; Mah, Dennis; Chuang, Keh-Shih; Hong, Linda; Yaparpalvi, Ravi; Guha, Chandan; Kalnicki, Shalom

    2010-01-01

    To evaluate and compare the biological impact on different proposed margin recipes for the same geometric uncertainties for intra-hepatic tumors with different tumor cell types or clinical stages. Three different margin recipes based on tumor motion were applied to sixteen IMRT plans with a total of twenty two intra-hepatic tumors. One recipe used the full amplitude of motion measured from patients to generate margins. A second used 70% of the full amplitude of motion, while the third had no margin for motion. The biological effects of geometric uncertainty in these three situations were evaluated with Equivalent Uniform Doses (EUD) for various survival fractions at 2 Gy (SF 2 ). There was no significant difference in the biological impact between the full motion margin and the 70% motion margin. Also, there was no significant difference between different tumor cell types. When the margin for motion was eliminated, the difference of the biological impact was significant among different cell types due to geometric uncertainties. Elimination of the motion margin requires dose escalation to compensate for the biological dose reduction due to the geometric misses during treatment. Both patient-based margins of full motion and of 70% motion are sufficient to prevent serious dosimetric error. Clinical implementation of margin reduction should consider the tumor sensitivity to radiation

  10. Volumetric parameters on FDG PET can predict early intrahepatic recurrence-free survival in patients with hepatocellular carcinoma after curative surgical resection

    Energy Technology Data Exchange (ETDEWEB)

    Lee, Jeong Won [Catholic Kwandong University College of Medicine, Department of Nuclear Medicine, Incheon (Korea, Republic of); Hwang, Sang Hyun; Kim, Hyun Jeong; Kim, Dongwoo; Cho, Arthur; Yun, Mijin [Yonsei University College of Medicine, Department of Nuclear Medicine, Seoul (Korea, Republic of)

    2017-11-15

    This study assessed the prognostic values of volumetric parameters on {sup 18}F-fluorodeoxyglucose (FDG) positron emission tomography (PET) in predicting early intrahepatic recurrence-free survival (RFS) after curative resection in patients with hepatocellular carcinoma (HCC). A retrospective analysis was performed on 242 patients with HCC who underwent staging FDG PET and subsequent curative surgical resection. The tumor-to-non-tumorous liver uptake ratio, metabolic tumor volume (MTV), and total lesion glycolysis (TLG) of the HCC lesions on PET were measured. The prognostic values of clinical factors and PET parameters for predicting overall RFS, overall survival (OS), extrahepatic RFS, and early and late intrahepatic RFS were assessed. The median follow-up period was 54.7 months, during which 110 patients (45.5%) experienced HCC recurrence and 62 (25.6%) died. Patients with extrahepatic and early intrahepatic recurrence showed worse OS than did those with no recurrence or late intrahepatic recurrence (p < 0.001). Serum bilirubin level, MTV, and TLG were independent prognostic factors for overall RFS and OS (p < 0.05). Only MTV and TLG were prognostic for extrahepatic RFS (p < 0.05). Serum alpha-fetoprotein and bilirubin levels, MTV, and TLG were prognostic for early intrahepatic RFS (p < 0.05) and hepatitis C virus (HCV) positivity and serum albumin level were independently prognostic for late intrahepatic RFS (p < 0.05). Intrahepatic recurrence showed different prognoses according to the time interval of recurrence in which early recurrence had as poor survival as extrahepatic recurrence. MTV and TLG on initial staging PET were significant independent factors for predicting early intrahepatic and extrahepatic RFS in patients with HCC after curative resection. Only HCV positivity and serum albumin level were significant for late intrahepatic RFS, which is mainly attributable to the de novo formation of new primary HCC. (orig.)

  11. Rescue of defective ATP8B1 trafficking by CFTR correctors as a therapeutic strategy for familial intrahepatic cholestasis

    NARCIS (Netherlands)

    van der Woerd, Wendy L.; Wichers, Catharina G. K.; Vestergaard, Anna L.; Andersen, Jens Peter; Paulusma, Coen C.; Houwen, Roderick H. J.; van de Graaf, Stan F. J.

    Background & Aims ATP8B1 deficiency is an autosomal recessive liver disease characterized by intrahepatic cholestasis. ATP8B1 mutation p.I661T, the most frequent mutation in European patients, results in protein misfolding and impaired targeting to the plasma membrane. Similarly, mutations in cystic

  12. Analysis of intrahepatic HBV-specific cytotoxic T-cells during and after acute HBV infection in humans

    NARCIS (Netherlands)

    Sprengers, Dave; van der Molen, Renate G.; Kusters, Johannes G.; de Man, Robert A.; Niesters, Hubert G. M.; Schalm, Solko W.; Janssen, Harry L. A.

    2006-01-01

    Characteristics of the intrahepatic virus-specific T-cell response in patients with acute hepatitis B virus (HBV) infection have not been studied due to the risk of complications associated with standard liver biopsies. In this study we aimed to characterize the virus-specific CD8 + T-cell response

  13. Production of an intrahepatic portocaval fistula in the dog with cirrhosis of the liver and portal hypertension

    Energy Technology Data Exchange (ETDEWEB)

    Burgener, F A; Gutierrez, O H

    1984-09-01

    Non-Operative intrahepatic shunts between the left branch of the portal vein and hepatic vein were produced in 14 dogs with periportal cirrhosis and portal hypertension. Dilatation of the intrahepatic fistula was achieved with a Gruentzig catheter with a balloon diameter of 12 m. The catheter was introduced into the left branch of the portal vein passing through the right jugular vein, vena cava and hepatic vein by means of a co-axial system, using a stiff Teflon catheter with an external diameter of 6 mm. Repeated maximal inflation of the balloon, which was placed across the portal vein and inferior vena cava, produced an intra-hepatic portocaval shunt in all dogs. This resulted in immediate abolition of previously stable portal hypertension. The only significant complication was shunt occlusion within a week. In such cases the shunt was reopened by the same technique. The procedure had to be repeated at weekly intervals up to five times before a permanent intrahepatic shunt was established.

  14. Decrease of deleted in malignant brain tumour-1 (DMBT-1) expression is a crucial late event in intrahepatic cholangiocarcinoma

    DEFF Research Database (Denmark)

    Sasaki, M; Huang, S-F; Chen, M-F

    2003-01-01

    AIMS: To investigate the participation of DMBT-1, a candidate tumour suppressor gene, in the development of intrahepatic cholangiocarcinoma via intraductal papillary neoplasm of the liver (IPN-L) arising in hepatolithiasis. DMBT-1 plays a role in mucosal immune defence. METHODS AND RESULTS: The e...

  15. Intrahepatic tissue pO2 during continuous or intermittent vascular inflow occlusion in a pig liver resection model

    NARCIS (Netherlands)

    van Wagensveld, B. A.; van Gulik, T. M.; Gabeler, E. E.; van der Kleij, A. J.; Obertop, H.; Gouma, D. J.

    1998-01-01

    BACKGROUND: Temporary vascular inflow occlusion of the liver (clamping of the hepatic pedicle) can prevent massive blood loss during liver resections. In this study, intrahepatic tissue pO2 was assessed as parameter of microcirculatory disturbances induced by ischemia and reperfusion (I/R) in the

  16. Cholecystectomy of an Intrahepatic Gallbladder in an Ectopic Pelvic Liver: A Case Report and Review of the Literature

    Directory of Open Access Journals (Sweden)

    Rachel Mathis

    2017-01-01

    Full Text Available Introduction. Ectopic pelvic liver is an exceedingly rare condition usually resulting after repair of congenital abdominal wall defects. Intrahepatic gallbladder is another rare condition predisposing patients to cholelithiasis and its sequelae. We describe a cholecystectomy in a patient with an intrahepatic gallbladder in a pelvic ectopic liver. Presentation of Case. A 33-year-old woman with a history of omphalocele repair as an infant presented with signs and symptoms of symptomatic cholelithiasis and chronic cholecystitis, however, in an unusual location. After extensive workup and symptomatic treatment, cholecystectomy was recommended and performed via laparotomy and hepatotomy using microwave technology for parenchymal hepatic transection. Discussion. Given the rare combination of an intrahepatic gallbladder and an ectopic pelvic liver, advanced surgical techniques must be employed for cholecystectomies, in addition to involvement of hepatobiliary experienced surgeons due to the distortion of the biliary and hepatic vascular anatomy. Conclusion. Cholecystectomy by experienced hepatobiliary surgeons is a safe and effective treatment for cholecystitis in patients with intrahepatic gallbladders in ectopic pelvic livers.

  17. Rescue of defective ATP8B1 trafficking by CFTR correctors as a therapeutic strategy for familial intrahepatic cholestasis

    NARCIS (Netherlands)

    van der Woerd, Wendy L.; Wichers, Catharina G. K.; Vestergaard, Anna L.; Andersen, Jens Peter; Paulusma, Coen C.; Houwen, Roderick H. J.; van de Graaf, Stan F. J.

    2016-01-01

    ATP8B1 deficiency is an autosomal recessive liver disease characterized by intrahepatic cholestasis. ATP8B1 mutation p.I661T, the most frequent mutation in European patients, results in protein misfolding and impaired targeting to the plasma membrane. Similarly, mutations in cystic fibrosis

  18. Rapid intra-hepatic dissemination of hepatocellular carcinoma with pulmonary metastases following combined loco-regional therapy

    Energy Technology Data Exchange (ETDEWEB)

    Pua, Uei [Dept. of Diagnostic Radiology, Tan Tock Seng Hospital, Singapore (Singapore)

    2013-08-15

    This manuscript describes an unusual case of rapid intra-hepatic dissemination of hepatocellular carcinoma with pulmonary metastases occurring 1 month after combined chemoembolization and radiofrequency ablation. Inferior vena cava and portal vein invasion tumor thrombus was also detected, possibly accounting for the mechanism of disease dissemination route of disease.

  19. Rapid intra-hepatic dissemination of hepatocellular carcinoma with pulmonary metastases following combined loco-regional therapy

    International Nuclear Information System (INIS)

    Pua, Uei

    2013-01-01

    This manuscript describes an unusual case of rapid intra-hepatic dissemination of hepatocellular carcinoma with pulmonary metastases occurring 1 month after combined chemoembolization and radiofrequency ablation. Inferior vena cava and portal vein invasion tumor thrombus was also detected, possibly accounting for the mechanism of disease dissemination route of disease.

  20. Infiltración extramedular en la leucemia mielomonocítica crónica (LMMC Extramedullary infiltration in chronic myelomonocytic leukemia (CMML

    Directory of Open Access Journals (Sweden)

    Onel Ávila Cabrera

    2008-12-01

    Full Text Available La leucemia mielomonocítica crónica (LMMC constituye un proceso oncohematológico de naturaleza mixta, mieloproliferativa y mielodisplásica. Su forma habitual de presentación es consecuencia, generalmente, de las citopenias en sangre periférica (síndrome anémico, infecciones o diátesis hemorrágica. La infiltración extramedular en la LMMC frecuentemente involucra el bazo, hígado, piel y nódulos linfáticos; sin embargo, es poco frecuente su localización en otros sitios. Se describe un paciente de 70 años con el diagnóstico de LMMC con infiltración extramedular en piel (nódulos subcutáneos, paladar duro y testículos; se comentan aspectos diagnósticos, terapéuticos y evolutivos.Chronic myelomonocytic leukemia is an oncohematological process of mixed, myeloproliferative and myelodisplastic nature. Its habitual form of presentation generally results from cytopenias in peripheral blood (anemic syndrome, infections or hemorrhagic diathesis. The extramedullary infiltration in the CMML frequently involves the spleen, the liver, the skin and the lymphatic nodules; however, its localization in other sites is not usual. The case of a 70-year-old patient with diagnosis of CMML and with extramedullary infiltration in the skin (subcutaneous nodules, hard palate and testicles is described. Comments on diagnostic, therapeutic and evolutive aspects are made

  1. Intrahepatic and hilar mass-forming cholangiocarcinoma: Qualitative and quantitative evaluation with diffusion-weighted MR imaging

    Energy Technology Data Exchange (ETDEWEB)

    Fattach, Hassan El, E-mail: hassangreenmed@gmail.com [Department of Abdominal Imaging, Hôpital Lariboisière, Assistance Publique-Hôpitaux de Paris, 2 rue Ambroise Paré, 75010 Paris (France); Dohan, Anthony, E-mail: anthony.dohan@lrb.aphp.fr [Department of Abdominal Imaging, Hôpital Lariboisière, Assistance Publique-Hôpitaux de Paris, 2 rue Ambroise Paré, 75010 Paris (France); Université Paris-Diderot, Sorbonne Paris Cité, 10 Avenue de Verdun, 75010 Paris (France); UMR INSERM 965-Paris 7 “Angiogenèse et recherche translationnelle”, 2 rue Amboise Paré, 75010 Paris (France); Guerrache, Youcef, E-mail: docyoucef05@yahoo.fr [Department of Abdominal Imaging, Hôpital Lariboisière, Assistance Publique-Hôpitaux de Paris, 2 rue Ambroise Paré, 75010 Paris (France); Dautry, Raphael, E-mail: raphael.dautry@lrb.aphp.fr [Department of Abdominal Imaging, Hôpital Lariboisière, Assistance Publique-Hôpitaux de Paris, 2 rue Ambroise Paré, 75010 Paris (France); Université Paris-Diderot, Sorbonne Paris Cité, 10 Avenue de Verdun, 75010 Paris (France); and others

    2015-08-15

    Highlights: • DW-MR imaging helps depicts all intrahepatic or hilar mass-forming cholangiocarcinomas. • DW-MRI provides best conspicuity of intrahepatic or hilar mass-forming cholangiocarcinomas than the other MRI sequences (P < 0.001). • The use of normalized ADC using the liver as reference organ results in the most restricted distribution of ADC values of intrahepatic or hilar mass-forming cholangiocarcinomas (variation coefficient = 16.6%). - Abstract: Objective: To qualitatively and quantitatively analyze the presentation of intrahepatic and hilar mass-forming cholangiocarcinoma with diffusion-weighted magnetic resonance imaging (DW-MRI). Materials and methods: Twenty-eight patients with histopathologically proven mass-forming cholangiocarcinoma (hilar, n = 17; intrahepatic, n = 11) underwent hepatic DW-MRI at 1.5-T using free-breathing acquisition and three b-values (0,400,800 s/mm{sup 2}). Cholangiocarcinomas were evaluated qualitatively using visual analysis of DW-MR images and quantitatively with conventional ADC and normalized ADC measurements using liver and spleen as reference organs. Results: All cholangiocarcinomas (28/28; 100%) were visible on DW-MR images. DW-MRI yielded best conspicuity of cholangiocarcinomas than the other MRI sequences (P < 0.001). Seven cholangiocarcinomas (7/11; 64%) showed hypointense central area on DW-MR images. Conventional ADC value of cholangiocarcinomas (1.042 × 10{sup −3} mm{sup 2}/s ± 0.221 × 10{sup −3} mm{sup 2}/s; range: 0.616 × 10{sup −3} mm{sup 2}/s to 2.050 × 10{sup −3} mm{sup 2}/s) was significantly lower than that of apparently normal hepatic parenchyma (1.362 × 10{sup −3} mm{sup 2}/s ± 0.187 × 10{sup −3} mm{sup 2}/s) (P < 0.0001), although substantial overlap was found. No significant differences in ADC and normalized ADC values were found between intrahepatic and hilar cholangiocarcinomas. The use of normalized ADC using the liver as reference organ resulted in the most restricted

  2. Intrahepatic and hilar mass-forming cholangiocarcinoma: Qualitative and quantitative evaluation with diffusion-weighted MR imaging

    International Nuclear Information System (INIS)

    Fattach, Hassan El; Dohan, Anthony; Guerrache, Youcef; Dautry, Raphael

    2015-01-01

    Highlights: • DW-MR imaging helps depicts all intrahepatic or hilar mass-forming cholangiocarcinomas. • DW-MRI provides best conspicuity of intrahepatic or hilar mass-forming cholangiocarcinomas than the other MRI sequences (P < 0.001). • The use of normalized ADC using the liver as reference organ results in the most restricted distribution of ADC values of intrahepatic or hilar mass-forming cholangiocarcinomas (variation coefficient = 16.6%). - Abstract: Objective: To qualitatively and quantitatively analyze the presentation of intrahepatic and hilar mass-forming cholangiocarcinoma with diffusion-weighted magnetic resonance imaging (DW-MRI). Materials and methods: Twenty-eight patients with histopathologically proven mass-forming cholangiocarcinoma (hilar, n = 17; intrahepatic, n = 11) underwent hepatic DW-MRI at 1.5-T using free-breathing acquisition and three b-values (0,400,800 s/mm 2 ). Cholangiocarcinomas were evaluated qualitatively using visual analysis of DW-MR images and quantitatively with conventional ADC and normalized ADC measurements using liver and spleen as reference organs. Results: All cholangiocarcinomas (28/28; 100%) were visible on DW-MR images. DW-MRI yielded best conspicuity of cholangiocarcinomas than the other MRI sequences (P < 0.001). Seven cholangiocarcinomas (7/11; 64%) showed hypointense central area on DW-MR images. Conventional ADC value of cholangiocarcinomas (1.042 × 10 −3 mm 2 /s ± 0.221 × 10 −3 mm 2 /s; range: 0.616 × 10 −3 mm 2 /s to 2.050 × 10 −3 mm 2 /s) was significantly lower than that of apparently normal hepatic parenchyma (1.362 × 10 −3 mm 2 /s ± 0.187 × 10 −3 mm 2 /s) (P < 0.0001), although substantial overlap was found. No significant differences in ADC and normalized ADC values were found between intrahepatic and hilar cholangiocarcinomas. The use of normalized ADC using the liver as reference organ resulted in the most restricted distribution of ADC values of cholangiocarcinomas (variation

  3. Normal hematopoiesis and lack of β-catenin activation in osteoblasts of patients and mice harboring Lrp5 gain of function mutations

    DEFF Research Database (Denmark)

    Galan-Diez, Marta; Isa, Adiba; Ponzetti, Marco

    2016-01-01

    of hematopoiesis and leukemogenic properties of β-catenin activation in osteoblasts, that lead to development of acute myeloid leukemia (AML). Using mice with gain-of-function (GOF) Lrp5 alleles (Lrp5(A214V)) that recapitulate the human high bone mass (HBM) phenotype, as well as patients with the T253I HBM Lrp5...... mutation, we show here that Lrp5 GOF mutations in both humans and mice do not activate β-catenin signaling in osteoblasts. Consistent with a lack of β-catenin activation in their osteoblasts, Lrp5(A214V) mice have normal trilinear hematopoiesis. In contrast to leukemic mice with constitutive activation...... of β-catenin in osteoblasts (Ctnnb1(CAosb)), accumulation of early myeloid progenitors, a characteristic of AML, myeloid-blasts in blood, and segmented neutrophils or dysplastic megakaryocytes in the bone marrow, are not observed in Lrp5(A214V) mice. Likewise, peripheral blood count analysis in HBM...

  4. In vitro CT evaluation of intrahepatic stones: correlation with chemical composition

    International Nuclear Information System (INIS)

    Kim, Young Jun; Han, Joon Koo; Jeong, Jun Yong; Lee, Kyoung Ho; Kim, Se Hyung; Kim, Young Il; Lee, Jeong Min; Choi, Byung Ihn; Park, Youn-Chan; Kim, Sun-Whe

    2005-01-01

    Objective: To describe in vitro CT features of intrahepatic stones and to correlate CT attenuation with chemical composition. Materials and methods: Of the patients who underwent choledochoscopic intrahepatic stone removal between 1998 and 2001, 54 patients with stones larger than 3 mm were enrolled in this study. In each case, a chemical compositional analysis was performed to determine calcium, cholesterol, total bilirubin, and inorganic phosphorus compositions. The three largest stones obtained from each patient were imaged by CT. CT attenuation numbers were measured in the center images of each stone by drawing free-hand region of interest (ROI). The measured CT attenuation numbers were correlated with their chemical composition. Also, CT attenuation numbers of stones were compared with that of the liver on non-contrast CT (50-70 HU). Results: Stone size ranged from 3.1 to 10.5 mm (mean ± S.D.: 6.0 ± 1.4). The CT attenuation numbers (HU) of stones ranged from 36.4 to 410.19 (mean ± S.D.: 94.6 ± 49.9). CT numbers of stones were below 70 HU in 11 patients (20.4%), and below 90 HU in 33 patients (59.3%). The chemical analysis data of the stones were as follows: calcium (0.5-6.5 wt.%; mean ± S.D., 2.6 ± 1.4), total bilirubin (0.45-24.4 wt.%; 13.1 ± 6.2), cholesterol (5.4-73.9 wt.%; 29.3 ± 17.4), phosphorus (0.1-1.2 wt.%; 0.6 ± 0.3), and non-soluble residue (17.6-85.4 wt.%; 57.0 ± 22.6). There was a weak but significant correlation between calcium composition and CT attenuation (r = 0.38, P 0.01; total bilirubin, r = 0.05, P > 0.01; phosphorus, r = 0.01, P > 0.01). Conclusion: On non-contrast CT, intrahepatic stones would not be hyperattenuating with respect to liver parenchyma in about one fifth of patients. The CT attenuation of stones correlates with calcium and does not correlate with any other chemical composition

  5. Hepatic Encephalopathy Secondary to Intrahepatic Portosystemic Venous Shunt: Balloon-Occluded Retrograde Transvenous Embolization with n-Butyl Cyanoacrylate and Microcoils

    International Nuclear Information System (INIS)

    Yamagami, Takuji; Nakamura, Toshiyuki; Iida, Shigeharu; Kato, Takeharu; Tanaka, Osamu; Matsushima, Shigenori; Ito, Hirotoshi; Okuyama, Chio; Ushijima, Yo; Shiga, Kensuke; Nishimura, Tsunehiko

    2002-01-01

    We report a 70-year-old woman with hepatic encephalopathy due to an intrahepatic portosystemic venous shunt that was successfully occluded by percutaneous transcatheter embolization with n-butyl cyanoacrylate and microcoils

  6. Modified rendezvous intrahepatic bile duct cannulation technique to pass a PTBD catheter in ERCP.

    Science.gov (United States)

    Lee, Tae Hoon; Park, Sang-Heum; Lee, Sae Hwan; Lee, Chang-Kyun; Lee, Suck-Ho; Chung, Il-Kwun; Kim, Hong Soo; Kim, Sun-Joo

    2010-11-14

    The rendezvous procedure combines an endoscopic technique with percutaneous transhepatic biliary drainage (PTBD). When a selective common bile duct cannulation fails, PTBD allows successful drainage and retrograde access for subsequent rendezvous techniques. Traditionally, rendezvous procedures such as the PTBD-assisted over-the-wire cannulation method, or the parallel cannulation technique, may be available when a bile duct cannot be selectively cannulated. When selective intrahepatic bile duct (IHD) cannulation fails, this modified rendezvous technique may be a feasible alternative. We report the case of a modified rendezvous technique, in which the guidewire was retrogradely passed into the IHD through the C2 catheter after end-to-end contact between the tips of the sphincterotome and the C2 catheter at the ampulla's orifice, in a 39-year-old man who had been diagnosed with gallbladder carcinoma with a metastatic right IHD obstruction. Clinically this procedure may be a feasible and timesaving technique.

  7. Deep convolutional neural network for the classification of hepatocellular carcinoma and intrahepatic cholangiocarcinoma

    Science.gov (United States)

    Midya, Abhishek; Chakraborty, Jayasree; Pak, Linda M.; Zheng, Jian; Jarnagin, William R.; Do, Richard K. G.; Simpson, Amber L.

    2018-02-01

    Liver cancer is the second leading cause of cancer-related death worldwide.1 Hepatocellular carcinoma (HCC) is the most common primary liver cancer accounting for approximately 80% of cases. Intrahepatic cholangiocarcinoma (ICC) is a rare liver cancer, arising in patients with the same risk factors as HCC, but treatment options and prognosis differ. The diagnosis of HCC is based primarily on imaging but distinguishing between HCC and ICC is challenging due to common radiographic features.2-4 The aim of the present study is to classify HCC and ICC in portal venous phase CT. 107 patients with resected ICC and 116 patients with resected HCC were included in our analysis. We developed a deep neural network by modifying a pre-trained Inception network by retraining the final layers. The proposed method achieved the best accuracy and area under the receiver operating characteristics curve of 69.70% and 0.72, respectively on the test data.

  8. Hepatic Encephalopathy due to Congenital Multiple Intrahepatic Portosystemic Venous Shunts Successfully Treated by Percutaneous Transhepatic Obliteration

    Directory of Open Access Journals (Sweden)

    Shinsuke Takenaga

    2016-11-01

    Full Text Available Hepatic encephalopathy due to intrahepatic portosystemic venous shunts (IPSVS in a non-cirrhotic condition is rare. Here we report a rare case of a patient with congenital multiple IPSVS successfully treated by percutaneous transhepatic obliteration. The patient was a 67-year-old woman who presented to our hospital with progressive episodes of consciousness disorder and vomiting. Laboratory tests revealed hyperammonemia (192.0 μg/dL, and computed tomography revealed multiple IPSVS in both lobes. There was no evidence of underlying liver disease or hepatic trauma. Transcatheter embolization for IPSVS was performed because conservative therapy was not sufficiently effective. After endovascular shunt closure, hepatic encephalopathy improved. The serum ammonia level normalized during the 5-year follow-up period. Thus, transcatheter embolization may be an effective therapy for patients with symptomatic and refractory IPSVS. Careful follow-up is necessary for portal hypertension-related complications after transcatheter embolization for IPSVS.

  9. Intrahepatic portal-hepatic venous shunt diagnosed by ultrasonography and computed tomography. Report of two cases

    Energy Technology Data Exchange (ETDEWEB)

    Shinagawa, Takashi; Iino, Yasuo; Ukaji, Haruyasu; Ishizuka, Masaharu

    1986-02-01

    Two cases of intrahepatic portal-hepatic venous shunt found by ultrasonography and computed tomography are reported. The first case came to the hospital because of hematuria. A large shunt between the portal vein and the hepatic vein was demonstrated by ultrasonography done for screening, and confirmed by percutaneous transhepatic portography (PTP). The second case was admitted because of cholelithiasis. Computed tomography with contrast enhancement revealed a dilated portal vein in the upper portion of the right lobe. It was subsequently shown by PTP to be a portal-hepatic venous shunt. Portal vein pressure and histological finding of the liver were normal in both cases. The etiology of the shunt was thought to be congenital in these cases for lack of liver disease, portal hypertension and history of trauma. Both cases had no history of hepatic encephalopathy and did not show any symptom attributable to the shunt.

  10. Mutations in the nuclear bile acid receptor FXR cause progressive familial intrahepatic cholestasis

    Science.gov (United States)

    Gomez-Ospina, Natalia; Potter, Carol J.; Xiao, Rui; Manickam, Kandamurugu; Kim, Mi-Sun; Kim, Kang Ho; Shneider, Benjamin L.; Picarsic, Jennifer L.; Jacobson, Theodora A.; Zhang, Jing; He, Weimin; Liu, Pengfei; Knisely, A. S.; Finegold, Milton J.; Muzny, Donna M.; Boerwinkle, Eric; Lupski, James R.; Plon, Sharon E.; Gibbs, Richard A.; Eng, Christine M.; Yang, Yaping; Washington, Gabriel C.; Porteus, Matthew H.; Berquist, William E.; Kambham, Neeraja; Singh, Ravinder J.; Xia, Fan; Enns, Gregory M.; Moore, David D.

    2016-01-01

    Neonatal cholestasis is a potentially life-threatening condition requiring prompt diagnosis. Mutations in several different genes can cause progressive familial intrahepatic cholestasis, but known genes cannot account for all familial cases. Here we report four individuals from two unrelated families with neonatal cholestasis and mutations in NR1H4, which encodes the farnesoid X receptor (FXR), a bile acid-activated nuclear hormone receptor that regulates bile acid metabolism. Clinical features of severe, persistent NR1H4-related cholestasis include neonatal onset with rapid progression to end-stage liver disease, vitamin K-independent coagulopathy, low-to-normal serum gamma-glutamyl transferase activity, elevated serum alpha-fetoprotein and undetectable liver bile salt export pump (ABCB11) expression. Our findings demonstrate a pivotal function for FXR in bile acid homeostasis and liver protection. PMID:26888176

  11. Hypervitaminosis A inducing intra-hepatic cholestasis--a rare case report.

    Science.gov (United States)

    Ramanathan, Vivek S; Hensley, Gary; French, Samuel; Eysselein, Victor; Chung, David; Reicher, Sonya; Pham, Binh

    2010-04-01

    The use of over-the-counter supplements is commonplace in today's health conscious society. We present an unusual case of intrahepatic cholestasis caused by vitamin A intoxication. The patient consumed one Herbalife shake with two multivitamin tablets of the same brand for 12 years. When calculated this equated to more than the recommended daily allowance for vitamin A consumption. Deranged liver function tests were consistent with a cholestatic process. Liver biopsy was obtained and revealed features pathognomonic of vitamin A toxicity, without the usual fibrosis. When the supplements were ceased, his jaundice and alkaline phosphatase completely normalized. This case highlights the importance of health care providers documenting non-prescribed dietary supplements and considering them in the etiology of cholestatic liver disease. Copyright 2009 Elsevier Inc. All rights reserved.

  12. Cholangiocarcinoma of intrahepatic bile ducts with disseminated metastases in an African lion (Panthera leo).

    Science.gov (United States)

    Lepri, Elvio; Sforna, Monica; Brachelente, Chiara; Chiara, Brachelente; Vitellozzi, Giovanni; Giovanni, Vitellozzi

    2013-06-01

    A cholangiocarcinoma is reported in an 18-yr-old, female African lion (Panthera leo). The primary tumor consisted of multifocal to coalescing, hepatic, white-yellow masses distributed throughout the liver lobes. Metastases were present in regional lymph nodes, peritoneal surface, and lungs. Histologically, the tumor was characterized by a tubular pattern with alcian- and periodic acid-Schiff-positive secretory material in cystic spaces. The neoplastic cells were positive to broad-spectrum cytokeratins. Histochemical and immunohistochemical stains were consistent with bile duct carcinoma. Biliary tumors arising from the gallbladder have been reported in lions. However, to the authors' knowledge, this is the first case of intrahepatic bile duct carcinoma reported in an African lion.

  13. Transjugular intrahepatic portosystemic shunt by direct transcaval approach: indications and anatomic foundation

    International Nuclear Information System (INIS)

    Chu Jianguo; Sun Xiaoli; Huang He; Xu Xiaoming; Pu Longsong; Lv Chunyan; Sun Peng; Yang Shuhui; Liu Shuying

    2004-01-01

    Objective: To investigate into the indications and related anatomic foundation of transjugular intrahepatic portosystemic shunt (TIPS) creation by direct transcaval approach in patients with portal hypertension cirrhosis suffering unusual anatomy between the hepatic veins and portal bifurcation; and to evaluate the security, feasibility and clinical significance. Methods: Direct transcaval approach TIPS were performed in 65 patients including active variceal bleeding (n=52), intractable ascites (n=12), and as a bridge to liver transplantation (n=1). Results: Technical and functional success were achieved in all patients. The success rate was 100% without related complications including the technique and primary patency rate is obvious higher than classical TIPS. Conclusion: In patients with unusual anatomy between the hepatic veins and portal bifurcation, and inaccessible or inadequate hepatic veins, transcaval TIPS creation is secure and feasible. The results suggest that the direct transcaval approach offering favorable primary patency because the shunt has a straight line in construction

  14. Clinical benefits of new immobilization system for hypofractionated radiotherapy of intrahepatic hepatocellular carcinoma by helical tomotherapy

    Energy Technology Data Exchange (ETDEWEB)

    Hu, Yong; Zhou, Yong-Kang; Chen, Yi-Xing; Shi, Shi-Ming; Zeng, Zhao-Chong, E-mail: zeng.zhaochong@zs-hospital.sh.cn

    2017-04-01

    Objective: A comprehensive clinical evaluation was conducted, assessing the Body Pro-Lok immobilization and positioning system to facilitate hypofractionated radiotherapy of intrahepatic hepatocellular carcinoma (HCC), using helical tomotherapy to improve treatment precision. Methods: Clinical applications of the Body Pro-Lok system were investigated (as above) in terms of interfractional and intrafractional setup errors and compressive abdominal breath control. To assess interfractional setup errors, a total of 42 patients who were given 5 to 20 fractions of helical tomotherapy for intrahepatic HCC were analyzed. Overall, 15 patients were immobilized using simple vacuum cushion (group A), and the Body Pro-Lok system was used in 27 patients (group B), performing megavoltage computed tomography (MVCT) scans 196 times and 435 times, respectively. Pretreatment MVCT scans were registered to the planning kilovoltage computed tomography (KVCT) for error determination, and group comparisons were made. To establish intrafractional setup errors, 17 patients with intrahepatic HCC were selected at random for immobilization by Body Pro-Lok system, undergoing MVCT scans after helical tomotherapy every week. A total of 46 MVCT re-scans were analyzed for this purpose. In researching breath control, 12 patients, randomly selected, were immobilized by Body Pro-Lok system and subjected to 2-phase 4-dimensional CT (4DCT) scans, with compressive abdominal control or in freely breathing states, respectively. Respiratory-induced liver motion was then compared. Results: Mean interfractional setup errors were as follows: (1) group A: X, 2.97 ± 2.47 mm; Y, 4.85 ± 4.04 mm; and Z, 3.77 ± 3.21 mm; pitch, 0.66 ± 0.62°; roll, 1.09 ± 1.06°; and yaw, 0.85 ± 0.82°; and (2) group B: X, 2.23 ± 1.79 mm; Y, 4.10 ± 3.36 mm; and Z, 1.67 ± 1.91 mm; pitch, 0.45 ± 0.38°; roll, 0.77 ± 0.63°; and yaw, 0.52 ± 0.49°. Between-group differences were statistically significant in 6 directions (p

  15. [Intrahepatic cholestasis associated with parenteral nutrition: an experimental study in rats].

    Science.gov (United States)

    Salas Martínez, J; Morán Penco, J M; Mahedero Ruiz, G; García Gamito, F; Limón Mora, M; Maciá Botejara, E; Vinagre Velasco, L M

    1989-01-01

    Intrahepatic cholestasis is a condition often observed in patients receiving parenteral nutrition, especially in new born babies who are underweight (taurina. This makes it impossible to achieve a correct conjugation of toxic biliary acids. The access of nutrients to the liver may have an effect on this. An experimental study on rats was performed, administering an oral diet at the expense of lipids (20% Intralipid, 60% of caloric needs) and glucose (40% of caloric needs) in one group, another group received amino acid supplements to this diet (16N) at a proteic rate of 2 gr/kg of weight and day orally, with an identical diet to the above, except that the proteic intake was intraperitoneal. Two control groups were established. We found a microvacuolization in hepatic fat with the help of an electronic microscope in the groups lacking proteins and those with oral or intraperitoneal supplements of amino acids, as well as an increase in plasmatic AST.

  16. Clinical benefits of new immobilization system for hypofractionated radiotherapy of intrahepatic hepatocellular carcinoma by helical tomotherapy

    International Nuclear Information System (INIS)

    Hu, Yong; Zhou, Yong-Kang; Chen, Yi-Xing; Shi, Shi-Ming; Zeng, Zhao-Chong

    2017-01-01

    Objective: A comprehensive clinical evaluation was conducted, assessing the Body Pro-Lok immobilization and positioning system to facilitate hypofractionated radiotherapy of intrahepatic hepatocellular carcinoma (HCC), using helical tomotherapy to improve treatment precision. Methods: Clinical applications of the Body Pro-Lok system were investigated (as above) in terms of interfractional and intrafractional setup errors and compressive abdominal breath control. To assess interfractional setup errors, a total of 42 patients who were given 5 to 20 fractions of helical tomotherapy for intrahepatic HCC were analyzed. Overall, 15 patients were immobilized using simple vacuum cushion (group A), and the Body Pro-Lok system was used in 27 patients (group B), performing megavoltage computed tomography (MVCT) scans 196 times and 435 times, respectively. Pretreatment MVCT scans were registered to the planning kilovoltage computed tomography (KVCT) for error determination, and group comparisons were made. To establish intrafractional setup errors, 17 patients with intrahepatic HCC were selected at random for immobilization by Body Pro-Lok system, undergoing MVCT scans after helical tomotherapy every week. A total of 46 MVCT re-scans were analyzed for this purpose. In researching breath control, 12 patients, randomly selected, were immobilized by Body Pro-Lok system and subjected to 2-phase 4-dimensional CT (4DCT) scans, with compressive abdominal control or in freely breathing states, respectively. Respiratory-induced liver motion was then compared. Results: Mean interfractional setup errors were as follows: (1) group A: X, 2.97 ± 2.47 mm; Y, 4.85 ± 4.04 mm; and Z, 3.77 ± 3.21 mm; pitch, 0.66 ± 0.62°; roll, 1.09 ± 1.06°; and yaw, 0.85 ± 0.82°; and (2) group B: X, 2.23 ± 1.79 mm; Y, 4.10 ± 3.36 mm; and Z, 1.67 ± 1.91 mm; pitch, 0.45 ± 0.38°; roll, 0.77 ± 0.63°; and yaw, 0.52 ± 0.49°. Between-group differences were statistically significant in 6 directions (p

  17. Yttrium-90 resin microspheres and their use in the treatment of intrahepatic cholangiocarcinoma.

    Science.gov (United States)

    Filippi, Luca; Schillaci, Orazio; Cianni, Roberto; Bagni, Oreste

    2018-04-01

    Intrahepatic cholangiocarcinoma (ICC) is a severe and rapidly progressive hepatic tumor. Surgery is often impracticable due to locally advanced presentation. On the other hand, chemotherapy has demonstrated only limited effectiveness. For these reasons, liver-directed therapies have been successfully applied for treating ICC. In particular, radioembolization with Yttrium-90 ( 90 Y)-labeled spheres has been reported to be a promising therapeutic approach for this neoplasia. Two commercial forms of 90 Y-labeled spheres are available: glass (TheraSphere ® ) and resin (SIR-Spheres ® ) microspheres. The aim of the present paper is to review the existing literature on the use of the resin microspheres for the treatment of unresectable and chemorefractory ICC, focusing on the methodology, clinical applications and side effects.

  18. Transjugular intrahepatic portosystemic shunt in a patient with cavernomatous portal vein occlusion

    International Nuclear Information System (INIS)

    Kawamata, Hiroshi; Kumazaki, Tatsuo; Kanazawa, Hidenori; Takahashi, Shuji; Tajima, Hiroyuki; Hayashi, Hiromitsu

    2000-01-01

    A 23-year-old woman with liver cirrhosis secondary to primary sclerosing cholangitis was referred to us for the treatment of recurrent bleeding from esophageal varices that had been refractory to endoscopic sclerotherapy. Her portal vein was occluded, associated with cavernous transformation. A transjugular intrahepatic portosystemic shunt (TIPS) was performed after a preprocedural three-dimensional computed tomographic angiography evaluation to determine feasibility. The portal vein system was recanalized and portal blood flow increased markedly after TIPS. Esophageal varices disappeared 3 weeks after TIPS. Re-bleeding and hepatic encephalopathy were absent for 3 years after the procedure. We conclude that with adequate preprocedural evaluation, TIPS can be performed safely even in patients with portal vein occlusion associated with cavernous transformation.

  19. Intrahepatic portal-hepatic venous shunt diagnosed by ultrasonography and computed tomography

    International Nuclear Information System (INIS)

    Shinagawa, Takashi; Iino, Yasuo; Ukaji, Haruyasu; Ishizuka, Masaharu

    1986-01-01

    Two cases of intrahepatic portal-hepatic venous shunt found by ultrasonography and computed tomography are reported. The first case came to the hospital because of hematuria. A large shunt between the portal vein and the hepatic vein was demonstrated by ultrasonography done for screening, and confirmed by percutaneous transhepatic portography (PTP). The second case was admitted because of cholelithiasis. Computed tomography with contrast enhancement revealed a dilated portal vein in the upper portion of the right lobe. It was subsequently shown by PTP to be a portal-hepatic venous shunt. Portal vein pressure and histological finding of the liver were normal in both cases. The etiology of the shunt was thought to be congenital in these cases for lack of liver disease, portal hypertension and history of trauma. Both cases had no history of hepatic encephalopathy and did not show any symptom attributable to the shunt. (author)

  20. Intrahepatic ovulation

    OpenAIRE

    Wozniak, Artur L.; Visscher, Kari L.; Bhaduri, Mousumi

    2015-01-01

    Ectopic ovaries are a rare finding in the literature, with fewer than 50 published cases to date. This phenomenon has been found in the omentum, bladder, mesentery, and uterus; attached to the colon; inside the left labia majora; and in the kidney. Various etiologies have been proposed, including postsurgical or postinflammatory transplantation, malignant origins, and abnormal embryologic development. We report the ultrasonographic, computed tomographic (CT), and magnetic resonance (MR) imagi...

  1. Effects of andrographolide on intrahepatic cholestasis induced by alpha-naphthylisothiocyanate in rats.

    Science.gov (United States)

    Khamphaya, Tanaporn; Chansela, Piyachat; Piyachaturawat, Pawinee; Suksamrarn, Apichart; Nathanson, Michael H; Weerachayaphorn, Jittima

    2016-10-15

    Cholestasis is a cardinal manifestation of liver diseases but effective therapeutic approaches are limited. Therefore, alternative therapy for treating and preventing cholestatic liver diseases is necessary. Andrographolide, a promising anticancer drug derived from the medicinal plant Andrographis paniculata, has diverse pharmacological properties and multi-spectrum therapeutic applications. However, it is unknown whether andrographolide has a hepatoprotective effect on intrahepatic cholestasis. The aims of this study were to investigate the protective effect and possible mechanisms of andrographolide in a rat model of acute intrahepatic cholestasis induced by alpha-naphthylisothiocyanate (ANIT). Andrographolide was administered intragastrically for four consecutive days, with a single intraperitoneal injection of ANIT on the second day. Liver injury was evaluated biochemically and histologically together with hepatic gene and protein expression analysis. Rats pretreated with andrographolide prior to ANIT injection demonstrated lower levels of serum alanine aminotransferase, aspartate aminotransferase, alkaline phosphatase, gamma-glutamyltransferase, as well as bilirubin and bile acids as compared to rats treated with ANIT alone. Andrographolide also decreased the incidence and extent of periductular fibrosis and bile duct proliferation. Analysis of protein expression in livers from andrographolide-treated cholestatic rats revealed markedly decreased expression of alpha-smooth muscle actin and nuclear factor kappa-B (NF-κB). In conclusion, andrographolide has a potent protective property against ANIT-induced cholestatic liver injury. The mechanisms that underlie this protective effect are mediated through down-regulation of NF-κB expression and inhibition of hepatic stellate cell activation. These findings suggest that andrographolide could be a promising therapeutic option in prevention and slowing down the progression of cholestatic liver diseases. Copyright

  2. Intrahepatic recurrence after percutaneous radiofrequency ablation of hepatocellular carcinoma: Analysis of the pattern and risk factors

    International Nuclear Information System (INIS)

    Kim, Young-sun; Rhim, Hyunchul; Cho, On Koo; Koh, Byung Hee; Kim, Yongsoo

    2006-01-01

    Purpose: To evaluate the pattern and risks for intrahepatic recurrence after percutaneous radiofrequency (RF) ablation for hepatocellular carcinoma (HCC). Materials and methods: We studied 62 patients with 72 HCCs (≤4 cm) who were treated with percutaneous RF ablation. The mean follow-up period was 19.1 months (6.0-49.1). We assessed the incidence and cumulative disease-free survival of local tumor progression (LTP) and intrahepatic distant recurrence (IDR). To analyze the risk factors, we examined the following, for the LTP: (1) tumor diameter, (2) contact with vessels, (3) degree of approximation to hepatic hilum, (4) contact with hepatic capsule, (5) presence of ablative safety margin, (6) degree of benign periablational enhancement and (7) serum alpha-fetoprotein; for the IDR: (1) severity of hepatic disease, (2) presence of HBsAg, (3) serum alpha-fetoprotein, (4) whether RF ablation was the initial treatment and (5) multiplicity of tumor for IDR. Results: The incidence of overall recurrence, LTP and IDR was 62.9%, 26.4% and 53.2%, respectively. The cumulative disease-free survival rates were 52%, 82% and 56% at 1 year, 26%, 63% and 30% at 2 years, respectively. Univariate analysis showed that the significant risk factors for LTP were: a tumor with a diameter >3 cm, contact of HCC with a vessel and an insufficient safety margin (p 3 cm and insufficient safety margin were independent factors. Only the increased serum alpha-fetoprotein was a significant risk factor for IDR (p 3 cm) with high serum alpha-fetoprotein should be treated more aggressively because of higher risk for recurrence

  3. Are congenital malformations more frequent in fetuses with intrahepatic persistent right umbilical vein? A comparative study.

    Science.gov (United States)

    Adiego-Calvo, Ignacio; Saviron-Cornudella, Ricardo; Martinez-Payo, Cristina; Rubio-Aranda, Encarna; Sancho-Sauco, Javier; Cisneros-Gimeno, Ana Isabel; Perez-Perez, Pilar; Lerma-Puertas, Diego; Whyte-Orozco, Jaime

    2016-12-01

    Persistent right umbilical vein (PRUV) is a vascular anomaly where the right umbilical vein remains as the only conduit that returns oxygenated blood to the fetus. It has classically been described as associated with numerous defects. We distinguish the intrahepatic variant (better prognosis) and the extrahepatic variant (associated with worse prognosis). The objective of this study was to compare rates of congenital malformations in fetuses with intrahepatic PRUV (I-PRUV) versus singleton pregnancies without risk factors. A multicenter, crossover design, comparative study was performed between 2003 and 2013 on fetuses diagnosed with I-PRUV (n=56), and singleton pregnancies without congenital malformation risk factors (n=4050). Fifty-six cases of I-PRUV were diagnosed (incidence 1:770). A statistically significant association between I-PRUV and the presence of congenital malformations (odds ratio 4.321; 95% confidence interval 2.15-8.69) was found. This positive association was only observed with genitourinary malformations (odds ratio 3.038; 95% confidence interval 1.08-8.56). Our rate of malformations associated with I-PRUV (17.9%) is similar to previously published rates. I-PRUV has shown a significant increase in the rate of associated malformations, although this association has only been found to be statistically significant in the genitourinary system. Noteworthy is the fact that this comparative study has not pointed to a significant increase in the congenital heart malformation rate. Diagnosis of isolated I-PRUV does not carry a worse prognosis. Copyright © 2016. Published by Elsevier B.V.

  4. Importance of intrahepatic mechanisms to gluconeogenesis from alanine during exercise and recovery

    International Nuclear Information System (INIS)

    Wasserman, D.H.; Williams, P.E.; Lacy, D.B.; Green, D.R.; Cherrington, A.D.

    1988-01-01

    These studies were performed to assess the importance of intrahepatic mechanisms to gluconeogenesis in the dog during 150 min of treadmill exercise and 90 min of recovery. Sampling catheters were implanted in an artery and portal and hepatic veins 16 days before experimentation. Infusions of [U- 14 C]alanine, [3- 3 H]glucose, and indocyanine green were used to assess gluconeogenesis. During exercise, a decline in arterial and portal vein plasma alanine and in hepatic blood flow led to a decrease in hepatic alanine delivery. During recovery, hepatic blood flow was restored to basal, causing an increase in hepatic alanine delivery beyond exercise rates but still below resting rates. Hepatic fractional alanine extraction increased from 0.26 +/- 0.02 at rest to 0.64 +/- 0.03 during exercise and remained elevated during recovery. Net hepatic alanine uptake was 2.5 +/- 0.2 mumol.kg-1.min-1 at rest and remained unchanged during exercise but was increased during recovery. The conversion rate of [ 14 C]alanine to glucose had increased by 248 +/- 38% by 150 min of exercise and had increased further during recovery. The efficiency with which alanine was channeled into glucose in the liver was accelerated to a rate of 338 +/- 55% above basal by 150 min of exercise but declined slightly during recovery. In conclusion, 1) gluconeogenesis from alanine is accelerated during exercise, due to an increase in the hepatic fractional extraction of the amino acid and through intrahepatic mechanisms that more efficiently channel it into glucose

  5. Extracorporeal shock wave lithotripsy of intrahepatic stones with piezoelectric lithotriptor: in vitro study

    International Nuclear Information System (INIS)

    Choi, Byung Ihn; Yoon, Chong Hyun; Park, Yong Hyun; Han, Joon Koo; Yoon, Yong Bum; Shin, Yong Moon; Kim, Jin Q; Kim, Chu Wan; Han, Man Chung

    1992-01-01

    This study was designed to investigate effectiveness of fragmentation during lithotripsy using 103 intrahepatic stones collected from 10 patients, who had previously undergone biliary surgery. The size of each stone was measured and sonography was performed for the evaluation of the sonographic type of the stones. In vitro lithotripsy was performed on individual stones using piezoelectric lithotriptor to evaluate the fragmentation rate and average number of shock waves for fragmentation. Chemical analysis of each stone was done to determine chemical composition including calcium, bilirubin, and cholesterol. The size of the stones was from 5 mm to 20 mm in diameter. Sonographic type I (echo of whole stone with posterior acoustic shadow) was 68, and type II (are-like strong surface echo of stone with clear posterior acoustic shadow) was 35 in number. The majority (78%) of stones in group I (5-9 mm in diameter) showed sonographic type I characteristics, and 62% of stones in group 3 (larger than 15 mm in diameter) showed sonographic type II characteristics. There was a positive correlation between the size and sonographic type of stones. Fragmentation rates of stones were 100% in group I, 71.9% in group 2 (10-15 mm in diameter), 43.8% in group 3, respectively. Fragmentation rates of stones with sonographic type I and II were 91.2%, 65.7%, respectively. The average number of shock waves for partial and complete fragmentation was 2753 ± 4937 and 6219 ± 10133, respectively. There was a positive correlation between the number of shock waves for fragmentation and diameter of stones (r = 0.618, ρ < 0.05). There was no correlation between the number of shock waves for fragmentation and chemical composition of stones. In conclusion, the most important variable determining the degree of fragmentation of intrahepatic stones using ESWL is not their chemical composition but their size and sonographic characteristics

  6. Transjugular Intrahepatic Portosystemic Shunt Dysfunction: Concordance of Clinical Findings, Doppler Ultrasound Examination, and Shunt Venography.

    Science.gov (United States)

    Owen, Joshua M; Gaba, Ron Charles

    2016-01-01

    The objective of this study was to evaluate the concordance between clinical symptoms, Doppler ultrasound (US), and shunt venography for the detection of stent-graft transjugular intrahepatic portosystemic shunt (TIPS) dysfunction. Forty-one patients (M:F 30:11, median age 55 years) who underwent contemporaneous clinical exam, Doppler US, and TIPS venography between 2003 and 2014 were retrospectively studied. Clinical symptoms (recurrent ascites or variceal bleeding) were dichotomously classified as present/absent, and US and TIPS venograms were categorized in a binary fashion as normal/abnormal. US abnormalities included high/low (>190 or 50 cm/s), absent flow, and return of antegrade intra-hepatic portal flow. Venographic abnormalities included shunt stenosis/occlusion and/or pressure gradient elevation. Clinical and imaging concordance rates were calculated. Fifty-two corresponding US examinations and venograms were assessed. The median time between studies was 3 days. Forty of 52 (77%) patients were symptomatic, 33/52 (64%) US examinations were abnormal, and 20/52 (38%) TIPS venograms were abnormal. Concordance between clinical symptoms and TIPS venography was 48% (25/52), while the agreement between US and shunt venography was 65% (34/52). Clinical symptoms and the US concurred in 60% (31/52) of the patients. The sensitivity of clinical symptoms and US for the detection of venographically abnormal shunts was 80% (16/20) and 85% (17/20), respectively. Both clinical symptoms and the US had low specificity (25%, 8/32 and 50%, 16/32) for venographically abnormal shunts. Clinical findings and the US had low concordance rates with TIPS venography, with acceptable sensitivity but poor specificity. These findings suggest the need for improved noninvasive imaging methods for stent-graft TIPS surveillance.

  7. Minilaparotomy-Assisted Transmesenteric-Transjugular Intrahepatic Portosystemic Shunt: Comparison with Conventional Transjugular Approach

    Energy Technology Data Exchange (ETDEWEB)

    Jalaeian, Hamed, E-mail: hjalaeia@umn.edu; Talaie, Reza; D’Souza, Donna; Taleb, Shayandokht [University of Minnesota, Division of Interventional Radiology, Department of Radiology (United States); Noorbaloochi, Siamak [University of Minnesota, School of Medicine (United States); Flanagan, Siobhan; Hunter, David; Golzarian, Jafar [University of Minnesota, Division of Interventional Radiology, Department of Radiology (United States)

    2016-10-15

    PurposeThis study was performed to compare the intrahepatic shunt function outcome and procedural complications of minilaparotomy-assisted transmesenteric (MAT)-transjugular intrahepatic portosystemic shunt (TIPS) placement with the conventional transjugular method.MethodsThis is a retrospective review of all patients who had a MAT or conventional TIPS procedure over a 6-year period at our institute. The primary patency rate, fluoroscopy time, technical success, major procedure-related complications, and mortality data were compared between two treatment groups.ResultsWe included 49 patients with MAT-TIPS, and 63 with conventional TIPS, with an average follow-up of 21.43 months. The primary patency rates at 6 and 12 months were 82.9 and 66.7 % in the conventional TIPS group, and 81.0 and 76.5 % in the MAT-TIPS group (p = 1.000, and 0.529), respectively. There was no significant difference in technical success rate, post-procedure portosystemic pressure gradient, fluoroscopy time, and peri-procedural mortality rate between treatment groups. Major procedural-related complications were seen more frequently among MAT-TIPS patients (p = 0.012). In the MAT-TIPS group, 5 (10.2 %) patients developed post-procedure minilaparotomy, wound-related complications, and 5 (10.2 %) developed bacterial peritonitis; whereas, none of patients with conventional TIPS had either of these complications (p = 0.014).ConclusionWhile both MAT-TIPS and conventional TIPS had similar shunt primary patency rate and technical success rate, the MAT approach was associated with a significantly higher rate of minilaparotomy-related wound complications or infectious complications. These complications maybe prevented by a change in post-procedure monitoring and therapy.

  8. Transjugular Intrahepatic Portosystemic Shunt Dysfunction: Concordance of Clinical Findings, Doppler Ultrasound Examination, and Shunt Venography

    Directory of Open Access Journals (Sweden)

    Joshua M Owen

    2016-01-01

    Full Text Available Objectives: The objective of this study was to evaluate the concordance between clinical symptoms, Doppler ultrasound (US, and shunt venography for the detection of stent-graft transjugular intrahepatic portosystemic shunt (TIPS dysfunction. Materials and Methods: Forty-one patients (M:F 30:11, median age 55 years who underwent contemporaneous clinical exam, Doppler US, and TIPS venography between 2003 and 2014 were retrospectively studied. Clinical symptoms (recurrent ascites or variceal bleeding were dichotomously classified as present/absent, and US and TIPS venograms were categorized in a binary fashion as normal/abnormal. US abnormalities included high/low (>190 or 50 cm/s, absent flow, and return of antegrade intra-hepatic portal flow. Venographic abnormalities included shunt stenosis/occlusion and/or pressure gradient elevation. Clinical and imaging concordance rates were calculated. Results: Fifty-two corresponding US examinations and venograms were assessed. The median time between studies was 3 days. Forty of 52 (77% patients were symptomatic, 33/52 (64% US examinations were abnormal, and 20/52 (38% TIPS venograms were abnormal. Concordance between clinical symptoms and TIPS venography was 48% (25/52, while the agreement between US and shunt venography was 65% (34/52. Clinical symptoms and the US concurred in 60% (31/52 of the patients. The sensitivity of clinical symptoms and US for the detection of venographically abnormal shunts was 80% (16/20 and 85% (17/20, respectively. Both clinical symptoms and the US had low specificity (25%, 8/32 and 50%, 16/32 for venographically abnormal shunts. Conclusion: Clinical findings and the US had low concordance rates with TIPS venography, with acceptable sensitivity but poor specificity. These findings suggest the need for improved noninvasive imaging methods for stent-graft TIPS surveillance.

  9. Extracorporeal shock wave lithotripsy of intrahepatic stones with piezoelectric lithotriptor: in vitro study

    Energy Technology Data Exchange (ETDEWEB)

    Choi, Byung Ihn; Yoon, Chong Hyun; Park, Yong Hyun; Han, Joon Koo; Yoon, Yong Bum; Shin, Yong Moon; Kim, Jin Q; Kim, Chu Wan; Han, Man Chung [Seoul National University, College of Medicine, Seoul (Korea, Republic of)

    1992-05-15

    This study was designed to investigate effectiveness of fragmentation during lithotripsy using 103 intrahepatic stones collected from 10 patients, who had previously undergone biliary surgery. The size of each stone was measured and sonography was performed for the evaluation of the sonographic type of the stones. In vitro lithotripsy was performed on individual stones using piezoelectric lithotriptor to evaluate the fragmentation rate and average number of shock waves for fragmentation. Chemical analysis of each stone was done to determine chemical composition including calcium, bilirubin, and cholesterol. The size of the stones was from 5 mm to 20 mm in diameter. Sonographic type I (echo of whole stone with posterior acoustic shadow) was 68, and type II (are-like strong surface echo of stone with clear posterior acoustic shadow) was 35 in number. The majority (78%) of stones in group I (5-9 mm in diameter) showed sonographic type I characteristics, and 62% of stones in group 3 (larger than 15 mm in diameter) showed sonographic type II characteristics. There was a positive correlation between the size and sonographic type of stones. Fragmentation rates of stones were 100% in group I, 71.9% in group 2 (10-15 mm in diameter), 43.8% in group 3, respectively. Fragmentation rates of stones with sonographic type I and II were 91.2%, 65.7%, respectively. The average number of shock waves for partial and complete fragmentation was 2753 {+-} 4937 and 6219 {+-} 10133, respectively. There was a positive correlation between the number of shock waves for fragmentation and diameter of stones (r = 0.618, {rho} < 0.05). There was no correlation between the number of shock waves for fragmentation and chemical composition of stones. In conclusion, the most important variable determining the degree of fragmentation of intrahepatic stones using ESWL is not their chemical composition but their size and sonographic characteristics.

  10. Radioembolization of hepatic tumors. Flow redistribution after the occlusion of intrahepatic arteries

    International Nuclear Information System (INIS)

    Lauenstein, T.C.; Heusner, T.A.; Antoch, G.; Hamami, M.; Bockisch, A.; Ertle, J.; Schlaak, J.F.; Gerken, G.

    2011-01-01

    Radioembolization using 90yttrium is an emerging therapy option for unresectable liver malignancies. In order to reduce the number of yttrium injections, endovascular occlusion of a segmental hepatic artery has been proposed. The aim of this study was to assess whether sufficient vascular redistribution of the occluded liver segments through intrahepatic collaterals can be observed. 27 patients with hepatocellular carcinoma (n = 16) or hepatic metastases (n = 11) were studied. Hepatic angiography was performed on average 16 days prior to radioembolization. The segment II/III artery (n = 9) or the segment IV artery (n = 18) was occluded using coils. Technectium-99m-labeled macroaggregated albumin (99mTc-MAA) was injected into the right and the remaining part of the left hepatic artery in order to identify any hepatic volume not included in the perfused area. Patients underwent a SPECT/CT on average 1 h after the 99mTc-MAA injection. Two radiologists evaluated the SPECT/CT scans regarding the presence of non-perfused hepatic segments. Furthermore, hepatic perfusion was assessed by digital subtraction angiography (DSA) on the day of radioembolization. In 16 / 27 patients (59 %) a perfusion of the occluded liver segment was visible on the SPECT/CT scan. In 8 / 11 patients without flow redistribution at the time of the SPECT/CT, perfusion of the occluded segment through hepatic collaterals was observed during angiography prior to radioembolization. Hence, flow redistribution was eventually found in 24 / 27 patients (89 %). Flow redistribution after the occlusion of intrahepatic arteries prior to radioembolization can be successfully induced in the majority of patients with anatomical variants of the hepatic arteries. (orig.)

  11. Use of Sengstaken-Blakemore intrahepatic balloon: an alternative for liver-penetrating injuries.

    Science.gov (United States)

    Fraga, Gustavo Pereira; Zago, Thiago Messias; Pereira, Bruno Monteiro; Calderan, Thiago Rodrigues Araujo; Silveira, Henrique Jose Virgili

    2012-09-01

    Severe lesions in the liver are associated with a high mortality rate. Alternative surgical techniques such as the use of an intrahepatic balloon may be effective and reduce mortality in severe hepatic lesions. This study aimed to demonstrate the experience of a university hospital in the use of the Sengstaken-Blakemore balloon in patients with transfixing penetrating hepatic injury as an alternative way to treat these challenging injuries. A retrospective study based on the trauma registry of a university hospital was performed. All patients admitted with hepatic penetrating injuries and treated with the Sengstaken-Blakemore balloon within the period 1990-2010 were reviewed. Forty-six patients with transfixing hepatic injuries were treated with the Sengstaken-Blakemore balloon in the study period. The most frequent cause of injury was gunshot wound (87 % of the patients). The mean trauma scores on admission were Revised Trauma Score (RTS) = 7.12 ± 1.46, Injury Severity Score (ISS) = 22.4 ± 9.7, and Abdominal Trauma Index (ATI) = 19.5 ± 11. According to the severity of the hepatic trauma, 71.8 % of patients had grade III, 23.9 % grade IV, and 4.3 % grade V injuries. Associated abdominal injuries were found in 89.1 % of the patients. The most frequent liver-related complications were hepatic abscess postoperative bleeding (8.6 %), biliary fistula (8.6 %), (4.3 %), and biliary peritonitis (2.1 %). Surgical reintervention was necessary in 14 patients (31.1 %). From those 14, only 3 had the balloon removed. The overall morbidity and mortality rates were 56.5 % and 23.9 % (11 patients), respectively. The knowledge of alternative surgical techniques is essential in improving survival in patients with severe penetrating hepatic injuries. The use of intrahepatic balloon is a viable surgical strategy.

  12. Residual intrahepatic stones after percutaneous biliary extraction : longterm follow up of complications

    International Nuclear Information System (INIS)

    Yoo, Seung Min; Shim, Hyung Jin; Lee, Hwa Yeon; Lim, Sang Jun; Park, Hyo Jin; Kim, Yang Soo; Choi, Young Hee; Kwak, Byung Kuck; Park, Ji Young

    1997-01-01

    To evaluate and compare the radiologic and clinical follow-up of complications between a group in whom stone removal after percutaneous biliary extraction had been complete, and a group in whom this had been incomplete. Twenty-two patients in whom stone removal had been incomplete, and 20 from whom stones had been completely removed were evaluated with particular attention to complications such as cholangitis, liver abscess, biliary sepsis, and pain. Cholangitis was diagnosed on the basis of typical clinical symptoms such as pain, high fever, jaundice and leukocytosis. Pain without other cholangitic symptoms was excluded. Liver abscess was diagnosed by percutaneous aspiration of pus, and biliary sepsis by bacterial growth on blood culture, or laboratory findings such as increased fibrinogen products, decreased fibrinogen, and increased prothrombin time with cholangitic symptoms. 'Complete removal' means no residual stones on follow-up sonogram and cholangiogram performed within three to seven days after pecutaneous biliary extraction. Mean follow-up period was 26.5 months in the incomplete removal group and 34.2 months in the complete removal group. In twelve of 22 patients (54.5%) in the incomplete removal group, complications occurred, as follows:cholangitis, ten cases (45.5%);liver absces, one (4.5%);biliary sepsis, one (4.5%);and pain, seven(31.8%). In contrast, only two of twenty patients (10%) in the complete removal group suffered complications, all of which involved the recurrence of stones in the common duct, and cholangitis. Complete removal of intrahepatic stones significantly helps to reduce the incidence of possible complications. Even in the case of an impacted stone, aggressive interventional procedures, aimed at complete removal, should be considered. If nonsurgical procedures fail, early partial hepatectomy should be considered, particulary for the stones localized in the left intrahepatic duct

  13. The “Mystique” of Acute Leukemia: MPAL-BAL-AUL-ALAL-aBLL-HAL-MLL: Initial presentation of MPAL as extramedullary neurological compromise; A case report and review of literature

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    Soubhagya Ranjan Tripathy

    2016-12-01

    Conclusion: For routine neurosurgical practice, these entities are extremely rare; and hence a working knowledge is very essential for appropriate & timely management notwithstanding the neurosurgical desire to rule out the compressive lesions first. Neurological status deterioration may be halted with timely institution of appropriate chemotherapy. In the extensive literature review in pubmed, this may be only the 1st case of MPAL with extramedullary neurological manifestation, at the first clinical presentation.

  14. Studies on the effects of ionizing radiation and chemotherapeutic agents on hematopoiesis according to the stem-cell kinetics

    International Nuclear Information System (INIS)

    Hirashima, Kunitake

    1975-01-01

    The fundamental problem of the effects of ionizing radiation and antineoplastic drugs on hematopoiesis can be explained by the kinetic study on the hematopoietic stem-cell population. Quantitative comparison of a single x-irradiation and a single administration of several antineoplastic drugs on the stem-cell population was performed by the splenic colony-forming method. The repopulation pattern of stem-cells in mice after a single 150 rad irradiation was compared with that after the administration of corresponding dose of cyclophosphamide. It was demonstrated that the additional administration of cyclophosphamide immediately after the x-irradiation significantly accelerated repopulation of the stem-cell compartment. The mechanism of repopulation of the stem-cell compartment after partial irradiation was also studied according to the immigration theory of stem-cells. An in vitro colony-forming technique for the human bone marrow cells was introduced and compared with other assay methods for stem-cells. From the hematological observations of accidentally irradiated patients, it was determined that the thromboelastogram values were regarded as one of the most useful indicators for detecting the earliest recovery sign of the hematopoietic stem-cells. (Evans, J.)

  15. Hhex Regulates Hematopoietic Stem Cell Self-Renewal and Stress Hematopoiesis via Repression of Cdkn2a.

    Science.gov (United States)

    Jackson, Jacob T; Shields, Benjamin J; Shi, Wei; Di Rago, Ladina; Metcalf, Donald; Nicola, Nicos A; McCormack, Matthew P

    2017-08-01

    The hematopoietically expressed homeobox transcription factor (Hhex) is important for the maturation of definitive hematopoietic progenitors and B-cells during development. We have recently shown that in adult hematopoiesis, Hhex is dispensable for maintenance of hematopoietic stem cells (HSCs) and myeloid lineages but essential for the commitment of common lymphoid progenitors (CLPs) to lymphoid lineages. Here, we show that during serial bone marrow transplantation, Hhex-deleted HSCs are progressively lost, revealing an intrinsic defect in HSC self-renewal. Moreover, Hhex-deleted mice show markedly impaired hematopoietic recovery following myeloablation, due to a failure of progenitor expansion. In vitro, Hhex-null blast colonies were incapable of replating, implying a specific requirement for Hhex in immature progenitors. Transcriptome analysis of Hhex-null Lin - Sca + Kit + cells showed that Hhex deletion leads to derepression of polycomb repressive complex 2 (PRC2) and PRC1 target genes, including the Cdkn2a locus encoding the tumor suppressors p16 Ink 4 a and p19 Arf . Indeed, loss of Cdkn2a restored the capacity of Hhex-null blast colonies to generate myeloid progenitors in vitro, as well as hematopoietic reconstitution following myeloablation in vivo. Thus, HSCs require Hhex to promote PRC2-mediated Cdkn2a repression to enable continued self-renewal and response to hematopoietic stress. Stem Cells 2017;35:1948-1957. © 2017 AlphaMed Press.

  16. Donor hematopoiesis in mice following total lymphoid irradiation requires host T-regulatory cells for durable engraftment

    Science.gov (United States)

    Müller, Antonia M. S.; Poyser, Jessica; Küpper, Natascha J.; Burnett, Cassandra; Ko, Rose M.; Kohrt, Holbrook E.K.; Florek, Mareike; Zhang, Pei; Negrin, Robert S.

    2014-01-01

    Total lymphoid irradiation (TLI) with antithymocyte globulin (ATG) is a unique regimen that prepares recipients for allogeneic hematopoietic cell transplantation by targeting lymph nodes, while sparing large areas of the bone marrow. TLI is reported to increase the frequency of CD4+CD25+FoxP3+ T-regulatory cells (Treg) relative to conventional T cells. In this study, barriers to hematopoietic stem cell (HSC) engraftment following this nonmyeloablative conditioning were evaluated. TLI/ATG resulted in profound lymphoablation but endogenous host HSC remained. Initial donor HSC engraftment occurred only in radiation exposed marrow sites, but gradually distributed to bone marrow outside the radiation field. Sustained donor engraftment required host lymphoid cells insofar as lymphocyte deficient Rag2γc−/− recipients had unstable engraftment compared with wild-type. TLI/ATG treated wild-type recipients had increased proportions of Treg that were associated with increased HSC frequency and proliferation. In contrast, Rag2γc−/− recipients who lacked Treg did not. Adoptive transfer of Treg into Rag2γc−/− recipients resulted in increased cell cycling of endogenous HSC. Thus, we hypothesize that Treg influence donor engraftment post-TLI/ATG by increasing HSC cell cycling, thereby promoting the exit of host HSC from the marrow niche. Our study highlights the unique dynamics of donor hematopoiesis following TLI/ATG, and the effect of Treg on HSC activity. PMID:24591203

  17. Multiplex CRISPR/Cas9-Based Genome Editing in Human Hematopoietic Stem Cells Models Clonal Hematopoiesis and Myeloid Neoplasia.

    Science.gov (United States)

    Tothova, Zuzana; Krill-Burger, John M; Popova, Katerina D; Landers, Catherine C; Sievers, Quinlan L; Yudovich, David; Belizaire, Roger; Aster, Jon C; Morgan, Elizabeth A; Tsherniak, Aviad; Ebert, Benjamin L

    2017-10-05

    Hematologic malignancies are driven by combinations of genetic lesions that have been difficult to model in human cells. We used CRISPR/Cas9 genome engineering of primary adult and umbilical cord blood CD34 + human hematopoietic stem and progenitor cells (HSPCs), the cells of origin for myeloid pre-malignant and malignant diseases, followed by transplantation into immunodeficient mice to generate genetic models of clonal hematopoiesis and neoplasia. Human hematopoietic cells bearing mutations in combinations of genes, including cohesin complex genes, observed in myeloid malignancies generated immunophenotypically defined neoplastic clones capable of long-term, multi-lineage reconstitution and serial transplantation. Employing these models to investigate therapeutic efficacy, we found that TET2 and cohesin-mutated hematopoietic cells were sensitive to azacitidine treatment. These findings demonstrate the potential for generating genetically defined models of human myeloid diseases, and they are suitable for examining the biological consequences of somatic mutations and the testing of therapeutic agents. Copyright © 2017 Elsevier Inc. All rights reserved.

  18. Double-Balloon-Assisted n-Butyl-2-Cyanoacrylate Embolization of Intrahepatic Arterioportal Shunt Prior to Chemoembolization of Hepatocellular Carcinoma

    Energy Technology Data Exchange (ETDEWEB)

    Takao, Hidemasa, E-mail: takaoh-tky@umin.ac.jp; Shibata, Eisuke; Ohtomo, Kuni [University of Tokyo, Department of Radiology, Graduate School of Medicine (Japan)

    2016-10-15

    A case of multiple hepatocellular carcinomas with a severe intrahepatic arterioportal shunt that was successfully embolized with n-butyl-2-cyanoacrylate with coaxial double-balloon occlusion prior to transcatheter arterial chemoembolization is presented. A proximal balloon positioned at the proper hepatic artery was used for flow control, and a coaxial microballoon, positioned in the closest of three arterial feeding branches to the arterioportal shunt, was used to control the delivery of n-butyl-2-cyanoacrylate. This coaxial double-balloon technique can prevent proximal embolization and distal migration of n-butyl-2-cyanoacrylate and enable precise control of the distribution of n-butyl-2-cyanoacrylate. It could also be applicable to n-butyl-2-cyanoacrylate embolization for other than intrahepatic arterioportal shunt.

  19. Allogeneic Hematopoietic Cell Transplantation in Multiple Myeloma: Focus on Longitudinal Assessment of Donor Chimerism, Extramedullary Disease, and High-Risk Cytogenetic Features.

    Science.gov (United States)

    Rasche, Leo; Röllig, Christoph; Stuhler, Gernot; Danhof, Sophia; Mielke, Stephan; Grigoleit, Goetz Ulrich; Dissen, Lea; Schemmel, Lea; Middeke, Jan Moritz; Rücker, Viktoria; Schreder, Martin; Schetelig, Johannes; Bornhäuser, Martin; Einsele, Hermann; Thiede, Christian; Knop, Stefan

    2016-11-01

    Although generally not applied as first-line treatment of multiple myeloma, allogeneic hematopoietic cell transplantation (allo-SCT) can still be chosen as ultimate escalation approach in high-risk patients, preferentially within the framework of clinical trials. In this study, we investigated whether decreasing donor chimerism (DC) is predictive for relapse. In addition, we comprehensively determined the impact of several other disease- and treatment-related factors on outcome. One hundred fifty-five multiple myeloma patients whose DC status was followed serially by the short tandem repeat-based techniques at a single lab were included in this retrospective study. Outcome variables were studied in univariate and multivariable analyses. Available were 2.324 DC samples (median, 12 per patient). Loss of full DC was associated with shorter progression-free survival (PFS) (HR, 1.7; 95% CI, 1.1 to 2.6) but did not impact overall survival. Two-thirds of patients with International Myeloma Working Group-defined relapses still displayed a full DC in peripheral blood or bone marrow. Extramedullary manifestations were observed in 33% of patients, accounting for the discrepancy between DC analysis and the actual disease status. In multivariable analysis, the 2 most relevant variables for an unfavorable PFS were progressive disease before allo-SCT (HR, 3.0; 95% CI, 1.5 to 5.9) and allo-SCT at least the second relapse (HR, 2.8; 95% CI, 1.5 to 4.9), whereas for overall survival progressive disease or partial response before allo-SCT had the strongest negative effects (HR, 4.2; 95% CI, 1.9 to 9, and HR, 2.0; 95% CI, 1.0 to 3.8, respectively). Adverse cytogenetics such as del17p, t(4,14) or amp(1q21) were not associated with shorter survival after allo-SCT. Extensive DC sampling beyond robust engraftment does not appear to provide additional information helpful for disease management in most patients and is challenged by a significant incidence of extramedullary disease. In our

  20. Reversal of Transjugular Intrahepatic Portosystemic Shunt (TIPS)-Induced Hepatic Encephalopathy Using a Strictured Self-Expanding Covered Stent

    International Nuclear Information System (INIS)

    Cox, Mitchell W.; Soltes, George D.; Lin, Peter H.; Bush, Ruth L.; Lumsden, Alan B.

    2003-01-01

    Hepatic encephalopathy is a known complication following percutaneous transjugular intrahepatic portosystemic shunt (TIPS) placement. We describe herein a simple and effective strategy of TIPS revision by creating an intraluminal stricture within a self-expanding covered stent, which is deployed in the portosystemic shunt to reduce the TIPS blood flow. This technique was successful in reversing a TIPS-induced hepatic encephalopathy in our patient

  1. Intrahepatic and hilar mass-forming cholangiocarcinoma: Qualitative and quantitative evaluation with diffusion-weighted MR imaging.

    Science.gov (United States)

    Fattach, Hassan El; Dohan, Anthony; Guerrache, Youcef; Dautry, Raphael; Boudiaf, Mourad; Hoeffel, Christine; Soyer, Philippe

    2015-08-01

    To qualitatively and quantitatively analyze the presentation of intrahepatic and hilar mass-forming cholangiocarcinoma with diffusion-weighted magnetic resonance imaging (DW-MRI). Twenty-eight patients with histopathologically proven mass-forming cholangiocarcinoma (hilar, n=17; intrahepatic, n=11) underwent hepatic DW-MRI at 1.5-T using free-breathing acquisition and three b-values (0,400,800s/mm(2)). Cholangiocarcinomas were evaluated qualitatively using visual analysis of DW-MR images and quantitatively with conventional ADC and normalized ADC measurements using liver and spleen as reference organs. All cholangiocarcinomas (28/28; 100%) were visible on DW-MR images. DW-MRI yielded best conspicuity of cholangiocarcinomas than the other MRI sequences (Philar cholangiocarcinomas. The use of normalized ADC using the liver as reference organ resulted in the most restricted distribution of ADC values of cholangiocarcinomas (variation coefficient=16.6%). There is a trend towards a common appearance of intrahepatic and hilar mass-forming cholangiocarcinomas on DW-MRI but variations may be observed. Familiarity with these variations may improve the diagnosis of mass-forming cholangiocarcinoma. Copyright © 2015 Elsevier Ireland Ltd. All rights reserved.

  2. Lifestyle risk factors for intrahepatic stone: findings from a case-control study in an endemic area, Taiwan.

    Science.gov (United States)

    Momiyama, Masato; Wakai, Kenji; Oda, Koji; Kamiya, Junichi; Ohno, Yoshiyuki; Hamaguchi, Michinari; Nakanuma, Yasuni; Hsieh, Ling-Ling; Yeh, Ta-Sen; Chen, Tse-Ching; Jan, Yi-Yi; Chen, Miin-Fu; Nimura, Yuji

    2008-07-01

    To examine associations between lifestyle risk factors and intrahepatic stone (IHS), we conducted a case-control study in Taiwan, which has the highest incidence of IHS in the world. Study subjects were 151 patients newly diagnosed with IHS at Chang Gung Memorial Hospital between January 1999 and December 2001. Two control subjects per case were selected randomly from patients who underwent minor surgery at the same hospital and from family members or neighbors of the hospital staff. Controls were matched to each case by age and gender. Information on lifestyle factors was collected using a self-administered questionnaire. Strength of associations was assessed using odds ratios derived from conditional logistic models. Female patients were significantly shorter than female controls. Compared to subjects with two or fewer children, odds ratios for those with six or more children were 20.4 in men (95% confidence interval, 1.89-221) and 2.82 (0.97-8.22) in women. Increasing level of education lowered the risk of intrahepatic stone (trend P = 0.004 for men and ground-surface water for a long period had a somewhat increased risk (trend P = 0.05). Lower socioeconomic status and poor hygiene may be involved in the development of intrahepatic stones.

  3. A small solitary non-parasitic hepatic cyst causing an intra-hepatic bile duct stricture: a case report

    Directory of Open Access Journals (Sweden)

    Hong Taeho

    2010-08-01

    Full Text Available Abstract Introduction We report an unusual presentation of a small hepatic cyst causing cholangitis. Case presentation A 70-year-old Asian man was hospitalized for aggravated chronic pain in the right upper portion of his abdomen. Fever developed after admission. Laboratory tests revealed elevated hepatobiliary enzymes, inflammatory markers and carbohydrate antigen 19-9 without hyperbilirubinemia. Ultrasound and computed tomography demonstrated dilatation of the left intra-hepatic bile ducts. Endoscopic retrograde cholangiopancreatography showed that the right intra-hepatic bile ducts were normally filled with contrast medium, but the left intra-hepatic bile ducts were not seen in the confluence. A left hepatectomy was performed because a hidden malignancy could not be excluded. The surgical findings showed no tumor around the bile duct but rather a 2 cm cyst in segment four of Couinaud's category of the liver around the hilum. The pathology report was a solitary non-parasitic hepatic cyst compressing the bile duct. Conclusion A very small solitary hepatic cyst might cause hepatic duct stricture if it is located near the hepatic hilum, and should be considered in the differential diagnosis of a hepatic duct stricture.

  4. Platynosomum fastosum-induced chronic intrahepatic cholangitis and Spirometra spp. infections in feral cats from Grand Cayman.

    Science.gov (United States)

    Headley, S A; Gillen, M A; Sanches, A W D; Satti, M Z

    2012-06-01

    The occurrence of platynosomiasis and intestinal sparganosis is described in feral cats from Grand Cayman, Cayman Islands. Spirometra spp. was observed within the intestine of 18.18% (10/55) of cats; 1.18% (1/55) of cats demonstrated gross and histological manifestation of parasitism by Platynosomum fastosum, but 14.5% (8/55) of cats had the characteristic pathological manifestations of P. fastosum-induced intrahepatic cholangitis without the concomitant presence of the intraductal trematode. Combined parasitism (Spirometra spp. and P. fastosum) was observed in 9.09% (5/55) of feral cats. Significant pathological findings were only associated with the hepatic fluke, P. fastosum, and were grossly characterized by moderate hepatomegaly with enlarged and dilated bile ducts. Examples of cestodes with morphological features characteristic of Spirometra spp. were observed within the small intestine without any associated pathological lesion. The histopathological evaluation of liver fragments revealed chronic intrahepatic cholangitis with and without the associated intraductal trematode, and was characterized by marked periductal fibrosis, adenomatous proliferation of bile duct epithelium, dilation of intrahepatic bile ducts and portal accumulations of inflammatory cells. The occurrence of the cestode in feral cats coupled with factors that are unique to Grand Cayman makes this island the ideal location for sporadic cases of human sparganosis.

  5. [Clinical value of CT-guided high frequency-induced thermotherapy as a treatment for intrahepatic cholangiocarcinoma].

    Science.gov (United States)

    Fan, Wei-Jun; Zhang, Liang; Gu, Yang-Kui; Wang, Li-Gang; Ouyang, Yu-Shu

    2008-07-01

    To evaluate the therapeutic effect of CT-guided high frequency-induced thermotherapy (HiTT) for intrahepatic cholangiocarcinoma. Seventeen patients of intrahepatic cholangiocarcinoma with 21 lesions underwent comprehensive treatment with HiTT as the principle approach. As to the patients with obstructive jaundice, percutaneous trans-hepatic cholangial drainage (PTCD) or bile duct endoprosthesis placement was performed first to improve the liver function, then HiTT was performed; and patients without obstructive jaundice underwent CT-guided HiTT directly, 1-2 weeks later, chemotherapy was given for 4 - 6 courses. CT scan 1 week after HiTT showed a short-term achievement rate of 100% (17/17), and the single puncture in situ ablation rate was 76.1% (16/21). The average life span in the near future was 13.5 months. The adverse effects included topo-bleeding, pain after procedure, liver function damage, defervescence, etc. All the patients recovered after symptomatic treatment. The clinical value of CT-guided HiTT for intrahepatic cholangiocarcinoma is obvious.

  6. Tibialis Anterior Tendon: A Reliable Anatomical Landmark Indicating the Ankle Centre. Potential Utility in Extra-Medullary Alignment During Total Knee Replacement

    Directory of Open Access Journals (Sweden)

    Avadhoot P. Kantak

    2017-07-01

    Full Text Available Background Extramedullary alignment is a well established surgical technique during total knee replacement. There are different methods to achieve accuracy but variability is quite extensive. To attain uniformity in the surgical technique we have been using the tibialis tendon to align our resection guide. This may prove to be a useful aid for surgeons during knee replacement surgery. Objectives The purpose of our study was to establish if tibialis anterior tendon represents the centre of ankle joint and if it could be used as an anatomical reference for alignment during knee replacement. Methods We designed a retrospective radiological cohort study. We studied sixty MRI scans of normal ankles. The centre of ankle joint was marked as a bisection point of the intermalleolar line at the level of superior surface of the talus. A line was drawn connecting the centre of Achilles tendon to the ankle centre and this was extended anteriorly. This line was found to have a constant relation to the ankle centre and it would simulate the positioning of the standard alignment device used. Results The tibialis anterior tendon lies less than 3mm medial to the ankle centre in the frontal plane. Conclusions We conclude that the tibialis anterior tendon can be used during knee replacement surgery as an accurate alignment guide.

  7. Spinal perimedullary vein enlargement sign: an added value for the differentiation between intradural-extramedullary and intramedullary tumors on magnetic resonance imaging

    International Nuclear Information System (INIS)

    Gong, Tao; Wang, Guangbin; Gao, Fei; Chen, Xin; Liu, Yubo; Yang, Li; Chen, Weibo

    2016-01-01

    The purpose of this study was to determine the added value of the perimedullary spinal vein enlargement sign on magnetic resonance imaging (MRI) in distinguishing intradural-extramedullary tumors (IDEMTs) from intramedullary spinal tumors (IMTs). Two hundred and eight consecutive spinal intradural tumors with histopathologic confirmation (21 IMTs, 187 IDEMTs) were enrolled. Two readers blinded to the final pathological diagnosis and clinical data independently assessed the venous enlargement sign to determine the agreement between them and jointly distinguished IDEMTs from IMTs according to the common MRI findings. Sensitivity, specificity, and accuracy for the diagnosis of IDEMTs were calculated for the common MRI findings, vein enlargement sign, and a combination of both. Intraobserver agreement and interobserver agreement for both readers was excellent. The sensitivity, specificity, and accuracy of common MRI findings for differentiating IDEMTs from IMTs were 83.4, 95.2, and 89.3 %, respectively. Thirty-one IDEMTs were mistakenly diagnosed as IMTs, in which seven were cases with vein enlargement signs. By applying the vein enlargement sign to the common MRI findings, the specificity remained at 95.2 %, while the sensitivity improved to 89.3 % and the accuracy increased to 92.3 %. The spinal perimedullary vein enlargement sign is useful in assessing intradural tumors and to differentiate IDEMTs from IMTs. (orig.)

  8. Spinal perimedullary vein enlargement sign: an added value for the differentiation between intradural-extramedullary and intramedullary tumors on magnetic resonance imaging

    Energy Technology Data Exchange (ETDEWEB)

    Gong, Tao; Wang, Guangbin; Gao, Fei; Chen, Xin [Shandong University, Department of Shandong Medical Imaging Research Institute, Jinan (China); Liu, Yubo [Provincial Hospital Affiliated to Shandong University, Department of Radiology, Jinan (China); Yang, Li [Zhongshan Hospital, Department of Radiology, Shanghai (China); Chen, Weibo [Philips Healthcare, Shanghai (China)

    2016-11-15

    The purpose of this study was to determine the added value of the perimedullary spinal vein enlargement sign on magnetic resonance imaging (MRI) in distinguishing intradural-extramedullary tumors (IDEMTs) from intramedullary spinal tumors (IMTs). Two hundred and eight consecutive spinal intradural tumors with histopathologic confirmation (21 IMTs, 187 IDEMTs) were enrolled. Two readers blinded to the final pathological diagnosis and clinical data independently assessed the venous enlargement sign to determine the agreement between them and jointly distinguished IDEMTs from IMTs according to the common MRI findings. Sensitivity, specificity, and accuracy for the diagnosis of IDEMTs were calculated for the common MRI findings, vein enlargement sign, and a combination of both. Intraobserver agreement and interobserver agreement for both readers was excellent. The sensitivity, specificity, and accuracy of common MRI findings for differentiating IDEMTs from IMTs were 83.4, 95.2, and 89.3 %, respectively. Thirty-one IDEMTs were mistakenly diagnosed as IMTs, in which seven were cases with vein enlargement signs. By applying the vein enlargement sign to the common MRI findings, the specificity remained at 95.2 %, while the sensitivity improved to 89.3 % and the accuracy increased to 92.3 %. The spinal perimedullary vein enlargement sign is useful in assessing intradural tumors and to differentiate IDEMTs from IMTs. (orig.)

  9. Efficacy comparison of precise and traditional liver resection in treatment of intrahepatic bile duct stones

    Directory of Open Access Journals (Sweden)

    ZHANG Shengjun

    2015-10-01

    Full Text Available ObjectiveTo compare the efficacy of precise and traditional liver resection in the treatment of intrahepatic bile duct stones. MethodsOne hundred and twenty-seven patients with intrahepatic bile duct stones who were treated with surgery in our hospital from December 2008 to December 2014 were selected and divided into precise liver resection group (n=72 and traditional liver resection group (n=55 based on the type of surgery. The operation time, intraoperative blood loss, amount of postoperative drainage, postoperative time to recovery, postoperative complications (incision infection, biliary fistula, lung infection, and pleural effusion, hospitalization cost, postoperative residual calculi, and postoperative calculus recurrence were compared between the two groups. Between-group comparison of continuous data was made by t test, and between-group comparison of categorical data was made by χ2 test. Survival data were analyzed using survival function. ResultsThere were significant differences in operation time, intraoperative blood loss, amount of postoperative drainage, postoperative time to recovery, and hospitalization cost between the precise liver resection group and the traditional liver resection group (t=3.720, 58.681, 19.169, 5.990, and 6.944; all P<0.05. There were no significant differences in postoperative complications including incision infection, biliary fistula, lung infection, and pleural effusion between the two groups (all P>0.05. There were also no significant differences in the incidence rates of postoperative residual calculi and calculus recurrence between the two groups (all P>0.05. The survival analysis of postoperative calculus recurrence time showed that there was no significant difference in calculus recurrence time between the two groups (P>0.05. ConclusionCompared with traditional liver resection, precise liver resection has the advantages of shorter operation time, less intraoperative bleeding, less

  10. Beta1 integrin is not essential for hematopoiesis but is necessary for the T cell-dependent IgM antibody response

    DEFF Research Database (Denmark)

    Brakebusch, Cord; Fillatreau, Simon; Potocnik, Alexandre J

    2002-01-01

    Several experimental evidences suggested that beta1 integrin-mediated adhesion of hematopoietic stem cells (HSC) is important for their function in the bone marrow (BM). Using induced deletion of the beta1 integrin gene restricted to the hematopoietic system, we show that beta1 integrin...... is not essential for HSC retention in the BM, hematopoiesis, and trafficking of lymphocytes. However, immunization with a T cell-dependent antigen resulted in virtually no IgM production and an increased secretion of IgG in mutant mice, while the response to a T cell-independent type 2 antigen showed decreases...

  11. The Transjugular Intrahepatic Portosystemic Shunt in the Treatment of Portal Hypertension: Current Status

    Directory of Open Access Journals (Sweden)

    Gilles Pomier-Layrargues

    2012-01-01

    Full Text Available The transjugular intrahepatic portosystemic shunt (TIPS represents a major advance in the treatment of complications of portal hypertension. Technical improvements and increased experience over the past 24 years led to improved clinical results and a better definition of the indications for TIPS. Randomized clinical trials indicate that the TIPS procedure is not a first-line therapy for variceal bleeding, but can be used when medical treatment fails, both in the acute situation or to prevent variceal rebleeding. The role of TIPS to treat refractory ascites is probably more justified to improve the quality of life rather than to improve survival, except for patients with preserved liver function. It can be helpful for hepatic hydrothorax and can reverse hepatorenal syndrome in selected cases. It is a good treatment for Budd Chiari syndrome uncontrollable by medical treatment. Careful selection of patients is mandatory before TIPS, and clinical followup is essential to detect and treat complications that may result from TIPS stenosis (which can be prevented by using covered stents and chronic encephalopathy (which may in severe cases justify reduction or occlusion of the shunt. A multidisciplinary approach, including the resources for liver transplantation, is always required to treat these patients.

  12. Intrahepatic cholangiocarcinoma: impact of genetic hemochromatosis on outcome and overall survival after surgical resection.

    Science.gov (United States)

    Sulpice, Laurent; Rayar, Michel; Boucher, Eveline; Pele, Fabienne; Pracht, Marc; Meunier, Bernard; Boudjema, Karim

    2013-03-01

    The influence of genetic hemochromatosis (GH) on outcomes following surgical resections for intrahepatic cholangiocarcinoma (ICC) has not been evaluated. All patients with ICC who underwent a surgical resection between January 1997 and August 2011 were analyzed retrospectively. Risk factors were assessed by univariate and multivariate analyses. Eighty-seven patients were analyzed; 16 of these patients (18.4%) had GH. Among the 71 non-GH patients, 52 (73.2%) and 19 (26.8%) had normal or cirrhotic parenchyma, respectively. There was no significant difference in survival between the GH and non-GH patients. A univariate analysis showed that major hepatectomy (P = 0.012), intraoperative blood transfusion (P = 0.007), tumor size >5 cm (P = 0.006), several nodules (P < 0.001), and microvascular invasion (P = 0.04) were significantly associated with poor survival. A multivariate analysis showed that intraoperative blood infusion (HR 0.37; CI 95% [0.19; 0.71]) and more than one nodule (HR 2.5; CI 95% [1.06; 5.8]) were associated with a lower survival rate. Although the incidence of GH was high in our series, the presence of GH did not affect the outcomes after a liver hepatectomy for ICC. GH does not appear to increase recurrences or worsen the overall and disease-free survival. Copyright © 2013 Elsevier Inc. All rights reserved.

  13. Transjugular intrahepatic portosystemic shunt for severe jaundice in patients with acute Budd-Chiari syndrome.

    Science.gov (United States)

    He, Fu-Liang; Wang, Lei; Zhao, Hong-Wei; Fan, Zhen-Hua; Zhao, Meng-Fei; Dai, Shan; Yue, Zhen-Dong; Liu, Fu-Quan

    2015-02-28

    To evaluate the feasibility of transjugular intrahepatic portosystemic shunt (TIPS) for severe jaundice secondary to acute Budd-Chiari syndrome (BCS). From February 2009 to March 2013, 37 patients with severe jaundice secondary to acute BCS were treated. Sixteen patients without hepatic venule, hepatic veins (HV) obstruction underwent percutaneous angioplasty of the inferior vena cava (IVC) and/or HVs. Twenty-one patients with HV occlusion underwent TIPS. Serum bilirubin, liver function, demographic data and operative data of the two groups of patients were analyzed. Twenty-one patients underwent TIPS and the technical success rate was 100%, with no technical complications. Sixteen patients underwent recanalization of the IVC and/or HVs and the technical success rate was 100%. The mean procedure time for TIPS was 84.0±12.11 min and angioplasty was 44.11±5.12 min (Pjaundice in either group. Severe jaundice is not a contraindication for TIPS in patients with acute BCS and TIPS is appropriate for severe jaundice due to BCS.

  14. Pathomorphological study of Thorotrast-related intrahepatic cholangiocarcinoma with a comparison to non-Thorotrast cases

    International Nuclear Information System (INIS)

    Kojiro, Masamichi; Sugihara, Shigetaka; Ito, Yuji; Nakashima, Toshiro; Ikezaki, Hidefumi; Mori, Takesaburo; Kido, Choichiro.

    1986-01-01

    Thirty-five autopsy cases of Thorotrast (TH)-related intrahepatic cholangiocarcinoma were morphologically studied with a comparison to 45 non-TH cases. Latent periods ranged from 25 to 48 years, with a mean of 34.1 ± 6.6 years. As to tumor location, the peripheral-middle type, in which the main tumor was located in the peirphery to middle portion of the liver, was the most common (89.2 %) in the TH-related cases, and the hilar type, in which the main tumor was located in the hepatic hilum, was the most common (78.8 %) in the non-TH cases. However, there was no close relationship between the distribution of TH depositsoand tumor location by soft-X ray examination of the liver slices. Grossly, the massive type with an infiltrative growth was the most common both in the TH- and non-TH cases. Histologically, there were no remarkable differences between the two groups, and tubular adenocarcinoma with varying degrees of fibrous stroma was the most common. In noncancerous areas, proliferation of the bile ducts with slight to moderate atypism and ductular proliferation around Glisson's capsule were found in 30 %, and 10 %, respectively, of the TH-related cases. However, such changes were also found in the non-TH cases at almost the same incidence. (author)

  15. Transjugular intrahepatic portosystemic shunt for the treatment of Budd-Chiari syndrome

    International Nuclear Information System (INIS)

    Han Guohong; He Chuangye; Yin Zhanxin; Meng Xiangjie; Wang Jianhong; Zhang Hongbo; Zhou Xinming; Wu Kaichun; Ding Jie; Fan Daiming

    2008-01-01

    Objective: To evaluate the efficacy of transjugular intrahepatic portosystemic shunt (TIPS) for Budd-Chiari syndrome (BCS). Methods: 14 patients with clinical findings of progressive liver function abnormality and severe complication of portal hypertension (upper gastrointestinal bleeding and refractory ascites)were diagnosed as BCS. Based on imaging manifestations, these patients were classified as follows: obstruction of inferior vena cava (1 case); obstruction of hepatic veins (5 cases); combined obstruction (obstruction of hepatic veins and inferior vena cava)(8 cases). During the procedure, different puncture points were selected for fulfilling the real condition; including 7 cases from hepatic vein to portal vein; 4 cases from inferior vena cava to hepatic vein, 4 cases from the right hepatic vein to portal vein. After the operation, the follow-up evaluation of blood flow in the shunt was performed. Results: All of these patients were successfully performed with TIPS, with average pressure of portal vein decreasing from (4.9 ± 1.4) KPa to(3.2 ± 1.5) KPa. After 5 to 64 mouths follow-up, the recurrent upper gastrointestinal bleeding occurred in two patients due to stent stenosis and were well controlled through balloon angioplasty. Conclusions: TIPS is an effective treatment for BCS with progressive liver dysfunction or severe portal hypertension with upper gastrointestinal bleeding and refractory ascites. In addition, it also contributes to the treatment of the recurrent or severe portal hypertension after the inferior vena cava or hepatic vein angioplasty. (authors)

  16. Mid-term effect of direct intrahepatic portosystemic shunt for the treatment of portal hypertension

    International Nuclear Information System (INIS)

    Luo Jianjun; Yan Zhiping; Wang Jianhua; Liu Qingxin; Qu Xudong

    2009-01-01

    Objective: To retrospectively analyze the mid-term clinical results of direct intrahepatic portosystemic shunt (DIPS) in treating patients with portal hypertension. Methods: DIPS were created in 23 patients with portal hypertension. Both preoperative and postoperative portal systemic pressure gradient (PPG), liver function and clinical symptoms were recorded and compared. Shunt patency was checked by color Doppler ultrasonography and the data were statistically analyzed by Kaplan-Meier method. Results DIPS creation was successfully accomplished in all 23 patients. No serious complications occurred after DIPS except for hemorrhagic ascites (n = 1) and mild hepatic encephalopathy (n = 3). Mean PPG significantly decreased from preoperative (32.6 ± 5.3) mmHg with a range of (23 - 43) mmHg to postoperative (10.1 ± 2.7) mmHg with a range of (5-14) mmHg (P < 0.001). After the procedure, the albumin level also markedly decreased while the bilirubin level distinctly increased. Obvious improvement of the clinical symptoms was observed. The cumulated primary patency rate of the shunt one and two years after treatment was 77.4% and 50.2% respectively. Conclusion: The mid-term clinical results indicate that DIPS is an effective and safe procedure for treating patients with portal hypertension. (authors)

  17. MiR-145 functions as a tumor suppressor targeting NUAK1 in human intrahepatic cholangiocarcinoma

    Energy Technology Data Exchange (ETDEWEB)

    Xiong, Xinkui; Sun, Daoyi; Chai, Hao; Shan, Wengang [Liver Transplantation Center, First Affiliated Hospital of Nanjing Medical University, Nanjing, Jiangsu Province (China); Key Laboratory of Living Donor Liver Transplantation, Ministry of Public Health, Nanjing, Jiangsu Province (China); Yu, Yue [Key Laboratory of Living Donor Liver Transplantation, Ministry of Public Health, Nanjing, Jiangsu Province (China); Pu, Liyong [Liver Transplantation Center, First Affiliated Hospital of Nanjing Medical University, Nanjing, Jiangsu Province (China); Key Laboratory of Living Donor Liver Transplantation, Ministry of Public Health, Nanjing, Jiangsu Province (China); Cheng, Feng, E-mail: docchengfeng@njmu.edu.cn [Liver Transplantation Center, First Affiliated Hospital of Nanjing Medical University, Nanjing, Jiangsu Province (China); Key Laboratory of Living Donor Liver Transplantation, Ministry of Public Health, Nanjing, Jiangsu Province (China)

    2015-09-18

    The dysregulation of micro (mi)RNAs is associated with cancer development. The miRNA miR-145 is downregulated in intrahepatic cholangiocarcinoma (ICC); however, its precise role in tumor progression has not yet been elucidated. Novel (nua) kinase family (NUAK)1 functions as an oncogene in various cancers and is a putative target of miR-145 regulation. In this study, we investigated the regulation of NUAK1 by miR-145 in ICC. We found that miR-145 level was significantly decreased in ICC tissue and cell lines, which corresponded with an increase in NUAK1 expression. NUAK1 was found to be a direct target of miR-145 regulation. The overexpression of miR-145 in ICC cell lines inhibited proliferation, growth, and invasion by suppressing NUAK1 expression, which was associated with a decrease in Akt signaling and matrix metalloproteinase protein expression. Similar results were observed by inhibiting NUAK1 expression. These results demonstrate that miR-145 can prevent ICC progression by targeting NUAK1 and its downstream effectors, and can therefore be useful for clinical diagnosis and targeted therapy of ICC. - Highlights: • MiR-145 suppresses ICC proliferation and invasion abilities. • We demonstrated that miR-145 directly targets NUAK1 in ICC. • MiR-145 expression in ICC was associated with Akt signaling and MMPs expression.

  18. Transjugular Intrahepatic Portosystemic Shunt Placement During Pregnancy: A Case Series of Five Patients

    International Nuclear Information System (INIS)

    Ingraham, Christopher R.; Padia, Siddharth A.; Johnson, Guy E.; Easterling, Thomas R.; Liou, Iris W.; Kanal, Kalpana M.; Valji, Karim

    2015-01-01

    Background and AimsComplications of portal hypertension, such as variceal hemorrhage and ascites, are associated with significant increases in both mortality and complications during pregnancy. Transjugular intrahepatic portosystemic shunt (TIPS) is a well-established procedure for treating portal hypertension, but the safety of TIPS during pregnancy is largely unknown. In this series, we review five patients who underwent TIPS placement while pregnant and describe their clinical outcomes.MethodsFive pregnant patients with cirrhosis and portal hypertension underwent elective TIPS for complications of portal hypertension (four for secondary prevention of variceal bleeding and one for refractory ascites). Outcomes measured were recurrent bleeding episodes or need for further paracenteses during pregnancy, estimated radiation dose to the fetus and gestational age at delivery. All patients were followed after delivery to evaluate technical and clinical success of the procedure.ResultsAll five patients survived pregnancy and went on to deliver successfully. When TIPS was performed for secondary prevention of variceal bleeding (n = 4), no patients demonstrated variceal bleeding after TIPS placement. When TIPS was performed for refractory ascites (n = 1), no further paracenteses were required. All patients delivered successfully, albeit prematurely. Average radiation dose estimated to the fetus was 16.3 mGy.ConclusionsThis series suggests that TIPS can be performed in selective pregnant patients with portal hypertension, with little added risk to the mother or fetus

  19. Evaluation of fluency stent-grafts in transjugular intrahepatic portosystemic shunts

    International Nuclear Information System (INIS)

    Zhao Jianbo; Li Yanhao; Chen Yong; He Xiaofeng; Zeng Qingle; Mei Quelin; Lu Wei

    2009-01-01

    Objective: To evaluate the efficacy of Fluency stent-graft (Bard Corp) in transjugular intrahepatic portosystemic shunt (TIPS). Methods: The clinical data of 21 consecutive patients treated by TIPS using Fluency stent-grafts were retrospectively reviewed. All of them were recurrent variceal bleeding secondary to portal vein hypertension, 1 was bleeding secondary to primary hepatic carcinoma with port vein thrombus, and 1 was Budd-Chiari syndrome. They were followed-up after (10.1±4.6) months (2.0 to 24.0 months). Stent-grafts patancy, portal vein pressure and liver function were recorded and compared. Results: Twenty-five stent-grafts were successfully implanted in 21 patients, 23 stent grafts were 8 mm 2 were 10 mm in diameter. The covered length of the stents varied from 6 to 8 cm. The bleeding was stopped and the portal vein pressure decreased significantly from (25.4±3.5) mm Hg to (15.4±2.8) mm Hg (t= 12.495, P 0.05). Conclusion: The Fluency stent-grafts could increase the patency of the TIPS, but its efficacy on the long-term effect and hepatic encephalopathy need further investigation. (authors)

  20. Radiologic follow-up of the transjugular intrahepatic stent-shunt (TIPSS)

    International Nuclear Information System (INIS)

    Hansmann, H.J.; Noeldge, G.; Leutloff, U.; Radeleff, B.; Richter, G.M.

    2001-01-01

    The transjugular intrahepatic stent-shunt (TIPSS) is a well accepted minimal invasive therapy for complications of portal hypertension: recurrent variceal bleeding, refractory ascites and liver failure due to the Budd-Chiari syndrome. The high frequency of shunt stenoses and occlusions makes regular follow up examinations essential. Despite modern non invasive imaging methods direct portography still is the gold standard for shunt surveillance in TIPSS. Ultrasound is helpful to detect shunt dysfunction, but nevertheless its failure rate is considerable despite the use of contrast enhancers such as Levovist because of anatomic and physical limitations, particularly when TIPSS-tracts deep in the liver are present. Reintervention rates approach 90-100% after 24 months, with 100% in child's A patients with comparatively good liver function. However, a strict shunt surveillance program with early portography and reintervention when necessary guarantees high clinical success rates associated with very low rebleeding rates below 10%. Overall the secondary success rate is 80%. Secondary failures are mainly caused by lack of patient compliance during follow-up. In a subgroup of patients no shunt maturation is observed, requiring multiple shunt revisions. In cases of recurrent shunt occlusions an association with bile leaks is presumed. In selected cases patients with chronically recurrent shunt stenosis or occlusions may benefit from placement of TIPSS stent grafts. (orig.) [de

  1. Transjugular Intrahepatic Portosystemic Shunt Placement in Patients with Cirrhosis and Concomitant Portal Vein Thrombosis

    International Nuclear Information System (INIS)

    Ha, Thuong G. Van; Hodge, Justin; Funaki, Brian; Lorenz, Jonathan; Rosenblum, Jordan; Straus, Christopher; Leef, Jeff

    2006-01-01

    Purpose. To determine the safety and efficacy of transjugular intrahepatic portosystemic shunt (TIPS) creation in patients with liver cirrhosis complicated by thrombosed portal vein. Methods. This study reviewed 15 cases of TIPS creation in 15 cirrhotic patients with portal vein thrombosis at our institution over an 8-year period. There were 2 women and 13 men with a mean age of 53 years. Indications were refractory ascites, variceal hemorrhage, and refractory pleural effusion. Clinical follow-up was performed in all patients. Results. The technical success rate was 75% (3/4) in patients with chronic portal vein thrombosis associated with cavernomatous transformation and 91% (10/11) in patients with acute thrombosis or partial thrombosis, giving an overall success rate of 87%. Complications included postprocedural encephalopathy and localized hematoma at the access site. In patients with successful shunt placement, the total follow-up time was 223 months. The 30-day mortality rate was 13%. Two patients underwent liver transplantation at 35 days and 7 months, respectively, after TIPS insertion. One patient had an occluded shunt at 4 months with an unsuccessful revision. The remaining patients had functioning shunts at follow-up. Conclusion. TIPS creation in thrombosed portal vein is possible and might be a treatment option in certain patients

  2. Transjugular Intrahepatic Portosystemic Shunt: Histologic and Immunohistochemical Study of Autopsy Cases

    International Nuclear Information System (INIS)

    Terayama, Noboru; Matsui, Osamu; Kadoya, Masumi; Yoshikawa, Jun; Gabata, Toshifumi; Miyayama, Shiro; Takashima, Tsutomu; Kobayashi, Kenichi; Nakanishi, Isao; Nakanuma, Yasuni

    1997-01-01

    Purpose: To assess the histologic findings associated with stenosed and occluded transjugular intrahepatic portosystemic shunt (TIPS) tracts. Methods: Four TIPS tracts within three autopsy livers were histologically studied for vascular components by routine staining and immunohistochemical staining. TIPS had been performed for bleeding from esophageal varices in patients with cirrhosis of the liver. Results: Two TIPS, examined on days 4 and 53, showed occlusion by fibrin thrombus. In the former, no endothelial cells were detected, but coagulative necrosis of hepatocytes was found in the surrounding liver. In the latter, bile pigments were seen on the luminal surface. In the two other TIPS without tract occlusion, examined on days 49 and 293, a layer of endothelial cells, proliferation of smooth muscle cells, and deposition of an extracellular matrix such as collagen were confirmed. In the tract examined on day 293, there was protrusion of hepatocytes into the lumen through the stent wires. Conclusion: Short- and midterm TIPS occlusions were caused by thrombus forming after necrosis of hepatocytes and bile leakage, respectively. Long-term TIPS stenosis was associated with a combination of pseudointimal hyperplasia and ingrowth of hepatocytes

  3. In vitro proliferation and differentiation of hepatic oval cells and their potential capacity for intrahepatic transplantation

    Energy Technology Data Exchange (ETDEWEB)

    Li, Z.; Chen, J. [Liaocheng People' s Hospital, Department of Hepatobiliary Surgery, Liaocheng, Shandong, China, Department of Hepatobiliary Surgery, Liaocheng People’s Hospital, Liaocheng, Shandong (China); Li, L.; Ran, J.H.; Liu, J. [The First People' s Hospital of Kunming, Kunming, Yunnan, China, The First People’s Hospital of Kunming, Kunming, Yunnan (China); Gao, T.X.; Guo, B.Y. [Dongchangfu Hospital of Women and Child Health Care, Liaocheng, Shandong (China); Li, X.H.; Liu, Z.H.; Liu, G.J.; Gao, Y.C.; Zhang, X.L. [Liaocheng People' s Hospital, Department of Hepatobiliary Surgery, Liaocheng, Shandong, China, Department of Hepatobiliary Surgery, Liaocheng People’s Hospital, Liaocheng, Shandong (China)

    2013-07-30

    Hepatic oval cells (HOCs) are recognized as facultative liver progenitor cells that play a role in liver regeneration after acute liver injury. Here, we investigated the in vitro proliferation and differentiation characteristics of HOCs in order to explore their potential capacity for intrahepatic transplantation. Clusters or scattered HOCs were detected in the portal area and interlobular bile duct in the liver of rats subjected to the modified 2-acetylaminofluorene and partial hepatectomy method. Isolated HOCs were positive for c-kit and CD90 staining (99.8% and 88.8%, respectively), and negative for CD34 staining (3.6%) as shown by immunostaining and flow cytometric analysis. In addition, HOCs could be differentiated into hepatocytes and bile duct epithelial cells after leukemia inhibitory factor deprivation. A two-cuff technique was used for orthotopic liver transplantation, and HOCs were subsequently transplanted into recipients. Biochemical indicators of liver function were assessed 4 weeks after transplantation. HOC transplantation significantly prolonged the median survival time and improved the liver function of rats receiving HOCs compared to controls (P=0.003, Student t-test). Administration of HOCs to rats also receiving liver transplantation significantly reduced acute allograft rejection compared to control liver transplant rats 3 weeks following transplantation (rejection activity index score: control=6.3±0.9; HOC=3.5±1.5; P=0.005). These results indicate that HOCs may be useful in therapeutic liver regeneration after orthotopic liver transplantation.

  4. Risk factors for stent graft thrombosis after transjugular intrahepatic portosystemic shunt creation.

    Science.gov (United States)

    Jahangiri, Younes; Kerrigan, Timothy; Li, Lei; Prosser, Dominik; Brar, Anantnoor; Righetti, Johnathan; Schenning, Ryan C; Kaufman, John A; Farsad, Khashayar

    2017-12-01

    To identify risk factors of stent graft thrombosis after transjugular intrahepatic portosystemic shunt (TIPS) creation. Patients who underwent TIPS creation between June 2003 and January 2016 and with follow-up assessing stent graft patency were included (n=174). Baseline comorbidities, liver function, procedural details and follow-up liver function tests were analyzed in association with hazards of thrombosis on follow-up. Competing risk cox regression models were used considering liver transplant after TIPS creation as the competing risk variable. One-, 2- and 5-year primary patency rates were 94.1%, 91.7% and 78.2%, respectively. Patient age [sub-hazard ratio (sHR): 1.13; P=0.001], body mass index (BMI) value for trend=0.017). Older age, lower BMI and higher post-TIPS portosystemic gradients were associated with higher hazards of shunt thrombosis after TIPS creation using stent grafts. Higher rates of shunt thrombosis were seen in patients for whom TIPS creation was clinically unsuccessful. The association between TIPS thrombosis and higher post-TIPS portosystemic gradients may indicate impaired flow through the shunt, a finding which may be technical or anatomic in nature and should be assessed before procedure completion.

  5. Arsenicosis, possibly from contaminated groundwater, associated with noncirrhotic intrahepatic portal hypertension.

    Science.gov (United States)

    Goel, Ashish; Christudoss, Pamela; George, Renu; Ramakrishna, Banumathi; Amirtharaj, G Jayakumar; Keshava, Shyamkumar N; Ramachandran, Anup; Balasubramanian, K A; Mackie, Ian; Fleming, Jude J; Elias, Elwyn; Eapen, Chundamannil E

    2016-05-01

    Idiopathic noncirrhotic intrahepatic portal hypertension (NCIPH), a chronic microangiopathy of the liver caused by arsenicosis from use of contaminated groundwater, was reported from Asia. This study aimed to see, if in the twenty-first century, arsenicosis was present in NCIPH patients at our hospital and, if present, to look for groundwater contamination by arsenic in their residential locality. Twenty-seven liver biopsy proven NCIPH patients, 25 portal hypertensive controls with hepatitis B or C related cirrhosis and 25 healthy controls, matched for residential locality, were studied. Eighty-four percent to 96 % of study subjects belonged to middle or lower socioeconomic category. Arsenicosis was looked for by estimation of arsenic levels in finger/toe nails and by skin examination. Arsenic levels in nails and in ground water (in NCIPH patients with arsenicosis) was estimated by mass spectrometry. Nail arsenic levels were raised in five (10 %) portal hypertensive study subjects [two NCIPH patients (both had skin arsenicosis) and three portal hypertensive controls]. All of these five patients were residents of West Bengal or Bangladesh. Skin arsenicosis was noted in three NCIPH patients (11 %) compared to none of disease/healthy controls. Ground water from residential locality of one NCIPH patient with arsenicosis (from Bangladesh) showed extremely high level of arsenic (79.5 μg/L). Arsenicosis and microangiopathy of liver, possibly caused by environmental contamination continues in parts of Asia. Further studies are needed to understand the mechanisms of such 'poverty-linked thrombophilia'.

  6. Novel immunohistochemical markers differentiate intrahepatic cholangiocarcinoma from benign bile duct lesions.

    Science.gov (United States)

    Bertram, Stefanie; Padden, Juliet; Kälsch, Julia; Ahrens, Maike; Pott, Leona; Canbay, Ali; Weber, Frank; Fingas, Christian; Hoffmann, Andreas C; Vietor, Antonie; Schlaak, Joerg F; Eisenacher, Martin; Reis, Henning; Sitek, Barbara; Baba, Hideo A

    2016-07-01

    The distinction between intrahepatic cholangiocarcinoma (ICC) and benign bile duct lesions can be challenging. Using our previously identified potential biomarkers for ICC, we examined whether these are useful for the differential diagnosis of ICC, bile duct adenoma and reactive bile duct proliferations in an immunohistochemical approach and identified a diagnostic marker panel including known biomarkers. Subjects included samples from 77 patients with ICC, 33 patients with bile duct adenoma and 47 patients with ductular reactions in liver cirrhosis. Our previously identified biomarkers (stress-induced phosphoprotein 1 (STIP1), SerpinH1, 14-3-3Sigma) were tested immunohistochemically following comparison with candidates from the literature (cluster of differentiation 56, heat shock protein (HSP)27, HSP70, B-cell-lymphoma2, p53, ki67). The expression of SerpinH1 and 14-3-3Sigma was significantly higher in ICC than in bile duct adenomas and ductular reactions (p5% might be used for the distinction of malignant and non-malignant lesions. Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://www.bmj.com/company/products-services/rights-and-licensing/

  7. Predicting IDH mutation status of intrahepatic cholangiocarcinomas based on contrast-enhanced CT features

    Energy Technology Data Exchange (ETDEWEB)

    Zhu, Yong [Nanjing Drum Tower Hospital Clinical College of Traditional Chinese and Western Medicine, Nanjing University of Chinese Medicine, Department of Radiology, Nanjing, Jiangsu Province (China); Chen, Jun [Nanjing Drum Tower Hospital, the Affiliated Hospital of Nanjing University Medical School, Department of Pathology, Nanjing, Jiangsu Province (China); Kong, Weiwei [Nanjing Drum Tower Hospital, the Affiliated Hospital of Nanjing University Medical School, Department of Oncology, Nanjing, Jiangsu Province (China); Mao, Liang; Qiu, Yudong [Nanjing Drum Tower Hospital, the Affiliated Hospital of Nanjing University Medical School, Department of Hepatopancreatobiliary Surgery, Nanjing, Jiangsu Province (China); Kong, Wentao [Nanjing Drum Tower Hospital, the Affiliated Hospital of Nanjing University Medical School, Department of Ultrasonography, Nanjing, Jiangsu Province (China); Zhou, Qun [Nanjing Drum Tower Hospital Clinical College of Nanjing Medical University, Department of Radiology, Nanjing, Jiangsu Province (China); Zhou, Zhengyang; Zhu, Bin; He, Jian [Nanjing Drum Tower Hospital, the Affiliated Hospital of Nanjing University Medical School, Department of Radiology, Nanjing, Jiangsu Province (China); Wang, Zhongqiu [Jiangsu Province Hospital of Traditional Chinese Medicine, the Affiliated Hospital of Nanjing University of Chinese Medicine, Department of Radiology, Nanjing, Jiangsu Province (China)

    2018-01-15

    To explore the difference in contrast-enhanced computed tomography (CT) features of intrahepatic cholangiocarcinomas (ICCs) with different isocitrate dehydrogenase (IDH) mutation status. Clinicopathological and contrast-enhanced CT features of 78 patients with 78 ICCs were retrospectively analysed and compared based on IDH mutation status. There were 11 ICCs with IDH mutation (11/78, 14.1%) and 67 ICCs without IDH mutation (67/78, 85.9%). IDH-mutated ICCs showed intratumoral artery more often than IDH-wild ICCs (p = 0.023). Most ICCs with IDH mutation showed rim and internal enhancement (10/11, 90.9%), while ICCs without IDH mutation often appeared diffuse (26/67, 38.8%) or with no enhancement (4/67, 6.0%) in the arterial phase (p = 0.009). IDH-mutated ICCs showed significantly higher CT values, enhancement degrees and enhancement ratios in arterial and portal venous phases than IDH-wild ICCs (all p < 0.05). The CT value of tumours in the portal venous phase performed best in distinguishing ICCs with and without IDH mutation, with an area under the curve of 0.798 (p = 0.002). ICCs with and without IDH mutation differed significantly in arterial enhancement mode, and the tumour enhancement degree on multiphase contrast-enhanced CT was helpful in predicting IDH mutation status. (orig.)

  8. In vitro proliferation and differentiation of hepatic oval cells and their potential capacity for intrahepatic transplantation

    International Nuclear Information System (INIS)

    Li, Z.; Chen, J.; Li, L.; Ran, J.H.; Liu, J.; Gao, T.X.; Guo, B.Y.; Li, X.H.; Liu, Z.H.; Liu, G.J.; Gao, Y.C.; Zhang, X.L.

    2013-01-01

    Hepatic oval cells (HOCs) are recognized as facultative liver progenitor cells that play a role in liver regeneration after acute liver injury. Here, we investigated the in vitro proliferation and differentiation characteristics of HOCs in order to explore their potential capacity for intrahepatic transplantation. Clusters or scattered HOCs were detected in the portal area and interlobular bile duct in the liver of rats subjected to the modified 2-acetylaminofluorene and partial hepatectomy method. Isolated HOCs were positive for c-kit and CD90 staining (99.8% and 88.8%, respectively), and negative for CD34 staining (3.6%) as shown by immunostaining and flow cytometric analysis. In addition, HOCs could be differentiated into hepatocytes and bile duct epithelial cells after leukemia inhibitory factor deprivation. A two-cuff technique was used for orthotopic liver transplantation, and HOCs were subsequently transplanted into recipients. Biochemical indicators of liver function were assessed 4 weeks after transplantation. HOC transplantation significantly prolonged the median survival time and improved the liver function of rats receiving HOCs compared to controls (P=0.003, Student t-test). Administration of HOCs to rats also receiving liver transplantation significantly reduced acute allograft rejection compared to control liver transplant rats 3 weeks following transplantation (rejection activity index score: control=6.3±0.9; HOC=3.5±1.5; P=0.005). These results indicate that HOCs may be useful in therapeutic liver regeneration after orthotopic liver transplantation

  9. Urinary reducing substances in neonatal intrahepatic cholestasis caused by citrin deficiency

    Directory of Open Access Journals (Sweden)

    Ajmal Kader

    2014-06-01

    Full Text Available Neonatal cholestasis due to citrin deficiency is an autosomal recessive metabolic disorder caused by mutations in SLC25A13 gene. Mutations in this gene have a relatively high prevalence in East-Asian races compared to European or Afro-Caribbean races. Mutations in both sets of chromosomes often lead to self-limiting early onset cholestasis and growth retardation referred as neonatal intrahepatic cholestasis caused by citrin deficiency (NICCD. It is associated with a wide range of metabolic derangements including galactosemia and aminoacidemia, which can be detected on the newborn blood spot screening. Galactose, being a reducing sugar, can also be detected using Clinitest® (Clinitest® Reagent Tablets, Bayer Corporation, Diagnostics Division, Elkhart, IN, USA, a common screening test used in the work up of metabolic and hepatic diseases. In the western population classical galactosemia is often suspected when non glucose reducing substances are detected in the urine of infants with cholestasis. However in East-Asian races the prevalence of classical galactosemia is very low whilst galactosemia due to altered uridine diphosphate-galactose epimerase activity in NICCD is more common. We present a case of NICCD in an East-Asian infant with cholestasis and persistently positive urine reducing substance. Conclusion: NICCD deficiency should be considered as a differential diagnosis in any infant with cholestasis and persistently positive urinary reducing substances.

  10. Effects of Three Different Fibrates on Intrahepatic Cholestasis Experimentally Induced in Rats

    Directory of Open Access Journals (Sweden)

    Alaa El-Sisi

    2013-01-01

    Full Text Available Background. Activation of PPARα modulates cholesterol metabolism and suppresses bile acid synthesis. This study aims to evaluate the effect of PPARα agonists, fenofibrate, bezafibrate, and gemfibrozil, on acute cholestasis induced by ethinylestradiol (EE plus chlorpromazine (CPZ in rats. Method. 100 male albino rats (150–200 gm were divided randomly into 10 equal groups. Control group received 1% methylcellulose vehicle; disease group received CPZ plus EE for 5 consecutive days; four groups received either ursodeoxycholic acid, fenofibrate, bezafibrate, or gemfibrozil for 7 days; 2 days before EE + CPZ, three other groups received one of the three fibrates after GW6471, a selective PPARα antagonist in addition to EE + CPZ. The final group received GW6471 alone. Results. The three fibrates showed marked reduction ( in serum levels of ALP, GGT, ALT, AST, total bile acids, bilirubin, TNFα, and IL-1β and in hepatic malondialdehyde level as well as a significant increase in bile flow rate ( in addition to improvements in histopathological parameters compared to diseased group. In groups which received GW6471, these effects were completely abolished with fenofibrate and partially blocked with bezafibrate and gemfibrozil. Conclusion. Short-term administration of fibrates to EE/CPZ-induced intrahepatic cholestatic rats exerted beneficial effects on hepatocellular damage and apoptosis. Fenofibrate anticholestatic effect was solely PPARα dependent while other mechanisms played part in bezafibrate and gemfibrozil actions.

  11. The transjugular intrahepatic portosystemic stentshunt (TIPSS): A new nonoperative, transjugular percutaneous procedure. [Radiological studies

    Energy Technology Data Exchange (ETDEWEB)

    Richter, G M; Noeldge, G; Siegerstetter, V; Franke, M; Wenz, W; Palmaz, J C; Roessle, M

    1989-08-01

    In a 49-year-old male patient suffering from severe symptoms of end-stage portal hypertension and Child's stage C metabolic status, an intrahepatic stent-assisted portosystemic shunt was established for the first time exclusively by means of interventional radiology. Via transjugular access, a modified Brockenbrough needle was used to puncture the right branch of the portal vein via the right liver vein. As a target, a Dormia-basket was used that had previously been exposed in the right main portal branch. After successful puncture and balloon dilation of the artificial tract, two Palmaz stents were implanted to keep the tract permanently open. The portosystemic pressure gradient dropped from 38 to 18 mm Hg. The clinical status of the patient improved substantially during the following days. However, the patient died on day 11 after the procedure because of sudden onset of acute respiratory distress arising from acute nosocomial fungus and cytomegalovirus infection worsened by his primary immunoincompetence. Autopsy demonstrated a totally patent shunt without superficial thrombus. Microscopically, a thin endothelial layer on the inner shunt surface was found to be present. (orig.).

  12. A New Mutation Causing Progressive Familiar Intrahepatic Cholestasis Type 3 in Association with Autoimmune Hepatitis

    Directory of Open Access Journals (Sweden)

    Hugo M Oliveira

    2017-03-01

    Full Text Available Background: Some patients exhibit features of both autoimmune hepatitis (AIH and primary sclerosing cholangitis (PSC. Similarly, patients with progressive familial intrahepatic cholestasis type 3 (PFIC3 may share histological features with PSC. Case report: We report the case of a 22-year-old man who, since he was 5 years of age, has presented with pruritus, an approximately ninefold elevation of aminotransferases, and γ-glutamyl transferase levels ~10 times the upper limit. Initially he was diagnosed with an overlap syndrome of small duct PSC plus AIH. However, fluctuations in liver enzymes were observed over the following years. Analysis of the ABCB4 gene indicated the diagnosis of PFIC3, revealing a mutation not previously reported. Conclusion: With this case report we aim to describe a new mutation, raise awareness of this rare pathology and highlight the importance of genetic testing of the ABCB4 gene in patients with autoimmune liver disease (mainly small duct PSC with incomplete response to immunosuppressive treatment.

  13. Transjugular Intrahepatic Portosystemic Shunt Placement During Pregnancy: A Case Series of Five Patients

    Energy Technology Data Exchange (ETDEWEB)

    Ingraham, Christopher R., E-mail: cringra@uw.edu; Padia, Siddharth A., E-mail: spadia@uw.edu; Johnson, Guy E., E-mail: gej@uw.edu [University of Washington, Department of Interventional Radiology (United States); Easterling, Thomas R., E-mail: easter@uw.edu [University of Washington, Department of Obstetrics and Gynecology (United States); Liou, Iris W., E-mail: irisl@medicine.washington.edu [University of Washington, Department of Medicine (United States); Kanal, Kalpana M., E-mail: kkanal@uw.edu [University of Washington, Physics Section, Department of Radiology (United States); Valji, Karim, E-mail: kvalji@uw.edu [University of Washington, Department of Interventional Radiology (United States)

    2015-10-15

    Background and AimsComplications of portal hypertension, such as variceal hemorrhage and ascites, are associated with significant increases in both mortality and complications during pregnancy. Transjugular intrahepatic portosystemic shunt (TIPS) is a well-established procedure for treating portal hypertension, but the safety of TIPS during pregnancy is largely unknown. In this series, we review five patients who underwent TIPS placement while pregnant and describe their clinical outcomes.MethodsFive pregnant patients with cirrhosis and portal hypertension underwent elective TIPS for complications of portal hypertension (four for secondary prevention of variceal bleeding and one for refractory ascites). Outcomes measured were recurrent bleeding episodes or need for further paracenteses during pregnancy, estimated radiation dose to the fetus and gestational age at delivery. All patients were followed after delivery to evaluate technical and clinical success of the procedure.ResultsAll five patients survived pregnancy and went on to deliver successfully. When TIPS was performed for secondary prevention of variceal bleeding (n = 4), no patients demonstrated variceal bleeding after TIPS placement. When TIPS was performed for refractory ascites (n = 1), no further paracenteses were required. All patients delivered successfully, albeit prematurely. Average radiation dose estimated to the fetus was 16.3 mGy.ConclusionsThis series suggests that TIPS can be performed in selective pregnant patients with portal hypertension, with little added risk to the mother or fetus.

  14. GNAS1 T393C Polymorphism Is Associated with Clinical Course in Patients with Intrahepatic Cholangiocarcinoma

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    Klaus J. Schmitz

    2007-02-01

    Full Text Available BACKGROUND/AIMS: The GNAS1 locus encodes the Gas protein, which stimulates the formation of cycloadenosinemonophosphate (cAMP. The cAMP pathway mediates pleiotropic effects, including the regulation of apoptosis and proliferation. We have recently shown that TT genotypes of the single-nucleotide polymorphism T393C in the gene GNAS1 predict the clinical outcome of patients with various carcinomas. METHODS: Eighty-seven patients with intrahepatic cholangiocarcinoma (ICC were retrospectively genotyped to elucidate a potential association between T393C genotypes and clinical outcome. RESULTS: ICCs of patients with homozygous TT genotypes revealed a higher proliferation rate and a lower apoptotic rate. Homozygous TT patients were at highest risk for cancer-related deaths (hazard ratio = 2.74; 95% confidence interval = 1.03-7.28 compared with C-allele carriers. Kaplan-Meier curves for disease-specific overall and local recurrence-free survival in a subgroup with Ro-resected ICC showed a significant association of T393 homozygosity with outcome, which was confirmed in multivariate Cox regression analysis. CONCLUSIONS: GNAS1 T393C is a novel independent host factor for disease progression in patients with ICC. Our finding that TT homozygosity (and not CC homozygosity was associated with unfavorable clinical outcome points to the complex and differing functional effects induced by GNAS1 T393C polymorphism in various human carcinomas.

  15. Adopting wedge hepatic venography with CO2 during transjugular intrahepatic portosystemic shunt procedures

    International Nuclear Information System (INIS)

    Zhang Linpeng; Chen Songtao; Shi Xiulan

    2012-01-01

    Objective: To renovate angiography in identifying portal vein anatomy during transjugular intrahepatic portosystemic shunt (TIPS) procedures, saving the time of TIPS procedures, decreasing the risk of the complications of the post-procedure. Methods: The difference between the Wedge hepatic venography with Carbon Dioxide in 6 cases and Inferior Mesenteric artery angiography in 7 cases during TIPS procedures were compared in the identification of portal vein anatomy. The quality of images, their effects on the procedures, the complications and the recovery post-procedure were evaluated. Results: Using CO 2 , the portal veins were opacified in all 6 cases. TIPS procedures succeeded in all cases except 1 case because of poor coagulation function. Using Inferior Mesenteric artery angiography, the portal veins were opacified in all 7 cases. TIPS procedure succeeded in all cases except 1 case because of chronic portal occlusion. Puncture-site hematoma occurred in 1 case after TIPS procedure. Conclusion: Wedge hepatic venography with Carbon Dioxide is superior, safer and more convenient than Inferior Mesenteric Artery angiography in identifying portal vein anatomy during TIPS. (authors)

  16. Shear Wave Elastography of the Spleen for Monitoring Transjugular Intrahepatic Portosystemic Shunt Function: A Pilot Study.

    Science.gov (United States)

    Gao, Jing; Zheng, Xiao; Zheng, Yuan-Yi; Zuo, Guo-Qing; Ran, Hai-Tao; Auh, Yong Ho; Waldron, Levi; Chan, Tiffany; Wang, Zhi-Gang

    2016-05-01

    To assess the feasibility of splenic shear wave elastography in monitoring transjugular intrahepatic portosystemic shunt (TIPS) function. We measured splenic shear wave velocity (SWV), main portal vein velocity (PVV), and splenic vein velocity (SVV) in 33 patients 1 day before and 3 days to 12 months after TIPS placement. We also measured PVV, SVV, and SWV in 10 of 33 patients with TIPS dysfunction 1 day before and 3 to 6 days after TIPS revision. Analyses included differences in portosystemic pressure gradient (PPG), PVV, SVV, and mean SWV before and after TIPS procedures; comparison of median SWV before and after TIPS procedures; differences in PVV, SVV, and SWV before and at different times up to 12 months after TIPS placement; accuracy of PVV, SVV, and SWV in determining TIPS dysfunction; and correlation between PPG and SWV. During 12 months of follow-up, 23 of 33 patients had functioning TIPS, and 10 had TIPS dysfunction. The median SWV was significantly different before and after primary TIPS placement (3.60 versus 3.05 m/s; P = .005), as well as before and after revision (3.73 versus 3.06 m/s; P = .003). The PPG, PVV, and SVV were also significantly different before and after TIPS placement and revision (P function and determining TIPS dysfunction. © 2016 by the American Institute of Ultrasound in Medicine.

  17. Hepatitis viruses infection and risk of intrahepatic cholangiocarcinoma: evidence from a meta-analysis

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    Zhou Yanming

    2012-07-01

    Full Text Available Abstract Background Studies investigating the association between Hepatitis B virus (HBV and hepatitis C virus (HCV infections and intrahepatic cholangiocarcinoma (ICC have reported inconsistent findings. We conducted a meta-analysis of epidemiological studies to explore this relationship. Methods A comprehensive search was conducted to identify the eligible studies of hepatitis infections and ICC risk up to September 2011. Summary odds ratios (OR with their 95% confidence intervals (95% CI were calculated with random-effects models using Review Manager version 5.0. Results Thirteen case–control studies and 3 cohort studies were included in the final analysis. The combined risk estimate of all studies showed statistically significant increased risk of ICC incidence with HBV and HCV infection (OR = 3.17, 95% CI, 1.88-5.34, and OR = 3.42, 95% CI, 1.96-5.99, respectively. For case–control studies alone, the combined OR of infection with HBV and HCV were 2.86 (95% CI, 1.60-5.11 and 3.63 (95% CI, 1.86-7.05, respectively, and for cohort studies alone, the OR of HBV and HCV infection were 5.39 (95% CI, 2.34-12.44 and 2.60 (95% CI, 1.36-4.97, respectively. Conclusions This study suggests that both HBV and HCV infection are associated with an increased risk of ICC.

  18. Serum hepatitis B core-related antigen is a satisfactory surrogate marker of intrahepatic covalently closed circular DNA in chronic hepatitis B.

    Science.gov (United States)

    Chen, En-Qiang; Feng, Shu; Wang, Meng-Lan; Liang, Ling-Bo; Zhou, Ling-Yun; Du, Ling-Yao; Yan, Li-Bo; Tao, Chuan-Min; Tang, Hong

    2017-03-14

    Recently, hepatitis B core-related antigen (HBcrAg) has been suggested as an additional marker of hepatitis B virus (HBV) infection. This study aimed to investigate whether serum quantitative HBcrAg (qHBcrAg) was a satisfactory surrogate marker of intrahepatic covalently closed circular DNA (cccDNA). A total of 139 patients with liver biopsy were enrolled, consisting of 59 patients in immune tolerance (IT) phase, 52 patients in immune clearance (IC) phase, 18 patients in low-replication (LR) phase, and 10 patients in reactivation phase. All patients in IC phase have received entecavir (ETV) therapy, and 32 of them undergone a second liver biopsy at 24 months. Among those patients, qHBcrAg was strongly correlated with intrahepatic cccDNA, which is superior to that of qHBsAg and HBV DNA. And similar findings were also observed in patients in IT, IC, LR and reactivation phases. Among the 32 ETV-treated patients with a second liver biopsy in IC phase, the decline of intrahepatic cccDNA was accompanied by changes in both qHBcrAg and qHBsAg. However, as compared to qHBsAg, the change of qHBcrAg was more strongly associated with intrahepatic cccDNA-decline. In summary, serum qHBcrAg should be a satisfactory surrogate of intrahepatic HBV cccDNA in CHB patients.

  19. Upregulation of PDZK1 by Calculus Bovis Sativus May Play an Important Role in Restoring Biliary Transport Function in Intrahepatic Cholestasis

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    Dong Xiang

    2017-01-01

    Full Text Available Intrahepatic cholestasis is a main cause of hepatic accumulation of bile acids leading to liver injury, fibrosis, and liver failure. Our previous studies proved that Calculus Bovis Sativus (CBS can restore biliary transport function through upregulating the multidrug resistance-associated protein 2 (MRP2 and breast cancer resistance protein (BCRP in 17α-ethynylestradiol- (EE- induced intrahepatic cholestasis rats. The regulation mechanism of CBS on these transporters, however, remains unclear. This study was designed to evaluate the possible relationship between the effect of CBS on transport activities and the regulation of CBS on the expression of PDZK1, a mainly scaffold protein which can regulate MRP2 and BCRP. Intrahepatic cholestasis model was induced in rats with injection of EE for five consecutive days and then the biliary excretion rates and cumulative biliary excretions were measured. The mRNA and protein expression levels of PDZK1 were detected by reverse transcription-quantitative real-time polymerase chain reaction, western blot, and immunohistochemical analysis. When treated with CBS, cumulative biliary excretions and mRNA and protein expressions of PDZK1 were significantly increased in intrahepatic cholestasis rats. This study demonstrated that CBS exerted a beneficial effect on EE-induced intrahepatic cholestasis rats by restoring biliary transport function, which may result from the upregulation of PDZK1 expression.

  20. Tetraspanin CD9 participates in dysmegakaryopoiesis and stromal interactions in primary myelofibrosis

    DEFF Research Database (Denmark)

    Desterke, Christophe; Martinaud, Christophe; Guerton, Bernadette

    2015-01-01

    Primary myelofibrosis is characterized by clonal myeloproliferation, dysmegakaryopoiesis, extramedullary hematopoiesis associated with myelofibrosis and altered stroma in the bone marrow and spleen. The expression of CD9, a tetraspanin known to participate in megakaryopoiesis, platelet formation,...

  1. VASCULAR PROLIFERATION AS AN UNUSUAL CAUSE OF HEMORRHAGIC DIATHESIS IN MYELOFIBROSIS

    NARCIS (Netherlands)

    ALBEDA, FW; VANDERMEER, J; VELLENGA, E

    One year after splenectomy, a patient with myelofibrosis developed spontaneously large hematomas that were not due to coagulation abnormalities or functionally defective platelets. At autopsy, the liver, muscle, and skin showed extramedullary hematopoiesis associated with capillary proliferation and

  2. Exploring the genetic role of the HLA-DPB1 locus in Chileans with intrahepatic cholestasis of pregnancy.

    Science.gov (United States)

    Mella, J G; Roschmann, E; Glasinovic, J C; Alvarado, A; Scrivanti, M; Volk, B A

    1996-03-01

    Intrahepatic cholestasis of pregnancy is a rare disease of unknown etiology, with a strikingly higher prevalence in Chile than in most other countries. Although several studies suggest that a genetic predisposition is involved in the pathogenesis, no genetic disease-marker has so far been identified. Using a recently developed HLA-genotyping technique, we performed an association study with a highly polymorphic HLA class II gene in patients with recurrent intrahepatic cholestasis of pregnancy and normal control patients. Genomic DNA was extracted from 26 unrelated patients with recurrent ICP and 30 unrelated multiparous women without a personal or family history of this disease among a Chilean population. The polymorphic second exon of the HLA-DPB1 gene was amplified by the polymerase chain reaction and hybridized with 25 sequence-specific oligonucleotide probes to assign the HLA-DPB1 alleles on the basis of known sequence variations. Out of more than 50 HLA-DPB1 alleles presently known, 13 were represented in the analyzed groups. Patients with ICP had a higher frequency of the allele DPB*0402 when compared to controls (69% vs 43%). This difference failed to reach statistical significance (x2 = 2.81, corrected p > 0.5). No significant differences were observed between the frequencies of other detected HLA-DPB1 alleles in the analyzed groups. In this study, we observed a high frequency of the allele HLA-DPB1*0402 among Chilean patients with recurrent ICP, but no association of the disease with HLA-DPB1 alleles. Therefore, HLA-DPB1 alleles do not play a major role in determining susceptibility or resistance to intrahepatic cholestasis of pregnancy.

  3. Clinical effect of anatomical hepatectomy in treatment of intrahepatic bile duct stones

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    WANG Dianbei

    2017-01-01

    Full Text Available ObjectiveTo investigate the clinical effect of anatomical hepatectomy in the treatment of intrahepatic bile duct stones. Methods A retrospective analysis was performed for the clinical data of 117 patients with intrahepatic bile duct stones who underwent hepatectomy in The Affiliated Hospital of Zunyi Medical College from July 2013 to December 2015. Among these patients, 66 underwent anatomical hepatectomy (anatomical hepatectomy group and 51 underwent irregular hepatectomy (irregular hepatectomy group. The time of operation, intraoperative blood loss, volume of drainage on the day after surgery, serum levels of aspartate aminotransferase (AST, alanine aminotransferase (ALT, and total bilirubin (TBil, and length of postoperative hospital stay were recorded, as well as the patients′ general status, liver function parameters, and abdominal ultrasound, and CT or MRI findings from 2 months to 2 years after discharge, to observe the presence or absence of residual stones or stone recurrence. The t-test was used for comparison of continuous data between groups, and the chi-square test was used for comparison of categorical data between groups. ResultsCompared with the irregular hepatectomy group, the anatomical hepatectomy group had a significantly longer time of operation (303.1±51.6 min vs 246.5±36.2 min, t=6.963, P<0.01, but it had significantly less intraoperative blood loss (467.9±80.6 ml vs 565.9±92.0 ml, t=6.026, P<0.01, a significantly lower volume of drainage on the day after surgery (212.6±54.9 ml vs 358.3±69.4 ml, t=12.682, P<0.01, a significantly lower incidence rate of complications (10.6% vs 25.5%, χ2=4.497, P=0.034, a significantly lower rate of postoperative residual stones (4.5% vs 15.7%, χ2=4.192, P=0.041, and a significantly shorter length of hospital stay (13.4±2.6 d vs 21.9±3.2 d, t=15.427, P<001. On day 3 after surgery, the anatomical hepatectomy group had significantly lower serum levels of ALT, AST, and

  4. A case of pancreatic arteriovenous malformation with portal hypertension: treatment with transjuguIar intrahepatic portosystemic shunt

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Seong Hoon; Kim, Young Whan; Kim, Yong Joo [School of Medicine, Kyungpook National Univ., Daegu (Korea, Republic of)

    2004-03-01

    Arteriovenous malformation of the pancreas is a rare disease, and it is manifested by gastrointestinal bleeding and/or portal hypertension. Surgery is definitely the treatment of choice at the early stage of the disease, and a transcatheter embolization is an alternative treatment for the control of bleeding and if the lesion is surgically inaccessible. We describe a 62-year-old man who had refractory ascites and esophageal variceal bleeding caused by a pancreatic arteriovenous malformation associated with portal hypertension; this was successfully treated by a transjugular intrahepatic portosystemic shunt.

  5. A case of pancreatic arteriovenous malformation with portal hypertension: treatment with transjuguIar intrahepatic portosystemic shunt

    International Nuclear Information System (INIS)

    Kim, Seong Hoon; Kim, Young Whan; Kim, Yong Joo

    2004-01-01

    Arteriovenous malformation of the pancreas is a rare disease, and it is manifested by gastrointestinal bleeding and/or portal hypertension. Surgery is definitely the treatment of choice at the early stage of the disease, and a transcatheter embolization is an alternative treatment for the control of bleeding and if the lesion is surgically inaccessible. We describe a 62-year-old man who had refractory ascites and esophageal variceal bleeding caused by a pancreatic arteriovenous malformation associated with portal hypertension; this was successfully treated by a transjugular intrahepatic portosystemic shunt

  6. Analysis of hepatitis B virus intrahepatic covalently closed circular DNA and serum viral markers in treatment-naive patients with acute and chronic HBV infection.

    Directory of Open Access Journals (Sweden)

    Weijie Li

    Full Text Available BACKGROUND: This study aimed to investigate the relationships of intrahepatic cccDNA with serum HBsAg and with HBV DNA in treatment-naive patients throughout acute and chronic HBV infection. METHODS: A total of 120 patients who had a liver biopsy were enrolled, including 19 with acute hepatitis B (AHB, and 101 patients with chronic HBV infection (CHB of whom were 10 in immune-tolerant (IT phase, 59 in immune-clearance (IC phase, 8 in low-replicative (LR phase, and 24 in HBeAg-negative hepatitis (ENH phase. Intrahepatic cccDNA, serum HBsAg and serum HBV DNA levels were comparatively analyzed. RESULTS: The median intrahepatic cccDNA levels were 0.18 4.80, 3.81, 0.22 and 0.97 copies/cell for patients with AHB, CHB-IT, CHB-IC, CHB-LR, and CHB-ENH, respectively. In AHB patients, intrahepatic cccDNA was positively correlated with serum HBsAg (r = 0.665, P = 0.003, as well as serum HBV DNA (r = 0.536, P = 0.022. In CHB patients, intrahepatic cccDNA was positively correlated with serum HBsAg in the IC phase (r = 0.392, P = 0.005, and with serum HBV DNA in the IC phase (r = 0.301, P = 0.036 and ENH phase (r = 0.588, P = 0.013. HBV replicative efficiency, defined as the ratio of serum HBV DNA to intrahepatic cccDNA, was obviously lower in AHB and CHB-LR patients than in CHB-IT, CHB-IC and CHB-ENH patients (0.70 and 0.53 vs. 1.12, 1.09 and 0.99, P<0.001, values were logarithmic transformed for analysis. In CHB-IC patients, HBV replicative efficiency was positively correlated with histological activity index of liver inflammation (r = 0.308, P = 0.009. CONCLUSION: Serum HBsAg and HBV DNA levels may reflect the amount of active intrahepatic cccDNA in treatment-naive AHB and CHB-IC patients. Reduced intrahepatic cccDNA and HBV replicative efficiency may imply effective immune control of HBV infection.

  7. Mutation inactivation of Nijmegen breakage syndrome gene (NBS1 in hepatocellular carcinoma and intrahepatic cholangiocarcinoma.

    Directory of Open Access Journals (Sweden)

    Yan Wang

    Full Text Available Nijmegen breakage syndrome (NBS with NBS1 germ-line mutation is a human autosomal recessive disease characterized by genomic instability and enhanced cancer predisposition. The NBS1 gene codes for a protein, Nbs1(p95/Nibrin, involved in the processing/repair of DNA double-strand breaks. Hepatocellular carcinoma (HCC is a complex and heterogeneous tumor with several genomic alterations. Recent studies have shown that heterozygous NBS1 mice exhibited a higher incidence of HCC than did wild-type mice. The objective of the present study is to assess whether NBS1 mutations play a role in the pathogenesis of human primary liver cancer, including HBV-associated HCC and intrahepatic cholangiocarcinoma (ICC. Eight missense NBS1 mutations were identified in six of 64 (9.4% HCCs and two of 18 (11.1% ICCs, whereas only one synonymous mutation was found in 89 control cases of cirrhosis and chronic hepatitis B. Analysis of the functional consequences of the identified NBS1 mutations in Mre11-binding domain showed loss of nuclear localization of Nbs1 partner Mre11, one of the hallmarks for Nbs1 deficiency, in one HCC and two ICCs with NBS1 mutations. Moreover, seven of the eight tumors with NBS1 mutations had at least one genetic alteration in the TP53 pathway, including TP53 mutation, MDM2 amplification, p14ARF homozygous deletion and promoter methylation, implying a synergistic effect of Nbs1 disruption and p53 inactivation. Our findings provide novel insight on the molecular pathogenesis of primary liver cancer characterized by mutation inactivation of NBS1, a DNA repair associated gene.

  8. Patterns of appearance and risk of misdiagnosis of intrahepatic cholangiocarcinoma in cirrhosis at contrast enhanced ultrasound.

    Science.gov (United States)

    Galassi, Marzia; Iavarone, Massimo; Rossi, Sandro; Bota, Simona; Vavassori, Sara; Rosa, Laura; Leoni, Simona; Venerandi, Laura; Marinelli, Sara; Sangiovanni, Angelo; Veronese, Letizia; Fraquelli, Mirella; Granito, Alessandro; Golfieri, Rita; Colombo, Massimo; Bolondi, Luigi; Piscaglia, Fabio

    2013-05-01

    Primary aim was to validate the percentage of intrahepatic cholangiocarcinomas (ICC) which have a contrast vascular pattern at contrast enhanced ultrasound (CEUS) at risk of misdiagnosis with hepatocellular carcinoma (HCC) and, secondary aim, to verify if any characteristics in the CEUS pattern helps to identify ICC. All ICC on cirrhosis seen in three Italian centres (Bologna, Milan and Pavia) between 2003 and 2011, in which CEUS and at least another imaging technique (CT or MRI) had been performed, were retrospectively identified. Those patients with ICC size comparable to the early HCC stage (Milan criteria, considered as small ICC) were enrolled for this study. The enhancement pattern at CEUS was analysed and compared with CT or MRI. A total of 25 small ICC made this study group. CEUS was at risk of misdiagnosis of ICC for HCC in a significantly higher number of cases than in CT (performed in 24 ICC) (52% vs. 4.2%, P = 0.009) and MRI (11 ICC) (52% vs. 9.1%, P = 0.02). A different contrast pattern among all techniques was found in 6 of 10 ICC lesions submitted to the three imaging methods. In the arterial phase, ICC lacked global hyperenhacement in approximately 50% of cases at CEUS and the degree of intensity of wash-out in the late phase was marked in 24% of nodules. CEUS misdiagnosed as HCC a significantly higher number of ICC lesions in cirrhotic patients than CT and MRI. However, some CEUS contrast features can help suspect ICC, especially in some cases with inconclusive CT or MRI. © 2013 John Wiley & Sons A/S.

  9. Rare but Lethal Hepatopathy-Sickle Cell Intrahepatic Cholestasis and Management Strategies.

    Science.gov (United States)

    Malik, Aamir; Merchant, Chandni; Rao, Mana; Fiore, Rosemary P

    2015-11-28

    Sickle cell disease can affect the liver by way of the disease process, including sickling in hepatic sinusoids, as well as its treatment, including repeated blood transfusions leading to hemosiderosis and hepatitis. Sickle cell intrahepatic cholestasis (SCIC) is an extreme variant of sickle cell hepatopathy, and is associated with high fatality. We present the case of a 31-year-old man with past medical history of sickle cell disease and cholecystectomy who was admitted with uncomplicated vaso occlusive crisis and during the hospital stay developed fever, upper abdominal pain, and jaundice. There was an accelerated rise in total bilirubin to 50 mg/dL, direct bilirubin 38 mg/dL, and Cr 3.0 mg/dL. Hb was 6.4 g/dL, reticulocyte count 16%, ALT 40 IU/L, AST 155 IU/L, ALP 320 IU/L, and LDH 475 IU/L. Hepatitis panel was negative and MRCP showed normal caliber of the common bile duct, with no obstruction. Exchange transfusion of 9 units of packed red blood cells led to great improvement in his condition. SCIC, unlike the other sickle cell hepatopathies, requires urgent and vigorous exchange transfusion. Renal impairment in SCIC has not been well studied but usually is reversible with the hepatic impairment, as in this case. Unresolved renal impairment requires dialysis and is associated with poor outcome. There is limited data on use of hydroxyurea to prevent SCIC, and liver transplant is associated with high mortality. A timely diagnosis of SCIC and appropriate management is life-saving.

  10. Combined transjugular intrahepatic portosystemic shunt and other interventions for hepatocellular carcinoma with portal hypertension.

    Science.gov (United States)

    Qiu, Bin; Zhao, Meng-Fei; Yue, Zhen-Dong; Zhao, Hong-Wei; Wang, Lei; Fan, Zhen-Hua; He, Fu-Liang; Dai, Shan; Yao, Jian-Nan; Liu, Fu-Quan

    2015-11-21

    To evaluate combination transjugular intrahepatic portosystemic shunt (TIPS) and other interventions for hepatocellular carcinoma (HCC) and portal hypertension. Two hundred and sixty-one patients with HCC and portal hypertension underwent TIPS combined with other interventional treatments (transarterial chemoembolization/transarterial embolization, radiofrequency ablation, hepatic arterio-portal fistulas embolization, and splenic artery embolization) from January 1997 to January 2010 at Beijing Shijitan Hospital. Two hundred and nine patients (121 male and 88 female, aged 25-69 years, mean 48.3 ± 12.5 years) with complete clinical data were recruited. We evaluated the safety of the procedure (procedure-related death and serious complications), change of portal vein pressure before and after TIPS, symptom relief [e.g., ascites, hydrothorax, esophageal gastric-fundus variceal bleeding (EGVB)], cumulative rates of survival, and distributary channel restenosis. The characteristics of the patients surviving ≥ 5 and portal hypertension symptoms were ameliorated. During the 5 year follow-up, the total recurrence rate of resistant ascites or hydrothorax was 7.2% (15/209); 36.8% (77/209) for EGVB; and 39.2% (82/209) for hepatic encephalopathy. The cumulative rates of distributary channel restenosis at 1, 2, 3, 4, and 5 years were 17.2% (36/209), 29.7% (62/209), 36.8% (77/209), 45.5% (95/209) and 58.4% (122/209), respectively. No procedure-related deaths and serious complications (e.g., abdominal bleeding, hepatic failure, and distant metastasis) occurred. Moreover, Child-Pugh score, portal vein tumor thrombosis, lesion diameter, hepatic arterio-portal fistulas, HCC diagnosed before or after TIPS, stent type, hepatic encephalopathy, and type of other interventional treatments were related to 5 year survival after comparing patient characteristics. TIPS combined with other interventional treatments seems to be safe and efficacious in patients with HCC and portal

  11. Pathological Predictors of Shunt Stenosis and Hepatic Encephalopathy after Transjugular Intrahepatic Portosystemic Shunt

    Directory of Open Access Journals (Sweden)

    Fuliang He

    2016-01-01

    Full Text Available Background. Transjugular intrahepatic portosystemic shunt (TIPS is an artificial channel from the portal vein to the hepatic vein or vena cava for controlling portal vein hypertension. The major drawbacks of TIPS are shunt stenosis and hepatic encephalopathy (HE; previous studies showed that post-TIPS shunt stenosis and HE might be correlated with the pathological features of the liver tissues. Therefore, we analyzed the pathological predictors for clinical outcome, to determine the risk factors for shunt stenosis and HE after TIPS. Methods. We recruited 361 patients who suffered from portal hypertension symptoms and were treated with TIPS from January 2009 to December 2012. Results. Multivariate logistic regression analysis showed that the risk of shunt stenosis was increased with more severe inflammation in the liver tissue (OR, 2.864; 95% CI: 1.466–5.592; P=0.002, HE comorbidity (OR, 6.266; 95% CI, 3.141–12.501; P<0.001, or higher MELD score (95% CI, 1.298–1.731; P<0.001. Higher risk of HE was associated with shunt stenosis comorbidity (OR, 6.266; 95% CI, 3.141–12.501; P<0.001, higher stage of the liver fibrosis (OR, 2.431; 95% CI, 1.355–4.359; P=0.003, and higher MELD score (95% CI, 1.711–2.406; P<0.001. Conclusion. The pathological features can predict individual susceptibility to shunt stenosis and HE.

  12. Fetal outcomes in pregnancies complicated by intrahepatic cholestasis of pregnancy in a Northern California cohort.

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    Michelle Rook

    Full Text Available Intrahepatic cholestasis of pregnancy (ICP has important fetal implications. There is increased risk for poor fetal outcomes, including preterm delivery, meconium staining of amniotic fluid, respiratory distress, fetal distress and demise.One hundred and one women diagnosed with ICP between January 2005 and March 2009 at San Francisco General Hospital were included in this study. Single predictor logistic regression models were used to assess the associations of maternal clinical and biochemical predictors with fetal complications. Clinical predictors analyzed included age, race/ethnicity, gravidity, parity, history of liver or biliary disease, history of ICP in previous pregnancies, and induction. Biochemical predictors analyzed included serum aspartate aminotransferase, alanine aminotransferase, alkaline phosphatase, total bilirubin, direct bilirubin, albumin, total protein, and total bile acids (TBA.The prevalence of ICP was 1.9%. Most were Latina (90%. Labor was induced in the majority (87% and most were delivered by normal spontaneous vaginal delivery (84%. Fetal complications occurred in 33% of the deliveries, with respiratory distress accounting for the majority of complications. There were no statistically significant clinical or biochemical predictors associated with an increased risk of fetal complications. Elevated TBA had little association with fetal complications until reaching greater than 100 µmoL/L, with 3 out of 5 having reported complications. ICP in previous pregnancies was associated with decreased risk of fetal complications (OR 0.21, p = 0.046. There were no cases of late term fetal demise.Maternal clinical and laboratory features, including elevated TBA, did not appear to be substantial predictors of fetal complications in ICP.

  13. Outcomes of Locoregional Tumor Therapy for Patients with Hepatocellular Carcinoma and Transjugular Intrahepatic Portosystemic Shunts

    Energy Technology Data Exchange (ETDEWEB)

    Padia, Siddharth A., E-mail: spadia@uw.edu; Chewning, Rush H., E-mail: rchewnin@uw.edu; Kogut, Matthew J., E-mail: kogutm@uw.edu; Ingraham, Christopher R., E-mail: cringa@uw.edu; Johnson, Guy E., E-mail: gej@uw.edu [University of Washington Medical Center, Section of Interventional Radiology, Department of Radiology (United States); Bhattacharya, Renuka, E-mail: renuka@uw.edu [University of Washington Medical Center, Division of Gastroenterology and Hepatology, Department of Medicine (United States); Kwan, Sharon W., E-mail: shakwan@uw.edu; Monsky, Wayne L., E-mail: wmonsky@uw.edu; Vaidya, Sandeep, E-mail: svaidya@uw.edu [University of Washington Medical Center, Section of Interventional Radiology, Department of Radiology (United States); Hippe, Daniel S., E-mail: dhippe@uw.edu [University of Washington Medical Center, Department of Radiology (United States); Valji, Karim, E-mail: kvalji@uw.edu [University of Washington Medical Center, Section of Interventional Radiology, Department of Radiology (United States)

    2015-08-15

    PurposeLocoregional therapy for hepatocellular carcinoma (HCC) can be challenging in patients with a transjugular intrahepatic portosystemic shunt (TIPS). This study compares safety and imaging response of ablation, chemoembolization, radioembolization, and supportive care in patients with both TIPS and HCC.MethodsThis retrospective study included 48 patients who had both a TIPS and a diagnosis of HCC. Twenty-nine of 48 (60 %) underwent treatment for HCC, and 19/48 (40 %) received best supportive care (i.e., symptomatic management only). While etiology of cirrhosis and indication for TIPS were similar between the two groups, treated patients had better baseline liver function (34 vs. 67 % Child-Pugh class C). Tumor characteristics were similar between the two groups. A total of 39 ablations, 17 chemoembolizations, and 10 yttrium-90 radioembolizations were performed on 29 patients.ResultsAblation procedures resulted in low rates of hepatotoxicity and clinical toxicity. Post-embolization/ablation syndrome occurred more frequently in patients undergoing chemoembolization than ablation (47 vs. 15 %). Significant hepatic dysfunction occurred more frequently in the chemoembolization group than the ablation group. Follow-up imaging response showed objective response in 100 % of ablation procedures, 67 % of radioembolization procedures, and 50 % of chemoembolization procedures (p = 0.001). When censored for OLT, patients undergoing treatment survived longer than patients receiving supportive care (2273 v. 439 days, p = 0.001).ConclusionsAblation appears to be safe and efficacious for HCC in patients with TIPS. Catheter-based approaches are associated with potential increased toxicity in this patient population. Chemoembolization appears to be associated with increased toxicity compared to radioembolization.

  14. Inpatient Cost Assessment of Transjugular Intrahepatic Portosystemic Shunt in the USA from 2001 to 2012.

    Science.gov (United States)

    Kuei, Andrew; Lee, Edward Wolfgang; Saab, Sammy; Busuttil, Ronald W; Durazo, Francisco; Han, Steven-Huy; ElKabany, Mohamed; McWilliams, Justin P; Kee, Stephen T

    2016-10-01

    Despite widespread use of transjugular intrahepatic portosystemic shunt (TIPS) for treatment of portal hypertension, a paucity of nationwide data exists on predictors of the economic impact related to TIPS. Using the National Inpatient Sample (NIS) database from 2001 to 2012, we aimed to evaluate factors contributing to hospital cost of patients admitted to US hospitals for TIPS. Using the NIS, we identified a discharge-weighted national estimate of 61,004 TIPS procedures from 2001 to 2012. Through independent sample analysis, we determined profile factors related to increases in hospital costs. Of all TIPS cases, the mean charge adjusted for inflation to the year 2012 is $125,044 ± $160,115. The mean hospital cost adjusted for inflation is $44,901 ± $54,565. Comparing pre- and post-2005, mean charges and cost have increased considerably ($98,154 vs. $142,652, p < 0.001 and $41,656 vs. $46,453, p < 0.001, respectively). Patients transferred from a different hospital, weekend admissions, Asian/Pacific Islander patients, and hospitals in the Northeastern and Western region had higher cost. Number of diagnoses and number of procedures show positive correlations with hospital cost, with number of procedures exhibiting stronger relationships (Pearson 0.613). Comorbidity measures with highest increases in cost were pulmonary circulation disorders ($32,157 increase, p < 0.001). The cost of the TIPS procedure is gradually rising for hospitals. Alongside recent healthcare reform through the Affordable Care Act, measures to reduce the economic burden of TIPS are of increasing importance. Data from this study are intended to aid physicians and hospitals in identifying improvements that could reduce hospital costs.

  15. Usefulness of Transjugular Intrahepatic Portosystemic Shunt in the Management of Bleeding Ectopic Varices in Cirrhotic Patients

    International Nuclear Information System (INIS)

    Vidal, V.; Joly, L.; Perreault, P.; Bouchard, L.; Lafortune, M.; Pomier-Layrargues, G.

    2006-01-01

    Purpose. To evaluate the safety and efficacy of transjugular intrahepatic portosystemic shunt (TIPS) in the control of bleeding from ectopic varices. Methods. From 1995 to 2004, 24 cirrhotic patients, bleeding from ectopic varices, mean age 54.5 years (range 15-76 years), were treated by TIPS. The etiology of cirrhosis was alcoholic in 13 patients and nonalcoholic in 11 patients. The location of the varices was duodenal (n = 5), stomal (n = 8), ileocolic (n = 6), anorectal (n = 3), umbilical (n = 1), and peritoneal (n 1). Results. TIPS controlled the bleeding in all patients and induced a decrease in the portacaval gradient from 19.7 ± 5.4 to 6.4 ± 3.1 mmHg. Postoperative complications included self-limited intra-abdominal bleeding (n = 2), self-limited hemobilia (n = 1), acute thrombosis of the shunt (n = 1), and bile leak treated by a covered stent (n = 1). Median follow-up was 592 days (range 28-2482 days). Rebleeding occurred in 6 patients. In 2 cases rebleeding was observed despite a post-TIPS portacaval gradient lower than 12 mmHg and was controlled by variceal embolization; 1 patient underwent surgical portacaval shunt and never rebled; in 3 patients rebleeding was related to TIPS stenosis and treated with shunt dilatation with addition of a new stent. The cumulative rate of rebleeding was 23% and 31% at 1 and 2 years, respectively. One- and 2-year survival rates were 80% and 76%, respectively. Conclusion. The present series demonstrates that bleeding from ectopic varices, a challenging clinical problem, can be managed safely by TIPS placement with low rebleeding and good survival rates

  16. Experimental study of an endothelial progenitor cell coated stent in transjugular intrahepatic portosystemic shunt

    International Nuclear Information System (INIS)

    Shi Hongjian; Teng Gaojun; Cao Aihong; Chen Jun; Deng Gang

    2009-01-01

    Objective: To evaluate the efficacy of a self-expandable metal stent coated with autologous endothelial progenitor cells (EPCs) for prevention of restenosis in transjugular intrahepatic portosystemic shunt (TIPS) in a swine model. Methods: EPCs were coated on the metal stents using fibrin gel before TIPS procedure. TIPS was performed in 15 young adult pigs, using an autologous EPC-seeded stent (treatment group, n=9) or a conventional bare metal stent (control group, n=6). All pigs were sacrificed at 2 weeks after TIPS procedure. Portography was performed immediately before the euthanasia. Gross and microscopic pathological exams and immunohistochemical exams of the TIPS track specimens were performed. Fisher test and t test were used to analyse the data. Results: TIPS was performed successfully in all the 15 swine. On day 14 of follow-up, direct portography and necropsy demonstrated that 5 shunts remained patent, 2 shunts stenosed, and the remaining 2 shunts occluded in the treatment group (n=9); while 5 shunts were occluded and one shunt was stenotic in the control group (n=6). The patency rate was 56% vs 0 (P=0.03) between the two groups. Histological analyses showed a greater pseudo-intimal hyperplasia in the TIPS track of the control group than that of the treatment group (pseudointimal thickness at hepatic vein, hepatic parenchyma and portal vein site was (1.2±0.4), (1.3±0.5), (1.5±0.4) mm vs (1.0±0.6), (0.9±0.5), (1.0±0.4) mm respectively (P<0.05). Conclusion: The EPC-coated metal stent is feasibly constructed in vitro and improves the patency in TIPS in a porcine model. (authors)

  17. Transjugular Intrahepatic Portosystemic Shunt Insertion for the Management of Portal Hypertension in Children.

    Science.gov (United States)

    Johansen, Lauren; McKiernan, Patrick; Sharif, Khalid; McGuirk, Simon

    2018-04-17

    To describe our 20-year experience with transjugular intrahepatic portosystemic shunt (TIPSS) procedures for children with resistant portal hypertension (PHTN). Retrospective review of all children that had a TIPSS performed at Birmingham Children's Hospital from 1 January 1995 - 1 January 2015. 40 children underwent 42 attempted TIPSS for resistant PHTN with recurrent variceal bleeding (n = 35), refractory ascites (n = 4), and hypersplenism (n = 1). Median age at operation was 12 years (range, 7 months - 17 years). Thirty-four procedures were elective and 8 were emergency cases.TIPSS was established in 33 cases (79%). Median portal venous pressure reduction was 10mmHg. Variceal bleeding ceased in 27 (96%) and ascites improved in all. Clinical improvement following TIPSS enabled 7 children to be bridged to transplantation and 7 others to become suitable for transplantation. The 1-year and 5-year survival with TIPSS was 57% and 35%, respectively. Child-Pugh score C was an independent risk factor for adverse outcome (LR = 8.0; 95% CI 2.7 - 23.5; P = 0.001).There were 6 major complications: hepatic artery thrombosis and infarction (n = 1), hepatic artery pseudoaneurysm (n = 1), bile leak (n = 1), and hepatic encephalopathy (n = 3). Encephalopathy was resistant to medical treatment in 2 cases, necessitating staged closure in one.Ten patients (30%) required intervention to maintain TIPSS patency. The 1-year and 5-year freedom from reintervention was 71% and 55%, respectively. A TIPSS is highly successful in controlling symptoms in children with resistant PHTN and facilitating liver transplantation. However, it is technically demanding and not without risk. Patients must be appropriately selected and counselled.

  18. A nomogram incorporating six easily obtained parameters to discriminate intrahepatic cholangiocarcinoma and hepatocellular carcinoma.

    Science.gov (United States)

    Wang, Mengmeng; Gao, Yuzhen; Feng, Huijuan; Warner, Elisa; An, Mingrui; Jia, Jian'an; Chen, Shipeng; Fang, Meng; Ji, Jun; Gu, Xing; Gao, Chunfang

    2018-03-01

    Intrahepatic cholangiocarcinoma (ICC) and hepatocellular carcinoma (HCC) are the most prevalent histologic types of primary liver cancer (PLC). Although ICC and HCC share similar risk factors and clinical manifestations, ICC usually bears poorer prognosis than HCC. Confidently discriminating ICC and HCC before surgery is beneficial to both treatment and prognosis. Given the lack of effective differential diagnosis biomarkers and methods, construction of models based on available clinicopathological characteristics is in need. Nomograms present a simple and efficient way to make a discrimination. A total of 2894 patients who underwent surgery for PLC were collected. Of these, 1614 patients formed the training cohort for nomogram construction, and thereafter, 1280 patients formed the validation cohort to confirm the model's performance. Histopathologically confirmed ICC was diagnosed in 401 (24.8%) and 296 (23.1%) patients in these two cohorts, respectively. A nomogram integrating six easily obtained variables (Gender, Hepatitis B surface antigen, Aspartate aminotransferase, Alpha-fetoprotein, Carcinoembryonic antigen, Carbohydrate antigen 19-9) is proposed in accordance with Akaike's Information Criterion (AIC). A score of 15 was determined as the cut-off value, and the corresponding discrimination efficacy was sufficient. Additionally, patients who scored higher than 15 suffered poorer prognosis than those with lower scores, regardless of the subtype of PLC. A nomogram for clinical discrimination of ICC and HCC has been established, where a higher score indicates ICC and poor prognosis. Further application of this nomogram in multicenter investigations may confirm the practicality of this tool for future clinical use. © 2018 The Authors. Cancer Medicine published by John Wiley & Sons Ltd.

  19. Anatomic variations in intrahepatic bile ducts in a north Indian population.

    Science.gov (United States)

    Sharma, Vijay; Saraswat, Vivek Anand; Baijal, Sanjay Saran; Choudhuri, Gourdas

    2008-07-01

    In the present study, we described the anatomical variations in the branching patterns of intrahepatic bile ducts (IHD) and determined the frequency of each variation in north Indian patients. There are no data from India. The study group consisted of 253 consecutive patients (131 women) undergoing endoscopic retrograde cholangiograms for different indications. Anatomical variations in IHD were classified according to the branching pattern of the right anterior segmental duct (RASD) and the right posterior segmental duct (RPSD), presence or absence of first-order branch of left hepatic duct (LHD) and of an accessory hepatic duct. Anatomy of the IHD was typical in 52.9% of cases (n = 134), showing triple confluence in 11.46% (n = 29), anomalous drainage of the RPSD into the LHD in 18.2% (n = 46), anomalous drainage of the RPSD into the common hepatic duct (CHD) in 7.1% (n = 18), drainage of the right hepatic duct (RHD) into the cystic duct 0.4% (n = 1), presence of an accessory duct leading to the CHD or RHD in 4.7% (n = 12), individual drainage of the LHD into the RHD or CHD in 2.4% (n = 6), and unclassified or complex variations in 2.7% (n = 7). None had anomalous drainage of RPSD into the cystic duct. The branching pattern of IHD was atypical in 47% patients. The two most common variations were drainage of the RPSD into the LHD (18.2%) and triple confluence of the RASD, RPSD, and LHD (11.5%).

  20. Radiation dose reduction during transjugular intrahepatic portosystemic shunt implantation using a new imaging technology

    Energy Technology Data Exchange (ETDEWEB)

    Spink, C., E-mail: c.spink@uke.de [Department of Diagnostic and Interventional Radiology, University Medical Center Hamburg-Eppendorf, Hamburg (Germany); Avanesov, M. [Department of Diagnostic and Interventional Radiology, University Medical Center Hamburg-Eppendorf, Hamburg (Germany); Schmidt, T. [Philips Healthcare, Hamburg (Germany); Grass, M. [Philips Research, Hamburg (Germany); Schoen, G. [Department of Medical Biometry and Epidemiology, University Medical Center Hamburg-Eppendorf, Hamburg (Germany); Adam, G.; Bannas, P.; Koops, A. [Department of Diagnostic and Interventional Radiology, University Medical Center Hamburg-Eppendorf, Hamburg (Germany)

    2017-01-15

    Highlights: • The new imaging technology halved the radiation exposure. • DSA image quality observed was not decreased after technology upgrade. • Radiation time and contrast consumption not significantly increased using the new technology. - Abstract: Objective: To compare patient radiation dose in patients undergoing transjugular intrahepatic portosystemic shunt (TIPS) implantation before and after an imaging-processing technology upgrade. Methods: In our retrospective single-center-study, cumulative air kerma (AK), cumulative dose area product (DAP), total fluoroscopy time and contrast agent were collected from an age- and BMI-matched collective of 108 patients undergoing TIPS implantation. 54 procedures were performed before and 54 after the technology upgrade. Mean values were calculated and compared using two-tailed t-tests. Two blinded, independent readers assessed DSA image quality using a four-rank likert scale and the Wilcoxcon test. Results: The new technology demonstrated a significant reduction of 57% of mean DAP (402.8 vs. 173.3 Gycm{sup 2}, p < 0.001) and a significant reduction of 58% of mean AK (1.7 vs. 0.7 Gy, p < 0.001) compared to the precursor technology. Time of fluoroscopy (26.4 vs. 27.8 min, p = 0.45) and amount of contrast agent (109.4 vs. 114.9 ml, p = 0.62) did not differ significantly between the two groups. The DSA image quality of the new technology was not inferior (2.66 vs. 2.77, p = 0.56). Conclusions: In our study the new imaging technology halved radiation dose in patients undergoing TIPS maintaining sufficient image quality without a significant increase in radiation time or contrast consumption.