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Sample records for alloimmune thrombocytopenic purpura

  1. Neonatal alloimmun trombocytopenisk purpura (NAITP

    Directory of Open Access Journals (Sweden)

    Bjørn Skogen

    2009-10-01

    Full Text Available  SAMMENDRAGNeonatal alloimmun trombocytopenisk purpura (NAITP opptrer før eller like etter fødselen, og erforårsaket av maternell alloimmunisering mot paternelle (føtale antigener som ikke er tilstede på morenstrombocytter. Incidensen er 1 pr. 2000/3000 nyfødte. Det finnes ikke noe screening-opplegg for å avsløremødre som kommer til å føde barn med NAITP. Derfor fins det heller ikke noe man kan gjøre før enkvinne føder sitt første barn med tilstanden. I påfølgende svangerskap kan moren følges, og man kanplanlegge tiltak for å redusere risikoen for skade på barnet.Skogen B. Neonatal alloimmune thrombocytopenic purpura. Nor J Epidemiol 1997; 7 (1: 69-72. ENGLISH SUMMARYNeonatal alloimmune thrombocytopenic purpura (NAITP manifests itself before or shortly after birthand is caused by maternal alloimmunization to a paternal (fetal antigen not present on the mother’splatelets. The incidence of the condition in the fetus and neonate is 1 in 2000/3000 live births. There areno screening programs for detecting mothers at risk of delivering infants affected with NAITP. Therefore,no antenatal management is possible in first pregnancies. In subsequent pregnancies there is an opportunityto detect affected fetuses and plan perinatal therapy.

  2. Living with Thrombotic Thrombocytopenic Purpura

    Science.gov (United States)

    ... Some people fully recover from thrombotic thrombocytopenic purpura (TTP). However, relapses (flareups) can occur in many people who have acquired and inherited TTP. If you've had TTP, call your doctor ...

  3. What Causes Thrombotic Thrombocytopenic Purpura?

    Science.gov (United States)

    ... protein in the blood) causes thrombotic thrombocytopenic purpura (TTP). The ADAMTS13 gene controls the enzyme, which is ... enough enzyme activity causes overactive blood clotting. In TTP, blood clots form in small blood vessels throughout ...

  4. Idiopathic thrombocytopenic purpura

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    L Kayal

    2014-01-01

    Full Text Available Idiopathic thrombocytopenic purpura (ITP is defined as a hematologic disorder, characterized by isolated thrombocytopenia without a clinically apparent cause. The major causes of accelerated platelet consumption include immune thrombocytopenia, decreased bone marrow production, and increased splenic sequestration. The clinical presentation may be acute with severe bleeding, or insidious with slow development with mild or no symptoms. The initial laboratory tests useful at the first visit to predict future diagnosis were erythrocyte count, leukocyte count, anti-glycoprotein IIb/IIIa antibodies, reticulated platelets, plasma thrombopoietin level. Treatment should be restricted to those patients with moderate or severe thrombocytopenia who are bleeding or at risk of bleeding. We present a case report on ITP with clinical presentation, diagnosis and management.

  5. How Is Thrombotic Thrombocytopenic Purpura Treated?

    Science.gov (United States)

    ... Is Thrombotic Thrombocytopenic Purpura Treated? Thrombotic thrombocytopenic purpura (TTP) can be fatal or cause lasting damage, such ... it's not treated right away. In most cases, TTP occurs suddenly and lasts for days or weeks, ...

  6. Hematologic case: Idiopathic thrombocytopenic purpura

    OpenAIRE

    São Simão, T.; Salgado, M.; Costa, E.; Barbot, J.

    2012-01-01

    The immune thrombocytopenic purpura (ITP) is a controversial disease. The generality of the literature argues that a historical objective clinical examination and a blood count with careful observation of the peripheral blood smear is sufficient for diagnosis. Some cases contradict this belief.

  7. Haemolytic uraemic syndrome and thrombocytopenic thrombotic purpura

    NARCIS (Netherlands)

    Zijlstra, JG

    1997-01-01

    Haemolytic uraemic syndrome thrombocytopenic thrombotic purpura (HUS/TTP) remains an incompletely understood complex disease process that involves many organs. It was first described, as thrombocytopenic purpura, by Moschcowitz in 1924 (1). Since that time the prognosis of this disease has improved

  8. How Is Thrombotic Thrombocytopenic Purpura Diagnosed?

    Science.gov (United States)

    ... Diagnosed? Your doctor will diagnosis thrombotic thrombocytopenic purpura (TTP) based on your medical history, a physical exam, and test results. If TTP is suspected or diagnosed, a hematologist will be ...

  9. Crohn's colitis and idiopathic thrombocytopenic purpura

    OpenAIRE

    Boyne, M.; Dye, K.

    2000-01-01

    A 17 year old girl with active Crohn's colitis developed idiopathic thrombocytopenic purpura that was managed with intravenous immune globulins and cyclosporin A. The possible association between Crohn's disease and immune thrombocytopenia is explored.


Keywords: Crohn's disease; colitis; thrombocytopenia

  10. Plasma exchange in thrombotic thrombocytopenic purpura.

    OpenAIRE

    Toffelmire, E B; Clark, W. F.; Cordy, P. E.; Linton, A. L.; Lohmann, R. C.

    1984-01-01

    Three patients were recently treated for thrombotic thrombocytopenic purpura (TTP). One presented with toxic shock syndrome; TTP developed but promptly responded to a regimen of antiplatelet agents, steroids and plasma exchange. In another the manifestations of TTP developed after presentation with hypertension and abdominal pain. This patient responded to a similar regimen but required extended treatment before remission could be maintained with medications alone. In the third patient the fu...

  11. The Kidney in Thrombotic Thrombocytopenic Purpura

    OpenAIRE

    Tsai, Han-Mou

    2007-01-01

    The kidney is commonly affected in thrombotic thrombocytopenic purpura (TTP), a multi-system disorder with microvascular thrombosis of the capillaries and arterioles. Nevertheless, due to difference in its diagnostic criteria, the frequency and severity of renal dysfunction in TTP remains controversial. With the recent studies indicating that severe deficiency of a VWF cleaving protease, ADAMTS13, is the main cause of platelet thrombosis in TTP, it is now possible to define TTP at the molecul...

  12. Thrombotic thrombocytopenic purpura in childhood

    NARCIS (Netherlands)

    M.C. Bouw; N. Dors; H. van Ommen; N.L. Ramakers-van Woerden

    2009-01-01

    Thrombotic thrombocytopenic puripura (TTP) is a rare disease, especially in childhood, and has a high mortality rate in the absence of appropriate treatment. it is characterised by microangiopathic haemolytic anaemia and consumptive thrombocytopenia. TTP may be difficult to distinguish from haemolyt

  13. [New marker in thrombotic thrombocytopenic purpura

    DEFF Research Database (Denmark)

    Hillarp, A.; Lindblom, A.; Bjork, P.;

    2008-01-01

    Thrombotic microangiopathy can be caused by several conditions which are difficult to diagnose from the clinical presentation alone. Deficient enzyme activity of a newly-discovered enzyme, ADAMTS-13, can lead to thrombotic thrombocytopenic purpura (TTP). Lack of ADAMTS-13 activity causes increased...... concentrations of high molecular weight von Willebrand factor forms and increased platelet aggregation. Measurement of ADAMTS-13 activity is useful for the diagnosis of TTP and may also be relevant as a prognostic test for recurrent TTP Udgivelsesdato: 2008/8/11...

  14. A case of immune thrombocytopenic purpura presenting with intracranial hemorrhage

    Institute of Scientific and Technical Information of China (English)

    Sinan Akbayram; Fesih Aktar; Cihangir Akgn; Mehmet Seluk Bekta; Hseyinaksen; Ahmet Faik Oner

    2013-01-01

    Immune thrombocytopenic purpura is an acute, generally considered a self-limiting benign disorder with a60%-80% change of spontaneous recovery occurring usually within a few months after onset.Intracranial hemorrhage is a rare but life-threatening complication of childhood immune thrombocytopenic purpura.We report a4-year-old girl who admitted with headache, vomiting, bleeding from noise and bruises on the extremities.Her neurological examination was normal.Based on laboratory finding she was diagnosed immune thrombocytopenic purpura and intracranial hemorrhage.We suggest that cranial imaging should be perform in patients with immune thrombocytopenic purpura admitted with bleeding symptoms, vomiting and headache even if they had no abnormal neurological signs.

  15. Chronic idiopathic thrombocytopenic purpura: present strategy, guidelines and new insights

    NARCIS (Netherlands)

    W. Stevens; H. Koene; J.J. Zwaginga; G. Vreugdenhil

    2006-01-01

    Idiopathic thrombocytopenic purpura. (ITP) is an immune-mediated thrombocytopenia. The diagnosis is made after exclusion of other secondary causes of thrombocytopenic disorders. The primary treatment goal is to prevent severe bleeding rather than achieve normal platelet counts. In adults ITP usually

  16. Recombinant thrombomodulin for secondary thrombotic thrombocytopenic purpura.

    Science.gov (United States)

    Nakamura, Kensuke; Inokuchi, Ryota; Hiruma, Takahiro; Ohshima, Kazuma; Sonoo, Tomohiro; Tokunaga, Kurato; Doi, Kent; Nakajima, Susumu

    2016-06-01

    In the pathogenesis of thrombotic thrombocytopenic purpura (TTP), reductions in the enzyme activity of ADAMTS13, which cuts ultralarge von Willebrand multimers, generates shear stress on the microvascular endothelium, leading to platelet aggregation and the formation of a thrombus. ADAMTS13 activity is markedly decreased in typical TTP, but is only mildly reduced in secondary TTP, which concomitantly develops with primary disease. The latter develops with septic disseminated intravascular coagulation (DIC) and often causes organ failure. Recombinant thrombomodulin (rTM) is a drug that is used to treat DIC and may also remit TTP because it improves vascular endothelial dysfunction. Therefore, we herein investigated the efficacy of rTM in patients treated for the pathology of secondary TTP. Patients who were admitted to the Emergency and Critical Care Center of our hospital and met the following conditions were extracted and retrospectively analyzed: hemolytic anemia accompanied by fragmented red blood cells (Hb TTP, significantly increased in the rTM treatment group: 3.3 ± 2.6→11.3 ± 14.6 versus 3.5 ± 3.7→5.7 ± 3.9 (×1000/μL) (P = 0.034). Thrombotic thrombocytopenic purpura originally requires invasive treatments and its prognosis is not favorable. Blood thrombomodulin levels also markedly increase due to vascular endothelial dysfunction, whereas rTM alleviates vascular endothelial dysfunction in TTP patients with high blood TM levels, suggesting the importance of administering rTM. Thus, rTM may be effective for secondary TTP and may be adopted as adjuvant therapy. PMID:27310951

  17. Pathophysiology of thrombotic thrombocytopenic purpura : the "two-hit" paradigm

    NARCIS (Netherlands)

    Lotta, Luca Andrea

    2012-01-01

    Thrombotic thrombocytopenic purpura (TTP) is a rare, life-threatening disease characterized by acute episodes of widespread thrombosis in capillaries and small arteries. The discovery that the plasmatic activity of the von Willebrand factor cleaving protease, ADAMTS13, is severely deficient in patie

  18. Splenectomy for the treatment of thrombotic thrombocytopenic purpura

    NARCIS (Netherlands)

    Kappers-Klunne, MC; Wijermans, P; Fijnheer, R; Croockewit, AJ; van der Holt, B; de Wolf, JTM; Lowenberg, B; Brand, A

    2005-01-01

    Plasma exchange is the treatment of choice for patients with thrombotic thrombocytopenic purpura (TTP) and results in remission in >80% of the cases. Treatment of patients who are refractory to plasma therapy or have relapsing disease is difficult. Splenectomy has been a therapeutic option in these

  19. Active von Willebrand factor in thrombotic thrombocytopenic purpura and malaria

    NARCIS (Netherlands)

    Groot, E.

    2009-01-01

    Thrombotic thrombocytopenic purpura (TTP) and malaria are two diseases of distinct origin. TTP is a rare disorder caused by a deficiency of the von Willebrand factor (VWF) cleaving protease ADAMTS13. Malaria is a poverty-related disease caused by protozoan parasites from the genus Plasmodium. TTP an

  20. Opana® ER induced thrombotic thrombocytopenic purpura

    OpenAIRE

    Kotbi, Nabil; Han,Bernadine; Cheng,Duncan; Odom, Anna E

    2015-01-01

    We present the case of a patient who developed thrombotic thrombocytopenic purpura (TTP) following intravenous injection of Opana® ER. TTP reemerged after three months of abstinence with Opana misuse. This case report brings awareness to the possibility of developing TTP in those who misuse Opana, which is a growing concern.

  1. Immune thrombocytopenic purpura-related hemotympanum presenting with hearing loss.

    Science.gov (United States)

    Fisgin, Tunc; Atmaca, Sinan; Duru, Feride; Ozyurek, Emel; Cetin, Recep; Albayrak, Davut

    2009-06-01

    A 5-year-old boy was admitted to our center with a major complaint of bilateral hearing loss for 2 days. He was diagnosed with acute immune thrombocytopenic purpura 3 months before the admission and treated with high-dose methylprednisolone 2 months ago. Physical examination revealed wet purpura in the oral mucosa, serous nasal discharge, multiple petechiae and ecchymosis of the lower lip. Otomicroscopic ear examination revealed the presence of bilateral hemotympanum. The patient denied head trauma, ear pain, fever, hypertension and medications, including salicylates. The patient received high-dose intravenous methylprednisolone because of low platelet count and wet purpura for 7 days and oral prophylactic amoxicillin-clavulanate for 14 days. The onset of the response to corticosteroids was rapid, and significant hematologic improvement was observed within a few days. The 2-week follow-up examination revealed intact tympanic membranes with normal color and mobility, and the patient restored normal hearing. In this patient, hemotympanum developed rapidly, and no predisposing cause other than immune thrombocytopenic purpura was found. However, presence of a serous nasal discharge may be a sign of viral upper respiratory tract infection. Therefore, it can be speculated that sneezing or coughing might have caused bilateral hemotympanum by increasing the middle ear pressure abruptly. We would like to emphasize that bleeding may occur in unusual sites and, unlike in healthy people, may cause bizarre symptoms in patients with bleeding diathesis. Hemotympanum can be considered among the indications to start treatment in patients with acute immune thrombocytopenic purpura. PMID:19530341

  2. Recurrent Acute Myocardial Infarction in Patients with Immune Thrombocytopenic Purpura

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    Fengyi Shen

    2014-01-01

    Full Text Available Immune thrombocytopenic purpura (ITP, also known as idiopathic thrombocytopenic purpura, is an acquired immune-mediated disease of adults and children characterized by a transient or persistent decrease of platelets and, depending upon the degree of thrombocytopenia, an increased risk of bleeding. The use of standard treatments for acute myocardial infarction (AMI, such as antiplatelet agents and anticoagulants, pose serious problems in patients with ITP due to the potential higher risk of bleeding complications. There are no current guidelines available for management of ITP patients with AMI. In this brief review of the limited available literature, we discuss the proposed pathophysiological link between ITP and arterial thrombosis and the challenging medical and interventional treatment of these patients.

  3. Thrombocytopenic purpura as adverse reaction to recombinant hepatitis B vaccine

    OpenAIRE

    Ronchi, F; Cecchi, P; Falcioni, F.; Marsciani, A; Minak, G.; Muratori, G; Tazzari, P; Beverini, S

    1998-01-01

    Three cases of immune thrombocytopenic purpura after the first dose of recombinant hepatitis B vaccine occurred in infants under 6 months of age. Other possible causes of this condition were excluded. Antiplatelet antibodies were present. A defect in platelet production was excluded in two children. Corticosteroid treatment was effective. Subsequent administration of other vaccines (against polio, diphtheria, and tetanus) did not cause relapse of thrombocytopenia.



  4. Risk Factors for Autoimmune Diseases Development After Thrombotic Thrombocytopenic Purpura

    OpenAIRE

    Roriz, Mélanie; Landais, Mickael; Desprez, Jonathan; Barbet, Christelle; Azoulay, Elie; Galicier, Lionel; Wynckel, Alain; Baudel, Jean-luc; Provôt, François; Pène, Frédéric; Mira, Jean-Paul; Presne, Claire; Poullin, Pascale; Delmas, Yahsou; Kanouni, Tarik

    2015-01-01

    Abstract Autoimmune thrombotic thrombocytopenic purpura (TTP) can be associated with other autoimmune disorders, but their prevalence following autoimmune TTP remains unknown. To assess the prevalence of autoimmune disorders associated with TTP and to determine risk factors for and the time course of the development of an autoimmune disorder after a TTP episode, we performed a cross sectional study. Two-hundred sixty-one cases of autoimmune TTP were included in the French Reference Center reg...

  5. Thrombotic thrombocytopenic purpura: The role of ADAMTS13.

    Science.gov (United States)

    Rogers, Heesun J; Allen, Charles; Lichtin, Alan E

    2016-08-01

    Thrombotic thrombocytopenic purpura (TTP) is an uncommon, life-threatening disease requiring prompt diagnosis and initiation of therapeutic plasma exchange to improve patient survival. However, diagnosis is often difficult because of atypical presentations and signs and symptoms that resemble other conditions. Measurements of ADAMTS13 activity, ADAMTS13 inhibitor, and ADAMTS13 autoantibody are useful for diagnosing TTP, guiding therapy, and predicting relapse. PMID:27505881

  6. Pathophysiology of thrombotic thrombocytopenic purpura: the "two-hit" paradigm

    OpenAIRE

    Lotta, Luca Andrea

    2012-01-01

    Thrombotic thrombocytopenic purpura (TTP) is a rare, life-threatening disease characterized by acute episodes of widespread thrombosis in capillaries and small arteries. The discovery that the plasmatic activity of the von Willebrand factor cleaving protease, ADAMTS13, is severely deficient in patients with TTP represented a turning point in the understanding of the pathophysiology of the disease. In spite of recent advances, the clinical course of TTP is characterized by considerable heterog...

  7. Thrombotic thrombocytopenic purpura in the first trimester of pregnancy

    Directory of Open Access Journals (Sweden)

    Pooja Sikka

    2013-01-01

    Full Text Available Thrombotic thrombocytopenic purpura (TTP occurs more commonly in women and so can be associated with pregnancy. The time during pregnancy with greatest risk for development of TTP is near term and during the post partum period. TTP occurring in early trimester is uncommon and is also associated with great maternal and fetal mortality. We report a successful outcome of pregnancy in a woman with TTP in early first trimester who was treated with therapeutic plasma exchange.

  8. A report of disseminated adenocarcinoma presenting as thrombotic thrombocytopenic purpura

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    Joaquín Valle Alonso

    2011-10-01

    Full Text Available Thrombotic microangiopathies (TMAs represent a heterogeneous group of diseases characterized by a microangiopathic hemolytic anemia, peripheral thrombocytopenia, and organ failure of variable severity. TMAs encompass thrombotic thrombocytopenic purpura (TTP, typically characterized by fever, central nervous system manifestations and hemolytic uremic syndrome (HUS, in which renal failure is the prominent abnormality. In patients with cancer TMAs may be related to various antineoplastic drugs or to the malignant disease itself. The reported series of patients with TMAs directly related to cancer are usually heterogeneous, retrospective, and encompass patients with hematologic malignancies with solid tumors or receiving chemotherapy, each of which may have distinct presentations and pathophysiological mechanisms. Patients with disseminated malignancy who present with microangiopathic hemolytic anemia and thrombocytopenia may be misdiagnosed as thrombotic thrombocytopenic purpura (TTP. Only a few cases of TTP secondary to metastatic adenocarcinoma are known in the literature. We present a case of a 34-year-old man with TTP syndrome secondary to metastatic small-bowel adenocarcinoma. Patients with disseminated malignancy had a longer duration of symptoms, more frequent presence of respiratory symptoms, higher lactate dehydrogenase levels, and more often failed to respond to plasma exchange treatment. A search for systemic malignancy, including a bone marrow biopsy, is appropriate when patients with TTP have atypical clinical features or fail to respond to plasma exchange.

  9. A case of thrombotic thrombocytopenic purpura induced by acute pancreatitis

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    Arimoto M

    2012-03-01

    Full Text Available Miyoko Arimoto1, Yutaka Komiyama2, Fumiko Okamae1, Akemi Ichibe1, Setsuko Teranishi1, Hirohiko Tokunaga1, Keiko Nakaya3, Michie Fujiwara3, Manabu Yamaoka4, Shuji Onishi4, Rie Miyamoto5, Naoto Nakamichi5, Shosaku Nomura51Blood Transfusion Unit, Kansai Medical University Takii Hospital, 2Department of Clinical Sciences and Laboratory Medicine, Kansai Medical University, 3Clinical Medical Technology Unit, Kansai Medical University Takii Hospital, 4Blood Transfusion Unit, Kansai Medical University Hirakata Hospital, 5First Department of Internal Medicine, Kansai Medical University, Moriguchi, JapanAbstract: Thrombotic thrombocytopenic purpura (TTP is a multisystemic microvascular disorder that may be caused by an imbalance between unusually large von Willebrand factor multimers and the cleaving protease ADAMTS13. In acquired TTP, especially in secondary TTP with various underlying diseases, the diagnosis is difficult because there are many cases that do not exhibit severe deficiency of ADAMTS13 or raised levels of ADAMST13 inhibitors. It is well known that collagen disease, malignancy, and hematopoietic stem cell transplantation can be underlying conditions that induce TTP. However, TTP induced by acute pancreatitis, as experienced by our patient, has rarely been reported. Our patient completely recovered with treatments using steroids and plasma exchange (PE only. In cases where patients develop acute pancreatitis with no apparent causes for hemolytic anemia and thrombocytopenia, the possibility of TTP should be considered. Treatments for TTP including PE should be evaluated as soon as a diagnosis is made.Keywords: thrombotic thrombocytopenic purpura, ADAMTS13, acute pancreatitis, plasma exchange

  10. Two Mechanistic Pathways for Thienopyridine-Associated Thrombotic Thrombocytopenic Purpura

    Science.gov (United States)

    Bennett, Charles L.; Kim, Benjamin; Zakarija, Anaadriana; Bandarenko, Nicholas; Pandey, Dilip K.; Buffie, Charlie G.; McKoy, June M.; Tevar, Amul D.; Cursio, John F.; Yarnold, Paul R.; Kwaan, Hau C.; De Masi, Davide; Sarode, Ravindra; Raife, Thomas J.; Kiss, Joseph E.; Raisch, Dennis W.; Davidson, Charles; Sadler, J. Evan; Ortel, Thomas L.; Zheng, X. Long; Kato, Seiji; Matsumoto, Masanori; Uemura, Masahito; Fujimura, Yoshihiro

    2011-01-01

    Objectives We sought to describe clinical and laboratory findings for a large cohort of patients with thienopyridine-associated thrombotic thrombocytopenic purpura (TTP). Background The thienopyridine derivatives, ticlopidine and clopidogrel, are the 2 most common drugs associated with TTP in databases maintained by the U.S. Food and Drug Administration (FDA). Methods Clinical reports of TTP associated with clopidogrel and ticlopidine were identified from medical records, published case reports, and FDA case reports (n = 128). Duration of thienopyridine exposure, clinical and laboratory findings, and survival were recorded. ADAMTS13 activity (n = 39) and inhibitor (n = 30) were measured for a subset of individuals. Results Compared with clopidogrel-associated TTP cases (n = 35), ticlopidine-associated TTP cases (n = 93) were more likely to have received more than 2 weeks of drug (90% vs. 26%), to be severely thrombocytopenic (84% vs. 60%), and to have normal renal function (72% vs. 45%) (p 15% (n = 13), TTP patients with severely deficient ADAMTS13 activity (n = 26) were more likely to have received ticlopidine (92.3% vs. 46.2%, p 2 weeks after thienopyridine, therapeutic plasma exchange (TPE) increased likelihood of survival (84% vs. 38%, p < 0.05). Among patients who developed TTP within 2 weeks of starting thienopyridines, survival was 77% with TPE and 78% without. Conclusions Thrombotic thrombocytopenic purpura is a rare complication of thienopyridine treatment. This drug toxicity appears to occur by 2 different mechanistic pathways, characterized primarily by time of onset before versus after 2 weeks of thienopyridine administration. If TTP occurs after 2 weeks of ticlopidine or clopidogrel therapy, therapeutic plasma exchange must be promptly instituted to enhance likelihood of survival. PMID:17868804

  11. Studies on megakaryopoiesis in patients with myelodysplasia and idiopathic thrombocytopenic purpura

    NARCIS (Netherlands)

    Houwerzijl, Ewout Johan

    2008-01-01

    In this thesis mechanisms of thrombocytopenia (a low number of platelets) in patients with myelodysplastic syndromes (MDS) or idiopathic thrombocytopenic purpura (ITP) were investigated. Thrombocytopenia can cause serious bleeding complications and elucidation of the underlying pathophysiology of th

  12. Current Concept for the Diagnosis and Treatment of Adult Idiopathic Thrombocytopenic Purpura

    Institute of Scientific and Technical Information of China (English)

    侯明

    2005-01-01

    @@ Idiopathic thrombocytopenic purpura (ITP) is a common hematologic disorder manifested by immunemediated thrombocytopenia. The estimated incidence ranges from 50 ~ 100 per million each year in western countries, roughly divided between adults and children.

  13. Immune thrombocytopenic purpura secondary to cytomegalovirus infection: A case report.

    Directory of Open Access Journals (Sweden)

    Bessy S Flores Chang

    2015-11-01

    Full Text Available Immune Thrombocytopenic Purpura (ITP is defined as an acquired thrombocytopenia with antibodies detected against platelet surface antigens, and it is the most common form of thrombocytopenia in otherwise asymptomatic adults. ITP secondary to an underlying condition is a diagnosis of exclusion that is essential to establish for treatment efficacy. Secondary thrombocytopenia caused by Cytomegalovirus (CMV is common, however case reports associated with diagnosis in immunocompetent adults are rare, and to the best of our knowledge only 20 publications have been associated with this diagnosis. Our report is based on a clinical presentation of a 37 year old female complaining of petechiae, heavy menses, shortness of breath and a platelet count of 1 X 109 /L. Treatment with IVIG and steroids failed to improve platelet count. Subsequently, an infectious laboratory workup was performed, detecting CMV infection, and treatment with antiviral agents was initiated, causing platelet count to increase as viral load decreased.

  14. Postinfluenza Vaccination Idiopathic Thrombocytopenic Purpura in Three Elderly Patients

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    Joji Nagasaki

    2016-01-01

    Full Text Available The etiologies of secondary idiopathic thrombocytopenic purpura (ITP include infection, autoimmune disease, and immunodeficiency. We report the cases of three elderly patients who developed ITP after receiving influenza vaccinations. The platelet count of an 81-year-old woman fell to 27,000/μL after she received an influenza vaccination. A 75-year-old woman developed thrombocytopenia (5,000 platelets/μL after receiving an influenza vaccination. An 87-year-old woman whose laboratory test values included a platelet count of 2,000/μL experienced genital bleeding after receiving an influenza vaccination. After Helicobacter pylori (HP eradication or corticosteroid treatment, all of the patients’ platelet counts increased. Influenza vaccination is an underlying etiology of ITP in elderly patients. HP eradication or corticosteroid treatment is effective for these patients. Clinicians should be aware of the association between ITP and influenza vaccinations.

  15. Initial management of adults with idiopathic (immune) thrombocytopenic purpura.

    Science.gov (United States)

    George, J N

    2002-03-01

    Since idiopathic (immune) thrombocytopenic purpura (ITP) in adults is usually a chronic condition with few spontaneous remissions, the goal of treatment is not cure, but to maintain a hemostatically safe platelet level. The indication for treatment should be based not merely on platelet counts, but also clinical indices of bleeding. Although most patients show good initial response to prednisone, the side effects of steroids limit this treatment. Currently, long-term management usually involves splenectomy. Since splenectomy has surgical risks and may also predispose the patient to sepsis, a clinical trial using anti-D (WinRho-SDR) has been performed to determine whether this treatment can safely delay or avoid the need for surgery. The use of WinRho may also reveal the occurrence of spontaneous remissions, a previously unrecognized subgroup of adults with chronic ITP. PMID:11913992

  16. Two cases of thrombocytopenic purpura at onset of Zika virus infection.

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    Chraïbi, Samy; Najioullah, Fatiha; Bourdin, Carole; Pegliasco, Jean; Deligny, Christophe; Résière, Dabor; Meniane, Jean-Côme

    2016-10-01

    We report here two cases of thrombocytopenic purpura at onset of Zika virus infection. A 26-year-old woman and a 21-year-old man had thrombocytopenia above 5×10(9) platelets/L. Hemorrhagic symptoms were mucosal and subcutaneous bleeding and gross hematuria and they reported episode of conjunctivitis. In both cases blood and bone marrow analysis suggested thrombocytopenic purpura, blood PCR tests for Dengue (DENV), Chikungunya (CHIKV) and Zika virus (ZIKV) were negative. In both cases urinary PCR for ZIKV was positive, Prednisolone yielded early remission. Only three similar cases have been reported so far. In the Caribbean, DENV is also epidemic and responsible for severe thrombocytopenia. Coinfections can occur. Our report underlines the need to include a ZIKV assay in the diagnostic work-up of thrombocytopenic purpura in epidemic areas.

  17. Pulmonary Hyalinizing Granuloma Associated with Idiopathic Thrombocytopenic Purpura

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    Christopher Coleman

    2014-01-01

    Full Text Available Pulmonary hyalinizing granuloma (PHG is a rare, benign lung disease of unknown etiology. It manifests as discrete, rounded nodules within the lung parenchyma. A 39-year-old woman presented for investigation after pulmonary nodules were found incidentally. Chest computed tomography showed multiple, discrete, non-enhancing pulmonary nodules bilaterally. Positron emission tomography (PET was negative. Biopsy demonstrated a non-specific lymphoplasmacytic infiltrate. Open resection yielded two nodules consistent with hyalinizing granulomas. The differential for multiple pulmonary nodules is broad. PET scan can help rule out metastatic disease, although some cancers are not hypermetabolic on PET. Furthermore, some non-malignant conditions, including hyalinizing granuloma, can show increased activity on PET. PHG should be included in the differential of multiple pulmonary nodules, especially if nodule stability can be demonstrated and/or needle biopsies are non-diagnostic. Associated immune-mediated conditions, such as idiopathic thrombocytopenic purpura (ITP in our patient, may also favor HG. In this case report we find an association between PHG and ITP.

  18. Current insights into thrombotic microangiopathies: Thrombotic thrombocytopenic purpura and pregnancy.

    Science.gov (United States)

    von Auer, Charis; von Krogh, Anne-Sophie; Kremer Hovinga, Johanna A; Lämmle, Bernhard

    2015-02-01

    The complex relation between thrombotic thrombocytopenic purpura (TTP) and pregnancy is concisely reviewed. Pregnancy is a very strong trigger for acute disease manifestation in patients with hereditary TTP caused by double heterozygous or homozygous mutations of ADAMTS13 (ADisintegrin And Metalloprotease with ThromboSpondin type 1 domains, no. 13). In several affected women disease onset during their first pregnancy leads to the diagnosis of hereditary TTP. Without plasma treatment mother and especially fetus are at high risk of dying. The relapse risk during a next pregnancy is almost 100% but regular plasma transfusion starting in early pregnancy will prevent acute TTP flare-up and may result in successful pregnancy outcome. Pregnancy may also constitute a mild risk factor for the onset of acute acquired TTP caused by autoantibody-mediated severe ADAMTS13 deficiency. Women having survived acute acquired TTP may not be at very high risk of TTP relapse during an ensuing next pregnancy but seem to have an elevated risk of preeclampsia. Monitoring of ADAMTS13 activity and inhibitor titre during pregnancy may help to guide management and to avoid disease recurrence. Finally, TTP needs to be distinguished from the much more frequent hypertensive pregnancy complications, preeclampsia and especially HELLP (Hemolysis, Elevated Liver Enzymes, Low Platelet count) syndrome. PMID:25903530

  19. Treatment options for chronic idiopathic (immune) thrombocytopenic purpura.

    Science.gov (United States)

    George, J N

    2000-01-01

    The goal of treatment for idiopathic (immune) thrombocytopenic purpura (ITP) is to prevent serious bleeding. Traditionally, corticosteroids have been used as first-line therapy followed by splenectomy. Experience with splenectomy over 60 years shows that approximately two thirds of patients achieve normal platelet counts during the initial observation, but that thrombocytopenia often recurs with longer follow-up. We know that long-term use of corticosteroids can lead to significant morbidities; there is no consensus regarding the appropriate timing or indications for splenectomy. To address the Issue of appropriate use of splenectomy, we designed a multicenter clinical trial that will randomize patients to either standard care, involving prednisone followed by splenectomy, or to a novel regimen of limited prednisone treatment followed by WinRho SDF (Nabi, Boca Raton, FL) (anti-D) therapy to maintain the platelet count in a safe range for 1 year. Anti-D can be administered easily in an outpatient setting with few side effects and can provide predictable, transient increases in platelet count. The hypothesis is that prolonged maintenance therapy with a nontoxic regimen may increase the percentage of patients who will experience a spontaneous remission from thrombocytopenia, thereby avoiding an invasive and permanent surgical procedure, splenectomy, and its potentially life-threatening sequelae. PMID:10676922

  20. Pulmonary hyalinizing granuloma associated with idiopathic thrombocytopenic purpura.

    Science.gov (United States)

    Coleman, Christopher; Nassar, Aziza; McComb, Barbara

    2014-01-01

    Pulmonary hyalinizing granuloma (PHG) is a rare, benign lung disease of unknown etiology. It manifests as discrete, rounded nodules within the lung parenchyma. A 39-year-old woman presented for investigation after pulmonary nodules were found incidentally. Chest computed tomography showed multiple, discrete, non-enhancing pulmonary nodules bilaterally. Positron emission tomography (PET) was negative. Biopsy demonstrated a non-specific lymphoplasmacytic infiltrate. Open resection yielded two nodules consistent with hyalinizing granulomas. The differential for multiple pulmonary nodules is broad. PET scan can help rule out metastatic disease, although some cancers are not hypermetabolic on PET. Furthermore, some non-malignant conditions, including hyalinizing granuloma, can show increased activity on PET. PHG should be included in the differential of multiple pulmonary nodules, especially if nodule stability can be demonstrated and/or needle biopsies are non-diagnostic. Associated immune-mediated conditions, such as idiopathic thrombocytopenic purpura (ITP) in our patient, may also favor HG. In this case report we find an association between PHG and ITP. PMID:24744965

  1. Pulmonary Hyalinizing Granuloma Associated with Idiopathic Thrombocytopenic Purpura

    Science.gov (United States)

    Coleman, Christopher; Nassar, Aziza; McComb, Barbara

    2014-01-01

    Pulmonary hyalinizing granuloma (PHG) is a rare, benign lung disease of unknown etiology. It manifests as discrete, rounded nodules within the lung parenchyma. A 39-year-old woman presented for investigation after pulmonary nodules were found incidentally. Chest computed tomography showed multiple, discrete, non-enhancing pulmonary nodules bilaterally. Positron emission tomography (PET) was negative. Biopsy demonstrated a non-specific lymphoplasmacytic infiltrate. Open resection yielded two nodules consistent with hyalinizing granulomas. The differential for multiple pulmonary nodules is broad. PET scan can help rule out metastatic disease, although some cancers are not hypermetabolic on PET. Furthermore, some non-malignant conditions, including hyalinizing granuloma, can show increased activity on PET. PHG should be included in the differential of multiple pulmonary nodules, especially if nodule stability can be demonstrated and/or needle biopsies are non-diagnostic. Associated immune-mediated conditions, such as idiopathic thrombocytopenic purpura (ITP) in our patient, may also favor HG. In this case report we find an association between PHG and ITP. PMID:24744965

  2. Pregnancy and Birth Outcomes among Women with Idiopathic Thrombocytopenic Purpura

    Directory of Open Access Journals (Sweden)

    Diego F. Wyszynski

    2016-01-01

    Full Text Available Objective. To examine pregnancy and birth outcomes among women with idiopathic thrombocytopenic purpura (ITP or chronic ITP (cITP diagnosed before or during pregnancy. Methods. A linkage of mothers and babies within a large US health insurance database that combines enrollment data, pharmacy claims, and medical claims was carried out to identify pregnancies in women with ITP or cITP. Outcomes included preterm birth, elective and spontaneous loss, and major congenital anomalies. Results. Results suggest that women diagnosed with ITP or cITP prior to their estimated date of conception may be at higher risk for stillbirth, fetal loss, and premature delivery. Among 446 pregnancies in women with ITP, 346 resulted in live births. Women with cITP experienced more adverse outcomes than those with a pregnancy-related diagnosis of ITP. Although 7.8% of all live births had major congenital anomalies, the majority were isolated heart defects. Among deliveries in women with cITP, 15.2% of live births were preterm. Conclusions. The results of this study provide further evidence that cause and duration of maternal ITP are important determinants of the outcomes of pregnancy.

  3. Helicobacter pylori infection in patients with autoimmune thrombocytopenic purpura

    Institute of Scientific and Technical Information of China (English)

    Erdal Kurtoglu; Ertugrul Kayacetin; Aysegul Ugur

    2004-01-01

    AIM: To compare the prevalence of Helicobacter pylori (Hpylori) infection in autoimmune thrombocytopenic purpura (AITP) patients with that of nonthrombocytopenic controls,and to evaluate the efficacy of the treatment in H pylori(+)and H pylori(-) AITP patients.METHODS: The prevalence of gastric H pylori infection in 38 adult AITP patients (29 female and 9 male; median age 27 years; range 18-39 years) who consecutively admitted to our clinic was investagated.RESULTS: H pylori infection was found in 26 of 38 AITP patients (68.5%). H pylori infection was found in 15 of 23control subjects (65.2%). The difference in H pylori infection between the 2 groups was not significant. Thrombocyte count of H pylori-positive AITP patients was significantly lower than that of H pylori-negative AITP patients (P<0.05).Thrombocyte recovery of H pylori-positive group was less than that of H pylori-negative group (P<0.05).CONCLUSION: H pylori infection should be considerecd in the treatment of AITP patients with H pylori infection.

  4. Rapid encephalopathy associated with anti-D immune globulin treatment for idiopathic thrombocytopenic purpura.

    Science.gov (United States)

    Golla, Sunitha; Horkan, Clare; Dogaru, Grigore; Teske, Thomas E; Christopher, Kenneth

    2008-01-01

    Rho (D) immune globulin intravenous (IV RhIG, WinRho SDF) has been shown to be a safe treatment for idiopathic thrombocytopenic purpura. Common side effects of IV RhIG include mild hemolysis, febrile reaction and headache. Significant hemolysis with renal impairment is infrequently noted. A single case of irreversible encephalopathy following IV RhIG has been reported. We report a second case of encephalopathy following an infusion of IV RhIG for treatment of idiopathic thrombocytopenic purpura. PMID:18957844

  5. A Case Associated with Comorbidities Among Cerebral Infarction, Idiopathic Thrombocytopenic Purpura, and Triple X Syndrome

    Directory of Open Access Journals (Sweden)

    Hanjun Kim

    2014-06-01

    Full Text Available A 46-year-old female presented to the emergency room due to the chief complaint of left-sided weakness. By imaging study, she was diagnosed with cerebral infarction. Thrombolytic and antiplatelet agents were not considered due to the “golden hour” for treatment having passed and a low platelet count. The peripheral blood smear, bone marrow biopsy, and aspirate findings were consistent with immune thrombocytopenic purpura. The chromosome analysis revealed the 47,XXX karyotype. To the best of our knowledge, this is the first case report associated with the comorbidities of cerebral infarction, idiopathic thrombocytopenic purpura, and triple X syndrome.

  6. Regulatory T Cells in Patients with Idiopathic Thrombocytopenic Purpura

    Directory of Open Access Journals (Sweden)

    Alev Akyol Erikçi

    2016-05-01

    Full Text Available Objective: Immune thrombocytopenic purpura (ITP is an immunemediated bleeding disorder in which platelets are opsonized by autoantibodies and destroyed by an Fc receptor-mediated phagocytosis by the reticuloendothelial system within the spleen. Autoimmune processes are also considered in the pathogenesis of this disorder. CD4+CD25+FoxP3+ regulatory T (Treg cells and CD8+CD28- Treg cells have roles in autoimmune diseases. We investigated these regulatory cells in ITP patients. Materials and Methods: We included 22 ITP patients and 16 age-matched healthy subjects. CD4+CD25+FoxP3+ Treg cells and CD8+CD28- cells were investigated by three-color flow cytometry. The ratios of these cell populations to total lymphocytes were calculated. Statistical analysis was carried out with the Mann-Whitney U test. Results: CD4+CD25+ Treg cells were 9.69±3.70% and 12.99±5.58% in patients with ITP and controls, respectively. CD4+CD25highFoxP3+ cells were 27.72±19.74% and 27.55±23.98% in ITP patients and controls, respectively. The percentages of both of these cell types were not statistically significant when compared to the control group. Conclusion: We did not find any differences in ratios of CD4+CD25+FoxP3+ Treg cells or CD8+CD28- T cells in lymphocytes between patients and healthy subjects. We conclude that these circulatory cells are not different in ITP, but further studies are needed to explore the putative roles of these regulatory cells.

  7. A Parturient with Chronic Immune Thrombocytopenic Purpura: Anaesthetic Management for Caesarean Section

    Directory of Open Access Journals (Sweden)

    Sushma KS

    2015-08-01

    Full Text Available Immune Thrombocytopenic Purpura (ITP accounts for 4-5% of cases of pregnancy with thrombocytopenia. Their clinical condition may deteriorate during pregnancy subjecting these patients at high risk of bleeding. We report anaesthetic management of a parturient with chronic ITP for caesarean section.

  8. Long-term outcomes of combined chemotherapy in chronic refractory idiopathic thrombocytopenic purpura

    Institute of Scientific and Technical Information of China (English)

    TAO Jie; HUANG Ying; LI Hong-qiang; WANG Ting-ting; WANG Xiao-yan; JI Lin-xiang; YANG Ren-chi

    2007-01-01

    @@ Adult idiopathic thrombocytopenic purpura (ITP) is a chronic acquired organ-specific autoimmune hemorrhagic disease characterized by the production of auto-antibodies against antigens on the membranes of platelet, resulting in enhanced Fc-mediated destruction of the platelets by macrophages in the reticuloendothelial system.

  9. A classification of hemolytic uremic syndrome and thrombotic thrombocytopenic purpura and related disorders.

    NARCIS (Netherlands)

    Besbas, N.; Karpman, D.; Landau, D.; Loirat, C.; Proesmans, W.; Remuzzi, G.; Rizzoni, G.; Taylor, C.M.; Kar, N.C.A.J. van de; Zimmerhackl, L.B.

    2006-01-01

    The diagnostic terms hemolytic uremic syndrome (HUS) and thrombotic thrombocytopenic purpura (TTP) are based on historical and overlapping clinical descriptions. Advances in understanding some of the causes of the syndrome now permit many patients to be classified according to etiology. The increase

  10. Ultrastructural study shows morphologic features of apoptosis and para-apoptosis in megakaryocytes from patients with idiopathic thrombocytopenic purpura

    NARCIS (Netherlands)

    Houwerzijl, EJ; Blom, NR; van der Want, JJL; Esselink, MT; Koornstra, JJ; Smit, JW; Louwes, H; Vellenga, E; de Wolf, JTM

    2004-01-01

    To investigate whether altered megakaryocyte morphology contributes to reduced platelet production in idiopathic thrombocytopenic purpura (ITP), ultrastructural analysis of megakaryocytes was performed in 11 ITP patients. Ultrastructural abnormalities compatible with (para-)apoptosis were present in

  11. Splenectomy in children with idiopathic thrombocytopenic purpura : A prospective study of 134 children from the Intercontinental Childhood ITP Study Group

    NARCIS (Netherlands)

    Kuehne, Thomas; Blanchette, Victor; Buchanan, George R.; Ramenghi, Ugo; Donato, Hugo; Tamminga, Rienk Y. J.; Rischewski, Johannes; Berchtold, Willi; Imbach, Paul

    2007-01-01

    Background. Splenectomy is an effective procedure for children and adults with severe or refractory idiopathic thrombocytopenic purpura (ITP). Data regarding pediatric patients are limited. Procedure. Sixty-eight Intercontinental Childhood ITP Study Group (ICIS) investigators from 57 institutions in

  12. CLINICAL FEATURES AND TREATMENT RESPONSE OF IMMUNE THROMBOCYTOPENIC PURPURA IN INFANTS

    Directory of Open Access Journals (Sweden)

    A. Ramyar N. Kalantari

    2007-09-01

    Full Text Available To determine the clinical features and treatment outcomes of infant with immune thrombo-cytopenic purpura (ITP. Retrospective analysis of 96 infant ITP patients treated from 1995 to 2005. The data abstracted comprised age, gender, clinical features, and treatment outcomes. The 56 male and 40 female infants had a median age of 3 months. Eighty presented with purpura, sixteen with active mucosal bleeding. The median platelet count was 13000 /l. Seventy-seven infants received intravenous immunoglobulin (IVIG, eighteen steroids and one patient was observed. Ninty-sex (96% responds to a single course of treatment. Infant with ITP respond favorably to treatment.

  13. Cardiac surgery in a patient with immunological thrombocytopenic purpura: Complications and precautions

    Directory of Open Access Journals (Sweden)

    Vivek Chowdhry

    2013-01-01

    Full Text Available Immune thrombocytopenic purpura (ITP patients are at high-risk for bleeding complications during and after cardiac surgeries involving cardiopulmonary bypass. We report a patient with ITP with severe coronary artery disease and mitral valve regurgitation who underwent uncomplicated coronary artery bypass grafting and mitral valve replacement. Three weeks later, the patient was readmitted in a very low general condition with signs of pericardial tamponade. We describe our experience of managing the case.

  14. Rapid irreversible encephalopathy associated with anti-D immune globulin treatment for idiopathic thrombocytopenic purpura.

    Science.gov (United States)

    Christopher, Kenneth; Horkan, Clare; Barb, Ilie T; Arbelaez, Christian; Hodgdon, Travis A; Yodice, Paul C

    2004-11-01

    Intravenous Rho (D) immune globulin (IV RhIG, WinRho SDF) has been shown to be a safe treatment for idiopathic thrombocytopenic purpura (ITP). Common side effects of IV RhIG include mild hemolysis, febrile reaction, and headache. Significant hemolysis with renal impairment following IV RhIG has been reported. We report a case of irreversible encephalopathy 48 hr following an infusion of IV RhIG for treatment of ITP. PMID:15495245

  15. A case of refractory thrombotic thrombocytopenic purpura treated with plasmapheresis and rituximab.

    Science.gov (United States)

    Kirui, Nicholas; Sokwala, Ahmed

    2016-07-01

     Thrombotic thrombocytopenic purpura (TTP) is a rare, life-threatening disorder with no prevalence or incidence studies in sub-Saharan Africa. Acquired TTP has several causes, all of which lead to decreased activity of von Willebrand factor cleaving protease (ADAMTS13) due to autoantibodies that are directed towards ADAMTS13. We report a case of a 46-year-old man who presented with most of the classic clinical manifestations of TTP. PMID:27384362

  16. Peripheral digit ischemic syndrome can be a manifestation of postoperative thrombotic thrombocytopenic purpura

    OpenAIRE

    Chang, J. C.; Ikhlaque, N

    2004-01-01

    In addition to common dysfunction of the brain and kidney, thrombotic thrombocytopenic purpura (TTP) may present with atypical clinical features due to the involvement of other organs such as the lung, pancreas, heart, eye, and skin. We have also observed the unusual presentation of peripheral digit ischemic syndrome (PDIS) in some patients with postoperative TTP To clarify this relationship between TTP and PDIS, the hematologic data from the medical records of patients with known diagnoses o...

  17. ADAMTS13 Deficiency and Thrombotic Thrombocytopenic Purpura Associated with Trimethoprim-Sulfamethoxazole

    OpenAIRE

    Bapani, Sowjanya; Epperla, Narendranath; Kasirye, Yusuf; Mercier, Richard; Garcia-Montilla, Romel

    2012-01-01

    Thrombotic thrombocytopenic purpura (TTP) is a hematological disease characterized by microangiopathic hemolytic anemia and thrombocytopenia. Although the link between ADAMTS13 deficiency and idiopathic TTP has been well-established, the role of trimethoprim-sulfamethoxazole (TMP-SMX) in the pathogenesis of TTP is not yet well elucidated. To the best of our knowledge, there have been only two previous reports linking this medication with the development of TTP. We present the case of a health...

  18. Response to rituximab in a refractory case of thrombotic thrombocytopenic purpura associated with systemic lupus erythematosus

    OpenAIRE

    Niaz Faraz; Aleem Aamer

    2010-01-01

    Thrombotic thrombocytopenic purpura (TTP) is a serious disorder with a significant morbidity and mortality. Majority of cases of TTP are idiopathic, but some cases may be secon-dary to connective tissue diseases. TTP has been rarely associated with systemic lupus erythe-matosus (SLE) and may be refractory to treatment with plasma exchange, requiring immuno-suppressive therapy. We describe a patient with TTP and SLE who was refractory to plasma exchange and corticosteroids but responded to ant...

  19. DIFFERENT DISPARITIES OF GENDER AND RACE AMONG THE THROMBOTIC THROMBOCYTOPENIC PURPURA AND HEMOLYTIC-UREMIC SYNDROMES

    OpenAIRE

    Terrell, Deirdra R.; Vesely, Sara K.; Kremer Hovinga, Johanna A.; Lämmle, Bernhard; George, James N.

    2010-01-01

    Thrombotic thrombocytopenic purpura (TTP) and hemolytic-uremic syndrome (HUS) represent multiple disorders with diverse etiologies. We compared the gender and race of 335 patients enrolled in the Oklahoma TTP-HUS Registry across 21 years for their first episode of TTP or HUS to appropriate control groups. The relative frequency of women and white race among patients with TTP-HUS associated with a bloody diarrhea prodrome and the relative frequency of women with quinine-associated TTP-HUS were...

  20. Thrombotic Thrombocytopenic Purpura Associated with Mixed Connective Tissue Disease: A Case Report

    OpenAIRE

    Aline Maria Yamaguti Rios Paes da Silva; Fernanda Alves Barbosa; Philipe Vianna de Barros; João Tadeu Damian Souto Filho; Gustavo Fernandes Ribas

    2011-01-01

    Thrombotic thrombocytopenic purpura (TTP) is a multisystemic disorder characterized by microangiopathic hemolytic anemia and thrombocytopenia, which may be accompanied by fever, renal, or neurologic abnormalities. Cases are divided into acute idiopathic TTP and secondary TTP. Autoimmune diseases, especially systemic lupus erythematosus, in association with TTP have been described so far in many patients. In contrast, TTP occurring in a patient with mixed connected tissue disease (MCTD) is ext...

  1. Thrombotic Thrombocytopenic Purpura in Black People: Impact of Ethnicity on Survival and Genetic Risk Factors

    OpenAIRE

    Martino, Suella; Jamme, Mathieu; Deligny, Christophe; Busson, Marc; Loiseau, Pascale; Azoulay, Elie; Galicier, Lionel; Pène, Frédéric; Provôt, François; Dossier, Antoine; Saheb, Samir; Veyradier, Agnès; Coppo, Paul

    2016-01-01

    French Reference Center for Thrombotic Microangiopathies International audience Black people are at increased risk of thrombotic thrombocytopenic purpura (TTP). Whether clinical presentation of TTP in Black patients has specific features is unknown. We assessed here differences in TTP presentation and outcome between Black and White patients. Clinical presentation was comparable between both ethnic groups. However, prognosis differed with a lower death rate in Black patients than in Whi...

  2. Atypical presentations of thrombotic thrombocytopenic purpura: a diagnostic role for ADAMTS13.

    Science.gov (United States)

    Kalish, Yosef; Rottenstreich, Amihai; Rund, Deborah; Hochberg-Klein, Sarit

    2016-08-01

    Thrombotic thrombocytopenic purpura (TTP) is an acute, life threatening disease. Only a minority of patients expresses the complete clinical presentation and unusual manifestations can occur. Demonstration of low activity levels of ADAMTS13 (importance of having a high clinical suspicion of TTP in cases of thrombosis even without hematological abnormalities in patients with previous attacks of TTP. In this clinical scenario, measurement of ADAMTS13 activity is important for diagnosis and early administration of treatment. PMID:26867546

  3. Thrombotic thrombocytopenic purpura-like syndrome associated with systemic lupus erythematosus--combined treatment with plasmapheresis and fresh frozen plasma infusion.

    OpenAIRE

    Lim, G. T.; Kim, S. S.; Park, S. H.; Choo, W. O.; Kang, D. H.; Park, I. S.; Chang, Y S; Y. S. YOON; Bang, B. K.

    1992-01-01

    We report on a patient with systemic lupus erythematosus, who, during the course of the illness, developed thrombotic thrombocytopenic purpura. In this case, the coexistence of these two conditions was confirmed by laboratory and pathologic findings. The infusion of fresh frozen plasma with plasmapheresis reversed the course of thrombotic thrombocytopenic purpura.

  4. Squamous cell carcinoma larynx presenting as idiopathic thrombocytopenic purpura

    Directory of Open Access Journals (Sweden)

    Bekur R

    2015-01-01

    Full Text Available Association of immune thrombocytpenic purpura with solid malignancy as paraneoplastic manifestation has been reported earlier mainly with lymphoma and breast cancer. We report the case of a patient with squamous cell carcinoma of the larynx presenting with idiopathic thombocytopenic purpura (ITP. A 67-year-old lady presented with multiple ecchymotic patches and petechiae all over the body and bleeding from oral cavity was found to have severe thrombocytopenia diagnosed as ITP with bone marrow evidence of peripheral destruction without infiltration of bone marrow. Five months later she was diagnosed to have squamous cell carcinoma of larynx. Platelet count improved after splenectomy.

  5. Thrombotic thrombocytopenic purpura and myoglobinuric acute renal failure following radiation therapy in a patient with polymyositis and cervical cancer

    International Nuclear Information System (INIS)

    A 73-year-old woman was admitted to receive radiation treatment for uterine cervical cancer, however a complex series of events ensued, leading to death. She developed an acute exacerbation of polymyositis complicated by thrombocytopenic purpura, rhabdomyolysis and acute renal failure. Radiation therapy may have produced an immune disturbance leading to the acute exacerbation of polymyositis. Auto-immune-mediated endothelial damage might have triggered a series of events leading to thrombotic thrombocytopenic purpura. Rhabdomyolysis seemed to be the main cause of acute renal failure. (author)

  6. Dameshek W, Miller EB. The megakaryocytes in idiopathic thrombocytopenic purpura, a form of hypersplenism. 1946.

    Science.gov (United States)

    2016-01-01

    This paper, by one of the legends of hematology, William Dameshek, and his colleague Edward Miller, is from the inaugural issue of Blood. By studying bone marrow specimens from controls, patients with acute or chronic immune thrombocytopenia, or patients with other thrombocytopenic disorders, the authors concluded that, in idiopathic thrombocytopenic purpura (ITP), production of platelets from megakaryocytes is defective, even while marrow megakaryocytes are greatly increased in number. This defect resolved after splenectomy. The authors appropriately credit E. Frank with having proposed defective platelet production from megakaryocytes in ITP in 1915. The idea that platelet production was defective in ITP was superseded or ignored for decades, but it has now been validated by the therapeutic effectiveness of the thrombopoietin mimetics in ITP.

  7. Pseudo-thrombotic thrombocytopenic purpura: A rare presentation of pernicious anemia

    Directory of Open Access Journals (Sweden)

    Ashvin K Tadakamalla

    2011-01-01

    Full Text Available Context: Schistocytes are fragmented red blood cells due to the flow of blood through damaged capillaries and indicate endothelial injury. They are typical of microangiopathic hemolytic anemia seen in life threatening conditions like disseminated intravascular coagulation or thrombotic thrombocytopenic purpura/hemolytic uremic syndrome .We report a rare sub-acute presentation of pernicious anemia with hemolysis, thrombocytopenia and numerous schistocytes that was initially diagnosed as a more serious thrombotic thrombocytopenic purpura. Case Report : A 31-year-old Caucasian woman presented with fatigue and paresthesia of both feet for 1 week. Past medical history included hypertension and gastro-esophageal reflux disease. Examination revealed scleral icterus and pallor. Examination of the abdomen did not show hepatosplenomegaly. Initial laboratory tests showed severe anemia, and low platelets. Indirect bilirubin and serum Lactate De Hydrogenase were elevated. Prothrombin time, partial thromboplastin time, serum fibrinogen, and serum fibrin degradation product levels were normal. Peripheral smear revealed numerous schistocytes, anisocytosis and macro-ovalocytes. Thrombotic thrombocytopenic purpura (TTP was suspected due to the constellation of sub-acute onset of fatigue and paresthesia along with thrombocytopenia, schistocytes and an elevated LDH. Plasmapheresis was initiated for possible TTP. However, platelet count worsened despite plasmapheresis for 4 days. On re-evaluation, vitamin B 12 was found to be low. Treatment with intra-muscular vitamin B 12 led to symptomatic and hematologic improvement. Pernicious anemia was confirmed by the presence of anti-intrinsic factor antibodies, elevated serum gastrin level and atrophic gastritis. Conclusion : Clinicians must be aware of unusual clinical presentation of vitamin B 12 deficiency with schistocytes as the management is simple and effective.

  8. Systemic lupus erythematosus and thrombotic thrombocytopenic purpura:report of three cases

    Institute of Scientific and Technical Information of China (English)

    张文; 尤欣; 董怡

    2004-01-01

    @@ Systemic lupus erythematosus (SLE) is a multisystemic disease characterized by an autoimmune reaction.Thrombotic thrombocytopenic purpura (TTP) is a rare but severe syndrome with the manifestations of fever,thrombocytopenia, microangiopathic hemolysis,neurological symptoms, and renal involvement. The initial prognosis was reported to be dismal, 90% of patients dying within 3 months of onset. However, with the possibility of combined treatments, the survival rate has considerably improved. Data suggest a possible role of immune mechanisms in the development of TTP. 1,2We here report three cases of SLE complicated with TTP,and review the diagnosis, treatment, and outcome of SLE with TTP.

  9. Does Helicobacter pylori play a role in the pathogenesis of childhood chronic idiopathic thrombocytopenic purpura?

    OpenAIRE

    Maryam Maghbool; Masood Maghbool; Mehdi Shahriari; Mehran Karimi

    2009-01-01

    Idiopathic thrombocytopenic purpura (ITP) is an acute self-limited bleeding disorder that can progress to chronic form in 10-15% of the cases. Helicobacter pylori (H. pylori) infection is a possible cause of chronic ITP. We studied 30 children with resistant chronic ITP for H. pylori infection based on the detection of H. pylori fecal antigen. This retrospective study was based on data obtained from medical records of 30 children aged between five and 17 years (median age at ITP diagnosis was...

  10. An Ischemic Stroke Related to Eltrombopag Use in a Patient with Idiopathic Thrombocytopenic Purpura

    Directory of Open Access Journals (Sweden)

    Demet Arslan

    2016-03-01

    Full Text Available Thrombopoetin receptor agonists like eltrombopag, used for Idiopathic Thrombocytopenic Purpura treatment rarely cause to the ischemic cerebrovascular disease. In this re­port, a patient, have been followed by ITP diagnosis for 5 years, admitted to the emergency service for right hemi­paresis and aphasia. It was diagnosed as ischemic cere­brovascular disease. Etiology of patient’s cerebrovascu­lar event was associated with eltrombopag. Because of this rare seen situation, it was argued by in the light of the literature.

  11. Intravenous anti-D immunoglobulin in the treatment of resistant immune thrombocytopenic purpura in pregnancy.

    Science.gov (United States)

    Sieunarine, K; Shapiro, S; Al Obaidi, M J; Girling, J

    2007-04-01

    A 35-week pregnant 38-year-old woman presented with isolated thrombocytopenia (platelet count 4 x 10(9)/l). Investigations confirmed immune thrombocytopenic purpura, and she received treatment with prednisolone and intravenous immunoglobulins with no increment in the platelet count. At 37 and 38 weeks of the pregnancy, she received two doses of WinRho (anti-D immunoglobulin) at 50 microg/kg. Five days later, with a platelet count of 46 x 10(9)/l, she had an uncomplicated normal vaginal delivery. WinRho is a useful adjunct to other first-line treatment modalities for immune thrombocytopenia in pregnancy. PMID:17309547

  12. Congenital thrombotic thrombocytopenic purpura caused by new compound heterozygous mutations of the ADAMTS13 gene

    DEFF Research Database (Denmark)

    Rank, Cecilie Utke; Kremer Hovinga, Johanna; Taleghani, Magnus Mansouri;

    2014-01-01

    , causing intravascular platelet clumping and thrombotic microangiopathy. Our patient, a 26-year-old man, had attacks of thrombotic thrombocytopenic purpura (TTP) with thrombocytopenia and a urine dipstick positive for hemoglobin (4+), often as the only sign of hemolytic activity. He had ADAMTS13 activity...... of A) leading to p.R1123H. This case report confirms the importance of the analysis of the ADAMTS13 activity and its inhibitor in patients who have episodes of TTP, with a very low platelet count and sometimes without the classic biochemical signs of hemolysis....

  13. Exudative Retinal Detachment Treatment in a Patient with Thrombotic Thrombocytopenic Purpura

    Directory of Open Access Journals (Sweden)

    Magali Sampo

    2016-02-01

    Full Text Available Purpose: We report a case of unilateral exudative retinal detachment in a patient with thrombotic thrombocytopenic purpura (TTP, without associated hypertension, successfully treated with plasmapheresis. Case Report: A 46-year-old woman with a medical history of TTP presented with unilateral exudative retinal detachment. Biological and radiological assessment eliminated other causes of exudative retinal detachment, including hypertension. Plasma exchange was performed, followed by a rapid improvement in visual acuity and total disappearance of serous detachment. Conclusion: Exudative unilateral retinal detachment is a rare complication of TTP and can be successfully treated by plasma exchange.

  14. Difficult Management of Coronary Artery Disease in a Patient with Thrombotic Thrombocytopenic Purpura

    Directory of Open Access Journals (Sweden)

    Fatemeh Jorfi

    2015-10-01

    Full Text Available Thrombotic thrombocytopenic purpura (TTP is a rare syndrome affecting multiple organs. There is no sufficient evidence regarding the clinical cardiac manifestations of TTP. Nonetheless, pathologic cardiac involvement is quite frequent in acute TTP, which is predominantly manifested as myocardial necrosis due to coronary arteriolar microthrombosis. The present case report describes a 43-year-old man with long-standing remitted TTP, who suffered from a sequence of refractory thrombotic epicardial coronary events. Aggressive medical and interventional therapies, including long-term dual antiplatelets and coronary angioplasty, were finally successful in remitting the thrombotic events. During his two-year follow up, he has been asymptomatic.

  15. ADAMTS13-binding IgG are present in patients with thrombotic thrombocytopenic purpura

    OpenAIRE

    Tsai, Han-Mou; Raoufi, Mojgan; Zhou, Wenhua; Guinto, Enriqueta; Grafos, Nickolas; Ranzurmal, Safi; Greenfield, Robert S.; Rand, Jacob H.

    2006-01-01

    Functional assays are commonly used to measure the antibodies of ADAMTS13 found in patients of thrombotic thrombocytopenic purpura (TTP). In this study we used an enzyme-linked immunoassay to analyze the ADAMTS13-binding IgG levels in six groups of individuals: normal, random hospitalized patients, acute TTP,TTP after receiving plasma therapy, TTP in remission, and other types of thrombotic microangiopathy (TMA). The results showed thatADAMTS13-binding IgG levels were elevated in 100% of the ...

  16. ADAMTS13 phenotype in plasma from normal individuals and patients with thrombotic thrombocytopenic purpura

    OpenAIRE

    Manea, Minola; Kristoffersson, AnnCharlotte; Tsai, Han-Mou; Zhou, Wenhua; Winqvist, Ingemar; Oldaeus, Göran; Billström, Rolf; Björk, Peter; Holmberg, Lars; Karpman, Diana

    2006-01-01

    The activity of ADAMTS13, the von Willebrand factor cleaving protease, is deficient in patients with thrombotic thrombocytopenic purpura (TTP). In the present study, the phenotype of ADAMTS13 in TTP and in normal plasma was demonstrated by immunoblotting. Normal plasma (n = 20) revealed a single band at 190 kD under reducing conditions using a polyclonal antibody, and a single band at 150 kD under non-reducing conditions using a monoclonal antibody. ADAMTS13 was not detected in the plasma fro...

  17. Successful rituximab treatment in an elderly patient with recurrent thrombotic thrombocytopenic purpura.

    Science.gov (United States)

    Matsubara, Etsuko; Yamanouchi, Jun; Hato, Takaaki; Takeuchi, Kazuto; Niiya, Toshiyuki; Yasukawa, Masaki

    2016-07-01

    An 81-year-old man presenting with fever, neurological symptoms, thrombocytopenia, and hemolytic anemia was diagnosed with acquired idiopathic thrombotic thrombocytopenic purpura (TTP). His disintegrin-like and metalloproteinase with thrombospondin type 1 motifs 13 (ADAMTS13) activity was TTP recurrence based on ADAMTS13 activity TTP in Japan, we report the efficacy and safety of rituximab in an elderly patient with recurrent TTP. We suggest that rituximab therapy should be started as soon as possible for recurrent TTP in patients with high titers of ADAMTS13 inhibitor. PMID:27498731

  18. Pulmonary hyalinizing granuloma. Bilateral pulmonary nodules associated with chronic idiopathic thrombocytopenic purpura.

    Science.gov (United States)

    Satti, Mohamed B; Batouk, Abdelnasir A; Abdelaziz, Muntasir M; Ahmad, Mohamed F; Abdelaal, Mohamed A

    2005-09-01

    We report a case of a 30-year-old female who had been treated periodically with steroids for idiopathic thrombocytopenic purpura ITP over the last 10 years. Recently, during the course of investigation, she was found to have incidental asymptomatic multiple pulmonary nodules on chest CT. Following a needle biopsy to exclude malignancy, 2 nodules were excised and were histologically confirmed as pulmonary hyalinizing granuloma PHG. The remaining 2 nodules regressed on increasing her dose of steroids. The case is discussed with emphasis on the histological and radiological differential diagnosis, in addition to including ITP among the spectrum of immunologic conditions associated with PHG. PMID:16155671

  19. Crohn’s Disease and Idiopathic Thrombocytopenic Purpura in a Patient with Ectodermal Dysplasia and Immunodeficiency

    Directory of Open Access Journals (Sweden)

    Farzaneh Motamed

    2006-09-01

    Full Text Available In this case report we will describe a rare association between anhyrotic ectodermal dysplasia (AED and immunodeficiency and autoimmunity [in our case: Idiopathic Thrombocytopenic Purpura (ITP and Crohn disease]. AED is a rare congenital disorder characterized by sparse hair, abnormal teeth and anhidrosis due to lack of eccrine glands. The survey of 87 cases with (AED revealed only one Irritable Bowel Disease (IBD.  AED has only two relevancies with immunodeficiency: (EDA-ID: Ectodermal Dysplasia Anhyrotic with Immunodeficiency and APE-CED (Autoimmune polyendocrinopathy, Candidiasis and Ectodermal Dysplasia that in our case EDA-ID is strongly suspected.

  20. Immune thrombocytopenic purpura in ulcerative colitis: a case report and systematic review

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    Subhash Chandra

    2014-04-01

    Full Text Available Over 100 extraintestinal manifestations are reported in ulcerative colitis (UC. A commonly reported hematological manifestation is autoimmune hemolytic anemia. On rare occasions, immune thrombocytopenic purpura (ITP has been reported with UC. The presence of thrombocytopenia can complicate the clinical scenario as the number of bloody bowel movements is an important indicator of disease activity in UC. A proposed theory for this association is antigenic mimicry between a platelet surface antigen and bacterial glycoprotein. We are reporting a case of UC and associated ITP managed successfully with anti-TNF therapy. We also performed a systemic review of case reports and a case series reporting this association.

  1. Quantifying the reduction in immunoglobulin use over time in patients with chronic immune thrombocytopenic purpura receiving romiplostim (AMG 531)

    NARCIS (Netherlands)

    Pullarkat, Vinod A.; Gernsheirner, Terry B.; Wasser, Jeffrey S.; Newland, Adrian; Guthrie, Troy H.; de Wolf, Joost Th. M.; Stewart, Ron; Berger, Dietmar

    2009-01-01

    Patients with Immune thrombocytopenic purpura (ITP) often require immunoglobulin (Ig) therapy with intravenous 19 (IVIG) or anti-D to prevent or treat the serious bleeding events. Because the thrombopoietin (TPO) mimetic romiplostim (AMG 531; Nplate) elevates platelet counts in patients with chronic

  2. Thrombotic thrombocytopenic purpura after allogeneic stem cell transplantation : a survey of the European Group for Blood and Marrow Transplantation (EBMT)

    NARCIS (Netherlands)

    Ruutu, T; Hermans, J; Niederwieser, D; Gratwohl, A; Kiehl, M; Volin, L; Bertz, H; Ljungman, P; Spence, D; Verdonck, LF; Prentice, HG; Bosi, A; du Toit, CE; Brinch, L; Apperley, JF

    2002-01-01

    A survey was carried out among the European Group for Blood and Marrow Transplantation (EBMT) centres to determine the incidence, risk factors, treatment and outcome of thrombotic thrombocytopenic purpura (TTP) following allogeneic haematopoietic stem cell transplantation. TTP was defined as the sim

  3. A review of immune thrombocytopenic purpura: focus on the novel thrombopoietin agonists

    Directory of Open Access Journals (Sweden)

    Meaghan Khan

    2010-03-01

    Full Text Available Meaghan Khan, Joseph MikhaelDivision of Hematology – Oncology, Scottsdale, AZ, USAAbstract: Immune thrombocytopenic purpura (ITP is an autoimmune disorder that is characterized by antibody-mediated platelet destruction and decreased platelet production. ITP and its treatments have been recognized to cause diminished quality of life in those afflicted with this illness on levels comparable to other chronic diseases. The disease can be self-limiting, but in adults it often is a chronic process requiring medical intervention to maintain appropriate platelet counts and to reduce bleeding events. Many patients go on to develop disease that is refractory to current interventions. Historically, the aim of treatment has been focused on reducing the amount of antibody-mediated destruction but newer therapies have centered on the decreased platelet production. Two new medications that target production of platelets have recently been USA, Food and Drug Administration (FDA approved for the treatment of chronic relapsing ITP. Here, we provide an overview of ITP and a comprehensive review of the newest therapies aimed at the stimulation of platelet production.Keywords: immune thrombocytopenic purpura, therapy, thrombopoietin, romiplostim, AMG 531, eltrombopag

  4. [Wilson's disease associated with olfactory paranoid syndrome and idiopathic thrombocytopenic purpura].

    Science.gov (United States)

    Sagawa, Morihiko; Takao, Masaki; Nogawa, Shigeru; Mizuno, Masafumi; Murata, Mitsuru; Amano, Takahiro; Koto, Atsuo

    2003-10-01

    In this study we report an individual of Wilson's disease associated with olfactory paranoid syndrome and idiopathic thrombocytopenic purpura. The initial symptom of this female patient was olfactory paranoia at age 17. Although that psychiatric symptom was well controlled under pharmacological treatment for two years, she developed olfactory paranoia as well as sialorrhea, dysarthria and finger tremor at age 20. A year later rigidity was also present in the extremities. At age 23, idiopathic thrombocytopenic purpura was found based on hematological examinations. Because her extrapyramidal symptoms were progressive, she was referred to our department to evaluate her neurologic condition. She was diagnosed as having Wilson's disease based on (1) the presence of Kayser-Fleischer rings, (2) extrapyramidal signs, and (3) a decreased level of serum copper and ceruloplasmin. T2 and FLAIR images of brain MRI showed hyperintense lesions in the putamen, thalamus and pontine tegmentum. Diffusion-weighted images also showed hyperintense lesions in the thalamus and pontine tegmentum. The biopsy specimen of the liver revealed chronic hepatitis with copper accumulation. Since D-penicillamine treatment was initiated, she has shown no olfactory paranoia and exacerbation of ITP. Her gait disturbance has also improved. Olfactory paranoia and ITP are rare clinical complications of Wilson's disease. Further analysis may warrant consideration of the pathophysiological mechanism of the psychiatric, hematological and neuroradiological condition seen in Wilson's disease.

  5. The spleen and splenectomy in immune (idiopathic) thrombocytopenic purpura.

    Science.gov (United States)

    Sandler, S G

    2000-01-01

    The benefits of surgical splenectomy in patients with immune (Idiopathic) thrombocytopenia purpura (ITP) probably reflect the combined effects of eliminating a source of antiplatelet antibody synthesis as well as the primary site of platelet destruction. The recent availability of intravenous Rho(D) Immune globulin (WinRho SDF; Nabi, Boca Raton, FL) presents an opportunity to extend the duration of nonsurgical (spleen-sparing) management of chronic ITP by inducing reversible Fc blockade. While new methods for laparoscopic splenectomy may offer improved surgical outcomes and reduced costs for ITP patients in the near-term, the long-term consequences of splenectomy remain to be determined. Partial splenectomy has been shown to be effective in the management of anemia in hereditary spherocytosis and elliptocytosis, while preserving vital splenic phagocytic and immune functions. The concept that cell destruction occurs in reticuloendothelial cells has been updated with recognition that the mononuclear phagocyte is neither a reticular nor an endothelial cell. Immune phagocytosis is now understood to be mediated by macrophage IgG Fc and complement receptors. A key factor for devising a strategy for selecting medical or surgical splenectomy, or postponing splenectomy, is an assessment of the relative importance of splenic immune versus phagocytic function in the pathogenesis of ITP. PMID:10676918

  6. Flow cytometric analysis of anti-platelet antibodies in idiopathic thrombocytopenic purpura.

    Science.gov (United States)

    Latorraca, A; Lanza, F; Moretti, S; Ferrari, L; Reverberi, R; Galluccio, L; Castoldi, G

    1994-01-01

    Anti-platelet antibody measurement may be important in defining the pathogenesis of thrombocytopenic states. In this paper we compared three anti-human immunoglobulin reagents by using them to detect anti-platelet antibodies on the platelet surface and in the serum of 14 patients with chronic idiopathic thrombocytopenic purpura (ITP) and 22 thrombocytopenic disorders. Samples were analyzed by both flow cytometry and a fluorescence microscope. In ITP patients, the direct test was positive in 50% of the cases, while the indirect technique proved to be positive in a slightly higher number of those tested (56%). Furthermore, the number of positive cases was similar for the three reagents used in this study, although the mean percentage of positive platelets was higher for the kappa/lambda monoclonal reagent. These data further support the sensitivity and reproducibility of flow cytometry analysis, which was capable of detecting antiplatelet antibodies in all patients with transfused Cooley's disease (regarded as positive control), as well as in a significant number of patients with ITP or related diseases. On the basis of the data presented here, definitive proof regarding the presence of anti-platelet antibodies in patients with thrombocytopenia still has to be found, and further studies are needed in order to ascertain the autoimmune nature of these disorders. PMID:7926978

  7. Multiple domains of ADAMTS13 are targeted by autoantibodies against ADAMTS13 in patients with acquired idiopathic thrombotic thrombocytopenic purpura

    Science.gov (United States)

    Zheng, X. Long; Wu, Haifeng M.; Shang, Dezhi; Falls, Erica; Skipwith, Christopher G.; Cataland, Spero R.; Bennett, Charles L.; Kwaan, Hau C.

    2010-01-01

    Background Type G immunoglobulins against ADAMTS13 are the primary cause of acquired (idiopathic) thrombotic thrombocytopenic purpura. However, the domains of ADAMTS13 which the type G anti-ADAMT13 immunoglobulins target have not been investigated in a large cohort of patients with thrombotic thrombocytopenic purpura. Design and Methods Sixty-seven patients with acquired idiopathic thrombotic thrombocytopenic purpura were prospectively collected from three major U.S. centers. An enzyme-linked immunosorbent assay determined plasma concentrations of anti-ADAMTS13 type G immunoglobulins, whereas immunoprecipitation plus western blotting determined the binding domains of these type G immunoglobulins. Results Plasma anti-ADAMTS13 type G immunoglobulins from 67 patients all bound full-length ADAMTS13 and a variant truncated after the eighth TSP1 repeat (delCUB). Approximately 97% (65/67) of patients harbored type G immunoglobulins targeted against a variant truncated after the spacer domain (MDTCS). However, only 12% of patients’ samples reacted with a variant lacking the Cys-rich and spacer domains (MDT). In addition, approximately 37%, 31%, and 46% of patients’ type G immunoglobulins interacted with the ADAMTS13 fragment containing TSP1 2-8 repeats (T2-8), CUB domains, and TSP1 5-8 repeats plus CUB domains (T5-8CUB), respectively. The presence of type G immunoglobulins targeted against the T2-8 and/or CUB domains was inversely correlated with the patients’ platelet counts on admission. Conclusions This multicenter study further demonstrated that the multiple domains of ADAMTS13, particularly the Cys-rich and spacer domains, are frequently targeted by anti-ADAMTS13 type G immunoglobulins in patients with acquired (idiopathic) thrombotic thrombocytopenic purpura. Our data shed more light on the pathogenesis of acquired thrombotic thrombocytopenic purpura and provide further rationales for adjunctive immunotherapy. PMID:20378566

  8. Splenectomy in patients with idiopathic thrombocytopenic purpura: Analysis of 109 cases

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    Enver Ay

    2012-03-01

    Full Text Available Objectives: Splenectomy is performed in order to provide the treatment in the patients with severe idiopathic thrombocytopenic purpura, refractory to medical treatment. In this study, we aimed to investigate the postoperatif and longterm outcomes in the patients who underwent splenectomy with the diagnosis of idiopathic thrombocytopenic purpura.Materials and Methods: Between 2001-2010 at Dicle University Medical Faculty, General Surgery Department, a retrospective review of the 109 patients who had undergone splenectomy for ITP was reviewed. Age, gender, presence of accessory spleens and location, duration of the operation, number of preoperative platelet tranfusion, number of preoperative and postoperative blood transfusion, length of hospital stay, long-term outcomes, morbidity and mortality were recorded.Results: The mean age was 37.10 ± 16.62 (16-72, and there were 88 (80.7% female and 21 (19.3% male patients. The mean operation time was 44.87 ± 10:32 (30-120 minutes. The average postoperative blood and preoperative platelet transfusion were 1.63 ± 0.85 (0-3 and 2.01 ± 0.71 (1-3 units, respectively. The accessory spleens were encountered in 20 (18.3% patients at the ultrasonographic examination. And also the accessory spleens were encountered in 23 (21.1% patients during operation and confirmed with histopathologic examination. The most common localization of accessory spleens were splenic hilus. The postoperative complications were occurred in 16 patients (14.7% and the most complication was atelectasia. The mean length of hospital stay was 4:56 ± 2:45 (2-12 days. Patients were followed for an average of 28 (9-48 months. At the follow-up period, 1 (0.9 % patient had died.Conclusion: Splenectomy can be performed safely in the treatment of the patients with idiopathic thrombocytopenic purpura unresponsive to medical treatment. Long-term good results can be obtained with splenectomy in these patients. The accessory spleens should not be

  9. Peliosis hepatis presenting with massive hepatomegaly in a patient with idiopathic thrombocytopenic purpura.

    Science.gov (United States)

    Kim, Sun Bean; Kim, Do Kyung; Byun, Sun Jeong; Park, Ji Hye; Choi, Jin Young; Park, Young Nyun; Kim, Do Young

    2015-12-01

    Peliosis hepatis is a rare condition that can cause hepatic hemorrhage, rupture, and ultimately liver failure. Several authors have reported that peliosis hepatis develops in association with chronic wasting disease or prolonged use of anabolic steroids or oral contraceptives. In this report we describe a case in which discontinuation of steroid therapy improved the condition of a patient with peliosis hepatis. Our patient was a 64-year-old woman with a history of long-term steroid treatment for idiopathic thrombocytopenic purpura . Her symptoms included abdominal pain and weight loss; the only finding of a physical examination was hepatomegaly. We performed computed tomography (CT) and magnetic resonance imaging (MRI) of the liver and a liver biopsy. Based on these findings plus clinical observations, she was diagnosed with peliosis hepatis and her steroid treatment was terminated. The patient recovered completely 3 months after steroid discontinuation, and remained stable over the following 6 months. PMID:26770928

  10. Cytokine-induced killer cell therapy-associated idiopathic thrombocytopenic purpura: rare but noteworthy.

    Science.gov (United States)

    Fu, Xiaomin; Zhang, Yong; Gao, Quanli; Lin, Jizhen; Zhang, Qinxian; Xu, Benling; Song, Yongping

    2016-09-01

    Idiopathic thrombocytopenic purpura (ITP) is characterized by a diminished platelet count, an autoimmune condition with antibodies against platelets and an increased tendency to bleed. The association between ITP and solid tumors is uncommon. Cytokine-induced killer (CIK) cell therapy is a well tolerated and promising cancer treatment with minimal toxicity. For the first time, CIK cell therapy was reported to be followed by ITP. The mechanism through which CIK induces ITP remains unclear. Imbalanced ratio of Th cells, decreased numbers or impaired function of Treg cells and excessive secretion of cytokines inducing abnormal activation of B cells may be among the possible reasons. Therefore, a better understanding of this rare condition will require further investigation of these cases. PMID:27485074

  11. Nocardia transvalensis Disseminated Infection in an Immunocompromised Patient with Idiopathic Thrombocytopenic Purpura

    Science.gov (United States)

    García-Méndez, Jorge; Carrillo-Casas, Erika M.; Rangel-Cordero, Andrea; Leyva-Leyva, Margarita; Xicohtencatl-Cortes, Juan; Arenas, Roberto; Hernández-Castro, Rigoberto

    2016-01-01

    Nocardia transvalensis complex includes a wide range of microorganisms with specific antimicrobial resistance patterns. N. transvalensis is an unusual Nocardia species. However, it must be differentiated due to its natural resistance to aminoglycosides while other Nocardia species are susceptible. The present report describes a Nocardia species involved in an uncommon clinical case of a patient with idiopathic thrombocytopenic purpura and pulmonary nocardiosis. Microbiological and molecular techniques based on the sequencing of the 16S rRNA gene allowed diagnosis of Nocardia transvalensis sensu stricto. The successful treatment was based on trimethoprim-sulfamethoxazole and other drugs. We conclude that molecular identification of Nocardia species is a valuable technique to guide good treatment and prognosis and recommend its use for daily bases diagnosis. PMID:27313917

  12. Thrombotic Thrombocytopenic Purpura in Black People: Impact of Ethnicity on Survival and Genetic Risk Factors.

    Science.gov (United States)

    Martino, Suella; Jamme, Mathieu; Deligny, Christophe; Busson, Marc; Loiseau, Pascale; Azoulay, Elie; Galicier, Lionel; Pène, Frédéric; Provôt, François; Dossier, Antoine; Saheb, Samir; Veyradier, Agnès; Coppo, Paul

    2016-01-01

    Black people are at increased risk of thrombotic thrombocytopenic purpura (TTP). Whether clinical presentation of TTP in Black patients has specific features is unknown. We assessed here differences in TTP presentation and outcome between Black and White patients. Clinical presentation was comparable between both ethnic groups. However, prognosis differed with a lower death rate in Black patients than in White patients (2.7% versus 11.6%, respectively, P = .04). Ethnicity, increasing age and neurologic involvement were retained as risk factors for death in a multivariable model (P TTP, HLA-DRB1*04, was dramatically decreased in Black individuals in comparison with White individuals. Black people with TTP may have a better survival than White patients despite a comparable disease severity. A low natural frequency of HLA-DRB1*04 in Black ethnicity may account for the greater risk of TTP in this population. PMID:27383202

  13. Neglect-induced pseudo-thrombotic thrombocytopenic purpura due to vitamin B12 deficiency.

    Science.gov (United States)

    Asano, Takeshi; Narazaki, Hidehiko; Kaizu, Kiyohiko; Matsukawa, Shouhei; Takema-Tochikubo, Yuki; Fujii, Shuichi; Saitoh, Nobuyuki; Mashiko, Kunihiko; Fujino, Osamu

    2015-10-01

    Although thrombotic thrombocytopenic purpura (TTP) is rare, early diagnosis and treatment are important for decreasing the mortality rate. Acquired vitamin B12 deficiency is frequently overlooked because of its rarity in developed countries, particularly in children and adolescents. The hematological changes in vitamin B12 deficiency present as megaloblastic anemia, increased lactate dehydrogenase, vasoconstriction, increased platelet aggregation, and abnormal activation of the coagulation followed by microangiopathy as well as neutropenia and thrombocytopenia. We report herein the case of a 15-year-old girl who had been neglected, which might have caused pseudo-TTP through malnutrition, particularly vitamin B12 deficiency. When we encounter cases of TTP in children, clinicians must be aware of the possibility of malnutrition, particularly with vitamin B12 deficiency, even in developed countries, and investigate the cause of malnutrition including neglect. PMID:26387768

  14. Nocardia transvalensis Disseminated Infection in an Immunocompromised Patient with Idiopathic Thrombocytopenic Purpura

    Directory of Open Access Journals (Sweden)

    Jorge García-Méndez

    2016-01-01

    Full Text Available Nocardia transvalensis complex includes a wide range of microorganisms with specific antimicrobial resistance patterns. N. transvalensis is an unusual Nocardia species. However, it must be differentiated due to its natural resistance to aminoglycosides while other Nocardia species are susceptible. The present report describes a Nocardia species involved in an uncommon clinical case of a patient with idiopathic thrombocytopenic purpura and pulmonary nocardiosis. Microbiological and molecular techniques based on the sequencing of the 16S rRNA gene allowed diagnosis of Nocardia transvalensis sensu stricto. The successful treatment was based on trimethoprim-sulfamethoxazole and other drugs. We conclude that molecular identification of Nocardia species is a valuable technique to guide good treatment and prognosis and recommend its use for daily bases diagnosis.

  15. Response to rituximab in a refractory case of thrombotic thrombocytopenic purpura associated with systemic lupus erythematosus

    Directory of Open Access Journals (Sweden)

    Niaz Faraz

    2010-01-01

    Full Text Available Thrombotic thrombocytopenic purpura (TTP is a serious disorder with a significant morbidity and mortality. Majority of cases of TTP are idiopathic, but some cases may be secon-dary to connective tissue diseases. TTP has been rarely associated with systemic lupus erythe-matosus (SLE and may be refractory to treatment with plasma exchange, requiring immuno-suppressive therapy. We describe a patient with TTP and SLE who was refractory to plasma exchange and corticosteroids but responded to anti-CD20 antibody rituximab with continued re-mission after eight months of follow-up. Rituximab appears to be an effective treatment in re-fractory cases of TTP associated with SLE.

  16. Clopidogrel-induced refractory thrombotic thrombocytopenic purpura successfully treated with rituximab.

    Science.gov (United States)

    Khodor, Sara; Castro, Miguel; McNamara, Colin; Chaulagain, Chakra P

    2016-06-01

    Thrombotic thrombocytopenic purpura (TTP) is a multisystem disorder characterized by microvascular aggregation of platelets and fibrin strands causing thrombocytopenia, microangiopathic hemolytic anemia, and organ dysfunction. TTP can develop as a result of a deficiency in ADAMTS13 enzyme activity due to either a genetic defect or, more commonly, the development of anti-ADAMTS13 autoantibodies. TTP can also be associated with pregnancy, organ transplant, lupus, infections, and drugs. Here, we present a case of TTP that developed shortly after the start of clopidogrel treatment for acute ischemic stroke and acute myocardial infarction, and describe the clinical presentation, refractory course of the disease, and successful induction of remission through the use of rituximab in a setting of pre-existing autoimmune diseases. PMID:26684918

  17. Is Tc-99m Sulfur Colloid Scintigraphy Necessary in Chronic Immune Thrombocytopenic Purpura Before Splenectomy?

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    Ilçe HT et al.

    2011-05-01

    Full Text Available One of the most common reasons for elective splenectomy on adults ischronic immune thrombocytopenic purpura. It is characterized by thrombocyte destruction in spleen, so, management of splenectomy is the gold standard. However if there is remnant spleen tissue postoperatively it cause to going on thrombocytopenia. The principal reason of remnant spleen tissue is accessory spleen. So it is important to detect this tissue pre or postoperatively. Thirty years old, male patient underwent splenectomy four years ago because of chronic immunethrombocytopenic purpura. When thrombocytopenia recurrence occurredabdominal ultrasonography was performed and there was no abnormal sign. Then, Tc–99 m Sulfur Colloid Spleen Scintigraphy was performed and spleen tissue was detected in left hypochondriac region. Tc-99mSulfur Colloid Spleen Scintigraphy is one of the imaging method for accessory spleen. Especially if it is performed preoperatively the surgeon can be careful during the operation and at the same time detected accessory spleen was removed and recurrence can be prevented.

  18. The relationship between T CD4+ cells count and IL-17, IL-11 serum level in idiopathic thrombocytopenic purpura

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    Nayereh Alizadeh

    2014-04-01

    Conclusion: In summary, our study indicated a role of IL-11 in ITP patients, also showed that ITP may not be associated with changes of plasma IL-17 levels and T CD4+ cells count relative to control population. Therefore, measurement of plasma IL-11 levels may be important criteria in development of ITP. In addition, it is concluded that determination of IL-11 can be a diagnostic marker to recognize thrombocytopenic purpura patients.

  19. Treatment of Infantile Chronic Idiopathic Thrombocytopenic Purpura by Auxiliary Use of Auriculo-Acupoint Pressing

    Institute of Scientific and Technical Information of China (English)

    卢燕

    2001-01-01

    @@According to the TCM theories of Meridian and Pulse-Picture, the author probed in the treatment of idiopathic thrombocytopenic purpura (ITP) with auriculo-acupoint pressing (AAP) and obtained good result. The study was reported as follows. METHODS General Materials Forty-five patients with chronic refractory of ITP selected from the 269 ITP in-patients, hospitalized from January 1991 to January 1998, were observed. They were diagnosed according to the clinical manifestations, peripheral blood picture and bone marrow examination, as well as platelet antibody test in some of them, which were all in accordance with the unified diagnostic standard of ITP in China(1). All of the patients had course of disease over half a year and their disease treated with hormone for 2-3 months ineffectively, and had hemorrhagic symptoms, such as dermatorrhagia and rhinorrhagia, with no hepatosplenomegaly, and platelet count within 8-72×109/L. The hormone therapy was withdrawn or stopped gradually in the observation period.

  20. Does Helicobacter pylori play a role in the pathogenesis of childhood chronic idiopathic thrombocytopenic purpura?

    Science.gov (United States)

    Maghbool, Maryam; Maghbool, Masood; Shahriari, Mehdi; Karimi, Mehran

    2009-01-01

    Idiopathic thrombocytopenic purpura (ITP) is an acute self-limited bleeding disorder that can progress to chronic form in 10-15% of the cases. Helicobacter pylori (H. pylori) infection is a possible cause of chronic ITP. We studied 30 children with resistant chronic ITP for H. pylori infection based on the detection of H. pylori fecal antigen. This retrospective study was based on data obtained from medical records of 30 children aged between five and 17 years (median age at ITP diagnosis was ten years). A specially-designed data sheet was used to record information on age, sex, duration of disease, family history of bleeding disorders, previous treatments and median platelet count. In patients with H. pylori infection, antimicrobial treatment consisted of amoxicillin, metronidazol and omeprazol. Response was assessed every month for one year and defined as complete (platelet count >150×10(9)/L) or partial (platelet count between 50 and 150×10(9)/L). We detected H. pylori infection in 5 patients. In 4 of them increased platelet count was seen during one year of follow-up and in one patient the platelet count was acceptable during six months. Although the pathological mechanism of H. pylori-induced thrombocytopenia was unclear in our patient sample, the assessment of H. pylori infection and use of eradication therapy should be attempted in chronic and resistant ITP patients. PMID:21589818

  1. Life-threatening autoimmune hemolytic anemia and idiopathic thrombocytopenic purpura: successful seletive splenic artery embolization

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    matteo molica

    2016-04-01

    Full Text Available Selective splenic artery embolization (SSAE is a nonsurgical intervention characterized by the transcatheter occlusion of the splenic artery and/or its branch vessels using metallic coils or other embolic devices. It has been applied for the management of splenic trauma, hypersplenism with portal hypertension, hereditary spherocytosis, thalassemia and splenic hemangioma. We hereby describe a case of a patient affected by idiopathic thrombocytopenic purpura (ITP and warm auto-immune hemolytic anemia (AIHA both resistant to immunosuppressive and biological therapies, not eligible for a surgical intervention because of her critical conditions. She underwent SSAE and achieved a hematologic complete response within a few days without complications. SSAE is a minimally invasive procedure to date not considered a standard option in the management of AIHA and ITP. However, following the progressive improvement of the techniques, its indications have been extended, with a reduction in morbidity and mortality compared to splenectomy in patients with critical clinical conditions. SSAE was a lifesaving therapeutic approach for our patient and it may represent a real alternative for the treatment of resistant AIHA and ITP patients not eligible for splenectomy.

  2. Does Helicobacter pylori play a role in the pathogenesis of childhood chronic idiopathic thrombocytopenic purpura?

    Directory of Open Access Journals (Sweden)

    Maryam Maghbool

    2009-07-01

    Full Text Available Idiopathic thrombocytopenic purpura (ITP is an acute self-limited bleeding disorder that can progress to chronic form in 10-15% of the cases. Helicobacter pylori (H. pylori infection is a possible cause of chronic ITP. We studied 30 children with resistant chronic ITP for H. pylori infection based on the detection of H. pylori fecal antigen. This retrospective study was based on data obtained from medical records of 30 children aged between five and 17 years (median age at ITP diagnosis was ten years. A specially-designed data sheet was used to record information on age, sex, duration of disease, family history of bleeding disorders, previous treatments and median platelet count. In patients with H. pylori infection, antimicrobial treatment consisted of amoxicillin, metronidazol and omeprazol. Response was assessed every month for one year and defined as complete (platelet count >150x109/L or partial (platelet count between 50 and 150x109/L. We detected H. pylori infection in 5 patients. In 4 of them increased platelet count was seen during one year of follow-up and in one patient the platelet count was acceptable during six months. Although the pathological mechanism of H. pylori-induced thrombocytopenia was unclear in our patient sample, the assessment of H. pylori infection and use of eradication therapy should be attempted in chronic and resistant ITP patients.

  3. Refractory Immune Thrombocytopenic Purpura and Cytomegalovirus Infection: A Call for a Change in the Current Guidelines

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    Alex Shimanovsky

    2016-01-01

    Full Text Available Immune thrombocytopenic purpura (ITP is characterized by a decreased platelet count caused by excess destruction of platelets and inadequate platelet production. In many cases the etiology is not known, but viral illness is thought to play a role in the development of some cases of ITP. The current (2011 American Society of Hematology ITP guidelines recommend initial diagnostic studies to include testing for HIV and Hepatitis C. The guidelines suggest that initial treatment consist of observation, therapy with corticosteroids, IVIG or anti D. While most cases respond to the standard therapy such that the steroids may be tapered and the platelet counts remain at a hemostatically safe level. Some patients with ITP are dependent on long term steroid maintenance and the thrombocytopenia persists with the tapering of the steroids. Recent case reports demonstrate that ITP related to cytomegalovirus (CMV can persist in spite of standard therapy and that antiviral therapy maybe indicated. Herein we report a case of a 26-year-old female with persistent ITP that resolved after the delivery of a CMV infected infant and placenta. Furthermore we review the current literature on CMV-associated ITP and propose that the current ITP guidelines be amended to include assessment for CMV as part of the work-up for severe and refractory ITP prior to splenectomy.

  4. Immunologic effects of anti-D (WinRho-SD) in children with immune thrombocytopenic purpura.

    Science.gov (United States)

    Zimmerman, S A; Malinoski, F J; Ware, R E

    1998-02-01

    Intravenous immunoglobulin (IVIG) is an effective treatment for immune thrombocytopenic purpura (ITP) that induces transient blockade of the reticuloendothelial system (RES) with additional effects including alteration of T lymphocyte subsets and suppression of in vitro T lymphocyte proliferation. As anti-D also is an effective treatment for ITP, we investigated its in vitro and in vivo immunologic effects. The in vitro effects of various agents used in ITP therapy were compared using T lymphocyte proliferation assays. Anti-D caused significantly less inhibition than IVIG or dexamethasone, but non-specific protein was as suppressive as IVIG. Six children with chronic ITP were studied following anti-D administration. Patients received a single dose of anti-D (WinRho-SD, 50 microg/kg i.v. over 5 min) and were studied on day 0, day 7, and 1 month later. Anti-D did not affect T lymphocyte subsets including the T cell receptor variable beta repertoire, in vitro T lymphocyte proliferation to mitogens, recall antigens, or interleukin-2, in vitro IgG synthesis induced by pokeweed mitogen, or T lymphocyte cytokine mRNA levels. We conclude that anti-D has no demonstrable in vitro or in vivo effects on lymphocyte enumeration or function, and therefore likely is effective in the treatment of ITP primarily through RES blockade. PMID:9462545

  5. Impact of Helicobacter pylori Eradication Therapy on Platelet Counts in Patients With Chronic Idiopathic Thrombocytopenic Purpura.

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    Amiri, Mohamadreza

    2016-01-01

    This study was a before and after clinical evaluation of Helicobacter pylori eradication on platelet counts in a group of 23 patients with chronic Idiopathic (Autoimmune) thrombocytopenic purpura (CITP). H. pylori infection was identified in patients by a (13)C-urea breath test and confirmed by an H. pylori stool antigen test. Eradication was conducted in patients testing positive. Infected (n = 10) and uninfected (n = 13) patient groups did not differ with respect to age, gender, history of previous splenectomy, treatment with anti-D, current treatment with corticosteroids, or initial platelet counts. H pylori eradication was successful in eight infected CITP patients, with two patients not responsive to treatment. Compared to the uninfected group, patients in the infected group who responded to eradication therapy had significantly increased platelet counts after six months (56.2 ± 22.2 vs. 233 ± 85.6 ×10(3) million cells/L; P < 0.01), whereas platelet counts in the non-responding patients and uninfected group did not differ after this period of time. H. pylori eradication promotes significant platelet count improvement in patients with CITP. Thus, all patients with CITP should be tested and treated for H. pylori infections. PMID:26925898

  6. Acquired Idiopathic ADAMTS13 Activity Deficient Thrombotic Thrombocytopenic Purpura in a Population from Japan

    Science.gov (United States)

    Matsumoto, Masanori; Bennett, Charles L.; Isonishi, Ayami; Qureshi, Zaina; Hori, Yuji; Hayakawa, Masaki; Yoshida, Yoko; Yagi, Hideo; Fujimura, Yoshihiro

    2012-01-01

    Thrombotic thrombocytopenic purpura (TTP) is a type of thrombotic microangiopathy (TMA). Studies report that the majority of TTP patients present with a deficiency of ADAMTS13 activity. In a database of TMA patients in Japan identified between 1998 and 2008, 186 patients with first onset of acquired idiopathic (ai) ADAMTS13-deficient TTP (ADAMTS13 activity <5%) were diagnosed. The median age of onset of TTP in this group of patients was 54 years, 54.8% were female, 75.8% had renal involvement, 79.0% had neurologic symptoms, and 97.8% had detectable inhibitors to ADAMTS13 activity. Younger patients were less likely to present with renal or neurologic dysfunction (p<0.01), while older patients were more likely to die during the TTP hospitalization (p<0.05). Findings from this cohort in Japan differ from those reported previously from the United States, Europe, and Korea with respect to age at onset (two decades younger in the other cohort) and gender composition (60% to 100% female in the other cohort). We conclude that in one of the largest cohorts of ai-TTP with severe deficiency of ADAMTS13 activity reported to date, demographic characteristics differ in Japanese patients relative to those reported from a large Caucasian registry from Western societies. Additional studies exploring these findings are needed. PMID:22427934

  7. Acquired idiopathic ADAMTS13 activity deficient thrombotic thrombocytopenic purpura in a population from Japan.

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    Masanori Matsumoto

    Full Text Available Thrombotic thrombocytopenic purpura (TTP is a type of thrombotic microangiopathy (TMA. Studies report that the majority of TTP patients present with a deficiency of ADAMTS13 activity. In a database of TMA patients in Japan identified between 1998 and 2008, 186 patients with first onset of acquired idiopathic (ai ADAMTS13-deficient TTP (ADAMTS13 activity <5% were diagnosed. The median age of onset of TTP in this group of patients was 54 years, 54.8% were female, 75.8% had renal involvement, 79.0% had neurologic symptoms, and 97.8% had detectable inhibitors to ADAMTS13 activity. Younger patients were less likely to present with renal or neurologic dysfunction (p<0.01, while older patients were more likely to die during the TTP hospitalization (p<0.05. Findings from this cohort in Japan differ from those reported previously from the United States, Europe, and Korea with respect to age at onset (two decades younger in the other cohort and gender composition (60% to 100% female in the other cohort. We conclude that in one of the largest cohorts of ai-TTP with severe deficiency of ADAMTS13 activity reported to date, demographic characteristics differ in Japanese patients relative to those reported from a large Caucasian registry from Western societies. Additional studies exploring these findings are needed.

  8. Thrombotic thrombocytopenic purpura (TTP or Moschowitz syndrome: a true hematologic emergency

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    Deborah Melis

    2012-01-01

    Full Text Available Introduction: Thrombotic thrombocytopenic purpura (TTP is a thrombotic microangiopathy caused by congenital or inherited disorders involving the processing of the ultra-large forms of von Willebrand factor. As a result, platelet-rich microthrombi form in the small arterial vessels of various organs, particularly those of the brain, heart, and kidneys. The idiopathic autoimmune form of TTP is the most common. There are various subgroups of acquired TTP associated with HIV infection, sepsis, pregnancy, autoimmune disease, various disseminated malignancies, and drugs. If not promptly treated, TTP is associated with high mortality, making it a true medical emergency. Materials and methods: The article is based on a review of the literature published between January and October of 2009. Its aim is to clarify the diagnosis, treatment, and follow-up of TTP. Results: Diagnostic criteria include the presence of microangiopathic hemolytic anemia associated with thrombocytopenia in the absence of other obvious causes. Assays of ADAMTS13 activity and titration of acquired antibodies against this enzyme are indicated in the follow-up of disease and as prognostic indicators. Treatment centers around daily plasma exchange associated with immunosuppressant drug therapy, particularly steroids and more recently the monoclonal anti-CD20 antibody rituximab. Discussion: Despite improved treatment, TTP is still associated with significant mortality (10—20%, particularly when plasma exchange is initiated late. Relapse also occurs in a substantial proportion of patients (10—40% although the frequency of this outcome may be reduced by rituximab therapy.

  9. When the picture is fragmented: Vitamin B12 deficiency masquerading as thrombotic thrombocytopenic purpura.

    Science.gov (United States)

    Panchabhai, Tanmay S; Patil, Pradnya D; Riley, Elizabeth C; Mitchell, Charlene K

    2016-01-01

    Thrombotic thrombocytopenic purpura (TTP) has high mortality and necessitates prompt recognition of microangiopathic hemolytic anemia (MAHA) and initiation of plasmapheresis. We present a challenging diagnostic workup and management of a 42-year-old man who presented with anemia, thrombocytopenia, and schistocytes on peripheral smear, all pointing to MAHA. Plasmapheresis and steroid therapy were promptly initiated, but hemolysis continued. Further workup showed megaloblastic anemia, severe Vitamin B12 deficiency, high iron saturation, and absent reticulocytosis, none of which could be explained by TTP. Severe Vitamin B12 deficiency can lead to hemolytic anemia from the destruction of red cells in the marrow that have failed the process of maturation. However, this should not cause thrombotic microangiopathy. Previous reports of B12 deficiency presenting with MAHA and a TTP-like manifestation have identified acute hyperhomocysteinemia as a missing link between B12 deficiency and MAHA, so this possibility was further explored. Our patient similarly had significantly elevated serum homocysteine levels, confirming this suspicion of Vitamin B12 deficiency. Vitamin B12 replacement led to normalization of the elevated levels of homocysteine, the disappearance of schistocytes on the peripheral smear, and resolution of the microangiopathic hemolysis, thereby confirming the diagnosis. It is pertinent that intensivists not only know the importance of early recognition and treatment of TTP but are also familiar with rare conditions that can present in a similar fashion. PMID:27308258

  10. Acute renal failure and severe rhabdomyolysis in a patient with resistant thrombotic thrombocytopenic purpura

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    Al Qahtani S

    2011-10-01

    Full Text Available Saad Al Qahtani Intensive Care Department, Critical Care Response Team, King Abdulaziz Medical City (KAMC, National Guard Health Affairs; King Saud Bin Abdulaziz University for Health Sciences, College of Medicine, Riyadh, Kingdom of Saudi Arabia Abstract: Thrombotic thrombocytopenic purpura (TTP is a rare, life-threatening disorder. This paper describes the case of a 39-year-old Sudanese male who presented to the emergency room with fever, jaundice, decreased level of consciousness, and worsening kidney function for 7 days, a high lactate dehydrogenase level (1947, severe thrombocytopenia (platelets 8, and numerous schistocytes in the peripheral blood smear. The patient was admitted with a diagnosis of TTP for plasma exchange. Fourteen days later, his creatinine kinase (CK level rose to >50,000 IU; rhabdomyolysis was suggested. Continuous venovenous hemodialysis (CVVHD was started. The patient's CK level remained high, despite CVVHD, until the 6th day, after which this parameter gradually started to decrease. This report highlights a resistant case of TTP that presented with concomitant severe rhabdomyolysis, which demanded aggressive, continuous intervention. Keywords: TTP, CVVHD, continuous venovenous hemodialysis

  11. Evaluation of humoral immune function in patients with chronic idiopathic thrombocytopenic purpura.

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    Mohammad Saeid Rahiminejad

    2013-03-01

    Full Text Available Coincidence of autoimmune diseases such as immune thrombocytopenic purpura (ITP with  immunodeficiencies has  been  reported  previously in  patients  who  suffered  from primary antibody deficiency (PAD. But there is no original study on immunological profiles of ITP patients to find out their probable immune deficiency.In this case-control study, ITP patients’ humoral immunity was investigated for diagnosis of PAD in comparison with normal population. To evaluate the humoral immune system against polysaccharide antigens, patients’ serum immunoglobulin levels were measured and a 23-valent pneumococcal  capsular polysaccharide vaccine (PPV23 was administrated  to evaluate the antibody response to vaccination.In  this  study, 14 out  of  36 patients  (39% were diagnosed with antibody mediated immune deficiency including 2 patients (5.5% with immunoglobulin class deficiency and 4 (11% with IgG subclass deficiency. The remaining patients suffered from specific antibody deficiency. The most frequent deficiency in ITP patients was specific antibody deficiency.Therefore, immunological survey on ITP patients may be important especially for those who have undergone splenectomy.

  12. Emergency Plasmapheresis in a case of ThromboticThrombocytopenic Purpura (TTP

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    Mariaserena Pioli Di Marco

    2013-12-01

    Full Text Available An 84 year-old female was admitted to our Department of Vascular Internal Medicine after a sudden onset of weakness on her right side and aphasia along with signs of myocardial ischemia from Electrocardiogram (EKG. Clinical and blood exams led to a suspicion of Moschcowitz syndrome, which was reinforced by the presence of numerous schistocytes on a peripheral blood smear.Due to a rapid deterioration of vital signs as well as alertness, the patient underwent an emergency transfusion and plasmapheresis treatment as recommended by American Society of Apheresis (ASFA guidelines: one plasma volume was replaced with fresh frozen plasma (FFP every 24 hours, for the first eight days, in order to reach at least a level of 150,000 platelets/mm3 over three consecutive days accompanied by a decrease in LDH until to 670 UI/l.After this therapy, the clinical picture significantly improved with a complete recovery of consciousness and the disappearance of neurological defects.Examinations to determine the etiology made us hypothesize a secondary status of thrombotic thrombocytopenic purpura due to an autoimmune disorder compatible with Sjogren’s syndrome. The patient was discharged and prescribed prednisone.Currently the patient is in good clinical condition and continues the therapy with prednisone (5 mg/die.

  13. [Antiphospholipid syndrome with autoimmune hemolytic anemia which mimics thrombotic thrombocytopenic purpura].

    Science.gov (United States)

    Karasawa, Naoki; Taniguchi, Yasuhiro; Hidaka, Tomonori; Katayose, Keiko; Kameda, Takuro; Side, Kotaro; Shimoda, Haruko; Nagata, Kenji; Kubuki, Yoko; Matsunaga, Takuya; Shimoda, Kazuya

    2010-04-01

    A 67-year-old woman was admitted to the hospital for lethargy, fever, hemolytic anemia, thrombocytopenia, and consciousness disturbance. Direct Coombs test was positive, and anti-cardiolipin beta2-glycoprotein I antibody was detected. She was diagnosed with antiphospholipid syndrome complicated with autoimmune hemolytic anemia (AIHA). She demonstrated variable consciousness disturbance, inability to distinguish right from left, dysgraphia and dyscalculia. Multiple cerebral infarctions, especially dominant cerebral hemisphere infarctions, were observed on magnetic resonance imaging. A ventilation-perfusion scan demonstrated the presence of a ventilation-perfusion mismatch in both lung fields, and multiple veinous embolisms in the right femoral, bilateral the great saphenous and popliteal veins. Therefore, pulmonary embolism and thrombophlebitis were diagnosed. Based on these findings, it was necessary to distinguish this diagnosis from thrombotic thrombocytopenic purpura (TTP). As ADAMTS-13 activity was within the normal range, TTP was denied. Thereafter, the patient was treated with 1 mg/kg of prednisolone for AIHA, 3 mg of warfarin, and 3500 units of low-molecular-weight heparin for thrombosis, and her condition improved. PMID:20467225

  14. Thrombotic Thrombocytopenic Purpura Associated with Mixed Connective Tissue Disease: A Case Report

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    João Tadeu Damian Souto Filho

    2011-01-01

    Full Text Available Thrombotic thrombocytopenic purpura (TTP is a multisystemic disorder characterized by microangiopathic hemolytic anemia and thrombocytopenia, which may be accompanied by fever, renal, or neurologic abnormalities. Cases are divided into acute idiopathic TTP and secondary TTP. Autoimmune diseases, especially systemic lupus erythematosus, in association with TTP have been described so far in many patients. In contrast, TTP occurring in a patient with mixed connected tissue disease (MCTD is extremely rare and has only been described in nine patients. We describe the case of a 42-year-old female with MCTD who developed thrombocytopenia, microangiopathic hemolytic anemia, fever, and neurological symptoms. The patient had a good clinical evolution with infusion of high volume of fresh frozen plasma, steroid therapy, and support in an intensive care unit. Although the occurrence of TTP is rare in MCTD patients, it is important to recognize TTP as a cause of thrombocytopenia and hemolytic anemia in any patient with autoimmune diseases. Prompt institution of treatment remains the cornerstone of treatment of TTP even if plasma exchange is not available like what frequently happens in developing countries.

  15. Common variable immunodeficiency unmasked by treatment of immune thrombocytopenic purpura with Rituximab

    DEFF Research Database (Denmark)

    Mogensen, Trine H; Jensen, Jens Magnus Bernth; Petersen, Charlotte C;

    2013-01-01

    BACKGROUND: Hypogammaglobulinemia may be part of several different immunological or malignant conditions, and its origin is not always obvious. Furthermore, although autoimmune cytopenias are known to be associated with common variable immunodeficiency (CVID) and even may precede signs of immunod......BACKGROUND: Hypogammaglobulinemia may be part of several different immunological or malignant conditions, and its origin is not always obvious. Furthermore, although autoimmune cytopenias are known to be associated with common variable immunodeficiency (CVID) and even may precede signs...... of immunodeficiency, this is not always recognized. Despite novel insight into the molecular immunology of common variable immunodeficiency, several areas of uncertainty remain. In addition, the full spectrum of immunological effects of the B cell depleting anti-CD20 antibody Rituximab has not been fully explored....... To our knowledge this is the first report of development of CVID in a patient with normal immunoglobulin prior to Rituximab treatment. CASE PRESENTATION: Here we describe the highly unusual clinical presentation of a 34-year old Caucasian male with treatment refractory immune thrombocytopenic purpura...

  16. Thrombotic Thrombocytopenic Purpura in Black People: Impact of Ethnicity on Survival and Genetic Risk Factors.

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    Suella Martino

    Full Text Available Black people are at increased risk of thrombotic thrombocytopenic purpura (TTP. Whether clinical presentation of TTP in Black patients has specific features is unknown. We assessed here differences in TTP presentation and outcome between Black and White patients. Clinical presentation was comparable between both ethnic groups. However, prognosis differed with a lower death rate in Black patients than in White patients (2.7% versus 11.6%, respectively, P = .04. Ethnicity, increasing age and neurologic involvement were retained as risk factors for death in a multivariable model (P < .05 all. Sixty-day overall survival estimated by the Kaplan-Meier curves and compared with the Log-Rank test confirmed that Black patients had a better survival than White patients (P = .03. Salvage therapies were similarly performed between both groups, suggesting that disease severity was comparable. The comparison of HLA-DRB1*11, -DRB1*04 and -DQB1*03 allele frequencies between Black patients and healthy Black individuals revealed no significant difference. However, the protective allele against TTP, HLA-DRB1*04, was dramatically decreased in Black individuals in comparison with White individuals. Black people with TTP may have a better survival than White patients despite a comparable disease severity. A low natural frequency of HLA-DRB1*04 in Black ethnicity may account for the greater risk of TTP in this population.

  17. Thrombotic Thrombocytopenic Purpura in Black People: Impact of Ethnicity on Survival and Genetic Risk Factors

    Science.gov (United States)

    Martino, Suella; Jamme, Mathieu; Deligny, Christophe; Busson, Marc; Loiseau, Pascale; Azoulay, Elie; Galicier, Lionel; Pène, Frédéric; Provôt, François; Dossier, Antoine; Saheb, Samir; Veyradier, Agnès; Coppo, Paul

    2016-01-01

    Black people are at increased risk of thrombotic thrombocytopenic purpura (TTP). Whether clinical presentation of TTP in Black patients has specific features is unknown. We assessed here differences in TTP presentation and outcome between Black and White patients. Clinical presentation was comparable between both ethnic groups. However, prognosis differed with a lower death rate in Black patients than in White patients (2.7% versus 11.6%, respectively, P = .04). Ethnicity, increasing age and neurologic involvement were retained as risk factors for death in a multivariable model (P < .05 all). Sixty-day overall survival estimated by the Kaplan-Meier curves and compared with the Log-Rank test confirmed that Black patients had a better survival than White patients (P = .03). Salvage therapies were similarly performed between both groups, suggesting that disease severity was comparable. The comparison of HLA-DRB1*11, -DRB1*04 and -DQB1*03 allele frequencies between Black patients and healthy Black individuals revealed no significant difference. However, the protective allele against TTP, HLA-DRB1*04, was dramatically decreased in Black individuals in comparison with White individuals. Black people with TTP may have a better survival than White patients despite a comparable disease severity. A low natural frequency of HLA-DRB1*04 in Black ethnicity may account for the greater risk of TTP in this population. PMID:27383202

  18. [Clinical significance of Helicobacter pylori in children with idiopathic thrombocytopenic purpura].

    Science.gov (United States)

    Tang, Ying; Wang, Shu-Chun; Wang, Lu-Juan; Liu, Yong; Wang, Hai-Ying; Wang, Zhan-Ju

    2013-04-01

    This study was aimed to investigate the clinic significance of helicobacter pylori (HP) in children with idiopathic thrombocytopenic purpura (ITP). The infection of HP in 92 ITP children was determined by (13) C-Urea Breath Test, the same test was also performed on 66 healthy children. The 68 children infected with HP were randomly divided into 2 groups: single drug group treated only with corticosteroid and; combined drug group treated with corticosteroid and anti-helicobacter pylori treatment. The results showed that 68 patients infected with HP were found in 92 ITP children (74.7%), 26 patients infected with HP were observed in 66 healthy children (39.4%), which was lower than that in ITP children (74.7%, P helicobacter pylori therapy, the total effective rate and cure rate of ITP patients increased respectively from 73.5% to 94.1%, and the total recurrence rate (17.0%) was much lower than single drug group (47.1%, P helicobacter pylori group was higher than that in the single drug group (P helicobacter pylori therapy would help to improve the therapeutic efficacy and reduce the recurrence of ITP children.

  19. Evaluation of von Willebrand factor-cleaving protease activity in patients with thrombotic thrombocytopenic purpura

    Institute of Scientific and Technical Information of China (English)

    高维强; 苏健; 白霞; 王兆钺; 阮长耿

    2004-01-01

    Background Thrombotic thrombocytopenic purpura (TTP) is a rare thrombotic microangiopathy. In this study we investigated the von Willebrand factor-cleaving protease (vWF-cp) activity deficiency in patients with TTP.Methods The plasma or serum vWF-cp activity was measured using a sensitive enzyme-linked immunosorbent assay (ELISA) by detecting the residual collagen binding activity (R-CBA) of von Willebrand factor (vWF) before and after digestion by vWF-cp. Multimers of vWF in plasma of patients with TTP were also analyzed by SDS-agarose electrophoresis. Moreover, the serum vWF-cp activities were compared between the patients with TTP and those with tumors.Results The coefficient of variation for intra-batch and inter-batch of the assay were 3.60% and 8.35%. The plasma and serum vWF-cp activity in healthy individuals were (78.79±9.17)% (n=30) and (79.47±10.78)% (n=53), respectively, while the plasma vWF-cp activity in 5 patients with TTP was markedly decreased [(21.83±19.98)%, P<0.001]. The unusually large vWF multimers were observed in two plasma samples of the patients with TTP. Although the vWF-cp activities in patients with benign and malignant tumors were also decreased (P<0.03 and P<0.001, respectively), they were relatively high in comparison with that of TTP patients (P<0.001).Conclusion Measurement of the vWF-cp activity using R-CBA is a simple and rapid method for diagnosing TTP. The vWF-cp activity in patients with TTP was markedly lower than those of patients with tumors.

  20. CD16 and CD32 Gene Polymorphisms May Contribute to Risk of Idiopathic Thrombocytopenic Purpura.

    Science.gov (United States)

    Xu, Jiannan; Zhao, Liyun; Zhang, Yan; Guo, Qingxu; Chen, Hui

    2016-01-01

    BACKGROUND Epidemiological studies have evaluated the associations of CD16 158F>V and CD32 131H>R gene polymorphisms with the risk of idiopathic thrombocytopenic purpura (ITP). MATERIAL AND METHODS Published studies on CD16 158F>V and CD32 131H>R polymorphisms with susceptibility to ITP were systematically reviewed until April 1, 2014. The Cochrane Library Database, Medline, CINAHL, EMBASE, Web of Science, and Chinese Biomedical Database (CBM) were used to search for relevant studies and then a meta-analysis was conducted by using Stata 12.0 software in order to produce consistent statistical results. RESULTS In total, 10 clinical case-control studies with 741 ITP patients and 1092 healthy controls were enrolled for quantitative data analysis. Results of this meta-analysis suggest that CD16 158F>V polymorphism had strong correlations with the susceptibility to ITP under 5 genetic models (all PR polymorphism and the susceptibility to ITP (all P>0.05). Subgroup analysis by ethnicity revealed that CD16 158F>V polymorphism was associated with the increased risk of ITP among both Caucasian and non-Caucasian populations. Nevertheless, no statistically significant correlations between CD32 131H>R polymorphism and the risk of ITP were observed among Caucasians and non-Caucasians (all P>0.05). CONCLUSIONS Our findings indicate that CD16 158F>V polymorphism may contribute to the increased risk of ITP, whereas CD32 131H>R polymorphism may not be an important risk factor for ITP. PMID:27315784

  1. Carboxiterminal pro-endothelin-1 as an endothelial cell biomarker in thrombotic thrombocytopenic purpura.

    Science.gov (United States)

    Mikes, Bálint; Sinkovits, György; Farkas, Péter; Csuka, Dorottya; Rázsó, Katalin; Réti, Marienn; Radványi, Gáspár; Demeter, Judit; Prohászka, Zoltán

    2016-05-01

    Thrombotic thrombocytopenic purpura (TTP) is characterised by the deficiency of the von Willebrand factor (VWF) cleaving protease (ADAMTS-13). Although several observations indicate an important role of endothelial activation in the pathogenesis of TTP, no reliable endothelial activation markers are available in the clinical management of TTP. Our aim was to investigate the presence of endothelial activation in TTP and to determine its connections with disease activity, therapy and complement activation. We enrolled 54 patients (median age 40.5; 44 females) and 57 healthy controls (median age 34; 30 females),VWF antigen, carboxiterminal-pro-endothelin-1 (CT-proET-1), complement Factor H and complement activation products (C3bBbP and SC5b-9) were measured. In both the acute and remission phase of TTP we found increased CT-proET-1 and VWF levels, while Factor H levels decreased compared with healthy controls. In remission, however, the elevated CT-proET-1 levels showed 22 % decrease when compared with the acute phase in paired samples (p=0.0031), whereas no changes for VWF and Factor H levels were observed. We also found positive correlations between CT-proET-1 levels and alternative pathway activation markers (C3bBbP; p=0.0360; r=0.4299). The data we present here demonstrate a role of endothelium activation in patients with acute TTP. The finding that CT-proET-1 levels decreased in remission compared with the acute phase further supports endothelial involvement. In addition, we show that endothelial activation also correlated with the activation of the alternative complement pathway. The data suggest that complement and endothelium activation jointly contribute to the development of TTP episodes in patients with predisposition to TTP. PMID:26763086

  2. Intravenous anti-D treatment of immune thrombocytopenic purpura: experience in 272 patients.

    Science.gov (United States)

    Scaradavou, A; Woo, B; Woloski, B M; Cunningham-Rundles, S; Ettinger, L J; Aledort, L M; Bussel, J B

    1997-04-15

    We report the results of intravenous anti-D (WinRho, WinRho SD) therapy in 261 non-splenectomized patients treated at the New York Hospital-Cornell Medical Center over the period from 1987 to 1994. Children (n = 124) and adult patients (n = 137) with classic immune thrombocytopenic purpura (ITP; n = 156) or human immunodeficiency virus (HIV) related thrombocytopenia (n = 105) and acute (n = 75) or chronic (n = 186) disease at the time of the initial anti-D treatment were studied. In addition, 11 previously splenectomized patients were treated as a separate group. Our objectives were to evaluate the following. (1) Efficacy of anti-D: The response after the initial infusion was analyzed according to clinical parameters, such as patient's age, HIV status, gender, disease duration, pretreatment platelet count, and hemoglobin value, as well as treatment-related factors, including the dose of anti-D, the solvent detergent treatment of the preparation, and the type of administration. (2) Use of anti-D as maintenance therapy: The duration of response after the initial infusion and the results of subsequent treatments were evaluated. (3) Safety/toxicity of anti-D: Postinfusion reactions and hemoglobin decrease after treatment were studied. Anti-D is a safe treatment providing a hemostatic platelet increase in greater than 70% of the Rh+ non-splenectomized patients. The group with the best results is HIV- children, but all patient groups respond and the effect lasts more than 21 days in 50% of the responders. Duration of response is not influenced by HIV status; furthermore, HIV+ patients show no adverse effects on hemoglobin decrease or HIV disease progression. Patients with chronic ITP after splenectomy have minimal or no response to intravenous anti-D. PMID:9108386

  3. Retrospective analysis of rituximab therapy and splenectomy in childhood chronic and refractory immune thrombocytopenic purpura.

    Science.gov (United States)

    Ay, Yilmaz; Karapinar, Tuba H; Oymak, Yesim; Toret, Ersin; Demirag, Bengu; Ince, Dilek; Ozcan, Esin; Moueminoglou, Nergial; Koker, Sultan A; Vergin, Canan

    2016-06-01

    Immune thrombocytopenic purpura (ITP) results from accelerated platelet destruction mediated by autoantibodies to platelet glycoproteins. Some patients with chronic ITP are refractory to all therapies [steroids, intravenous immunoglobulin (IVIG), anti-D and immunosuppresive drugs] and have chronic low platelet counts and episodic bleeding. We retrospectively evaluated the efficacy and safety of rituximab treatment and splenectomy in paediatric patients diagnosed with chronic and refractory ITP who were unresponsive to steroids, IVIG, cyclosporine and mycophenolate mofetil. Records of patients with chronic and refractory ITP in 459 patients with primary ITP who were followed up in our hospital from January 2005 to December 2014 were reviewed. Fifteen of patients received rituximab and/or applied splenectomy. Fifteen chronic ITP patients (10 boys, five girls) with a mean age of 10 years were enrolled in the study. Two of these patients were suffering from Evans syndrome. The median time since diagnosis of ITP was 10 years. The median follow-up duration after starting Rituximab and splenectomy were 13 and 9.5 months, respectively.None of the seven patients who were treated with rituximab achieved a response. A splenectomy was performed in six of the seven patients who had been treated with rituximab. Complete and partial responses were achieved in 67 and 33% of the patients, respectively. We evaluated the clinical characteristics and responses of chronic ITP patients who did not receive rituximab therapy and underwent a splenectomy. The success rate was 100% in the eight patients with chronic and refractory ITP. Rituximab therapy might not be beneficial for some children with severe chronic ITP who are refractory to standard agents. A splenectomy might be useful and preferable to rituximab. PMID:26656905

  4. The Effect of Costimulatory Factors in the Pathogenesis of Chronic Idiopathic Thrombocytopenic Purpura

    Institute of Scientific and Technical Information of China (English)

    崔国惠; 刘筱萍; 姚军霞

    2003-01-01

    To investigate the effect of costimulatory factors in the pathogenesis of chronic idiopathic thrombocytopenic purpura (CITP), we examined the expression of CD80 on platelets and megakaryocytes in patients with CITP and the controls by FACS. By using CD80 monoclonal antibody (McAb) to inhibit interaction among cells which is mediated by costimulatory factors, we observed the effect of CD80 McAb on the growth and maturation of megakaryocytic progenitors of patients with CITP in vitro. The results showed the expression of CD80 on platelets and megakaryocytes in CITP group was significantly higher than that in controls (P<0.01). There was a significantly positive correlation between the expression of CD80 on platelets and serum PAIgG in CITP (r =0.86, P<0. 05). The mean of various clone numbers (CFU-MK, BFU-MK and mCFU-MK) in CITP were all lower than those in controls (P<0. 05). In megakaryoeytes co-cultured with CD80 McAb, there was an increasing tendency of the number of CFU-MK and big CFU-MK (the number of megakaryocyte with GPⅢa positive was more than 20) and mediate CFU-MK (the number of megakaryocyte with GPⅢa positive was 11- 20). When the concentration of CD80 McAb was 10 μg/L, there was a significant difference in the number of megakaryocytic colony formation (CFUMK, BFU-MK and mCFU-MK) between the group with CD80 McAb and that without it (P<0.05). These showed the abnormality of costimulatory factors had important effect in the pathogenesis of CITP.

  5. Neonates born to mothers with immune thrombocytopenic purpura: a single-center experience of 20 years.

    Science.gov (United States)

    Bayhan, Turan; Tavil, Betül; Korkmaz, Ayşe; Ünal, Şule; Hanalioğlu, Damla; Yiğit, Şule; Gümrük, Fatma; Çetin, Mualla; Yurdakök, Murat

    2016-01-01

    Neonates born to mothers with immune thrombocytopenic purpura (ITP) have an increased risk of having thrombocytopenia and bleeding. The aim of our study was to determine maternal and fetal factors that can predict bleeding risk in neonates born to mothers with ITP, and effective treatment strategies by retrospective analysis of our single-center data. We performed a retrospective data review of neonates that were recorded as 'neonates born to mothers with ITP' in the Neonatal ICU of Hacettepe University, Ihsan Dogramacı Children's Hospital, Ankara, Turkey. Medical records of 36 neonates born from 35 mothers were analyzed. Among the 36 neonates born to mothers with ITP, thrombocytopenia (platelet count of less than 150 × 10/l) was detected in 20 (56.0%) neonates on the first day of life. Twelve of the 20 neonates with thrombocytopenia (60.0%) required treatment to increase the platelet counts. Clinical findings related to thrombocytopenia occurred in three (15.0%) neonates, but none of them presented with severe bleeding. There was no statistically significant association between neonatal lowest platelet count and maternal lowest platelet count, maternal platelet count at the time of delivery, and duration of thrombocytopenia, respectively. Neonates born to mothers with ITP have an increased tendency to develop thrombocytopenia, but severe bleeding is very rare in these neonates. Clinicians should pay special attention to follow these neonates. According to our results, both intravenous immunoglobulin and methyl prednisolone were found to be in equivalent efficacy for the treatment of neonatal thrombocytopenia due to maternal ITP. PMID:26258676

  6. Plasma microRNA profiling of pediatric patients with immune thrombocytopenic purpura.

    Science.gov (United States)

    Bay, Ali; Coskun, Enes; Oztuzcu, Serdar; Ergun, Sercan; Yilmaz, Fatih; Aktekin, Elif

    2014-06-01

    Immune thrombocytopenic purpura (ITP) is a commonly acquired autoimmune bleeding disorder in children. MicroRNAs (miRNAs) are small RNAs which are found in cells and circulation, and play a role in protein synthesis and regulation. In this study, we aimed to determine a biomarker for childhood ITP comparing the plasma miRNA levels of children having ITP with healthy children. A total of 86 patients with ITP and 56 healthy children followed up by the Department of Pediatric Hematology and Oncology in University of Gaziantep since July 2011 were enrolled in the study. The 86 patients with ITP were evaluated in two groups as 43 acute ITP (aITP) and 43 chronic ITP (cITP) patients. Plasma expression levels of 379 miRNAs were investigated by RT-PCR (quantitative RT-PCR) technique and they were compared between aITP, cITP, and control groups. For all miRNAs, the average of raw quantification cycle values of three groups separately in the analysis chip was accepted as the reference gene value, and normalization was done according to this value. Statistically significant differences were detected in seven miRNAs (miR-302c-3p, miR-483-5p, miR-410, miR-544a, miR-302a-3p, miR-223-3p, and miR-597) investigated between the groups with respect to the expression levels. The expression rates were found to be over 95% in miR-302c-3p and miR-483-5p, over 75% in miR-410, and over 40% in miR-544, miR-302a-3p, and miR-223-3p in all three groups. The detection of significant differences between plasma miRNA levels of aITP and cITP patients and healthy children may provide useful information in the prediction of the course of disease, determination of disease etiopathogenesis, and the development of new therapeutic modalities.

  7. Intracranial hemorrhage in acute and chronic childhood immune thrombocytopenic purpura over a ten-year period: an Egyptian multicenter study.

    Science.gov (United States)

    Elalfy, Mohsen; Elbarbary, Nancy; Khaddah, Normine; Abdelwahab, Magy; El Rashidy, Farida; Hassab, Hoda; Al-Tonbary, Youssef

    2010-01-01

    Intracranial hemorrhage (ICH) is a rare but major cause of death in immune thrombocytopenic purpura (ITP). The authors reviewed data of 1,840 patient with ITP, from 5 pediatric hematology centers in Egypt from 1997 to 2007, to study the incidence and risk factors of ICH. Ten cases of ICH were identified with a median age at presentation of 7.5 years; 4 patients had acute ITP, 2 persistent and 4 chronic. The platelet count was late referral to a specialized center. Our results suggest that treatment does not prevent ICH and that it can occur at any time during the course of the disease. Delayed referral can be considered a risk factor for unfavorable outcome of ICH, highlighting the importance of teaching sessions for patients and their parents to minimize subsequent morbidity and mortality of ICH in children with ITP. PMID:19955713

  8. Chronic idiopathic thrombocytopenic purpura in adult Chinese patients: a retrospective single-centered analysis of 1791 cases

    Institute of Scientific and Technical Information of China (English)

    LI Hong-qiang; ZHANG Lei; ZHAO Hui; JI Lin-xiang; YANG Ren-chi

    2005-01-01

    Background Adult chronic idiopathic thrombocytopenic purpura (ITP) is a common hematologic disease characterized by persistent thrombocytopenia. So far, there were only a few reports on adult Chinese patients with chronic ITP. This study aimed at defining the treatment outcome and prognostic factors for chronic ITP based on a large cohort of Chinese patients followed up for over 25 years at a single center.Methods The medical records of 1791 patients aged 14 years or older who were diagnosed as having chronic ITP at our hospital from 1974 to 1999 were retrospectively analyzed.Conclusions Adult Chinese chronic ITP patients can have long-term remission after steroid therapy and splenectomies. Primary steroid refractoriness is a prognostic factor predicting poor subsequent response to a splenectomy.

  9. Eltrombopag for the treatment of chronic immune or idiopathic thrombocytopenic purpura: a NICE single technology appraisal.

    Science.gov (United States)

    Boyers, Dwayne; Jia, Xueli; Jenkinson, David; Mowatt, Graham

    2012-06-01

    The National Institute for Health and Clinical Excellence (NICE) invited the manufacturer of eltrombopag (GlaxoSmithKline) to submit evidence for the clinical and cost effectiveness of this drug for the treatment of patients with chronic immune or idiopathic thrombocytopenic purpura (ITP), as part of the their Single Technology Appraisal (STA) process. The Aberdeen Technology Assessment Review (TAR) Group, commissioned to act as the evidence review group (ERG), critically reviewed and supplemented the submitted evidence. This paper describes the company submission, the ERG review and NICE's subsequent decisions. The ERG critically appraised the clinical and cost-effectiveness evidence submitted by the manufacturer, independently searched for relevant literature, conducted a critical appraisal of the submitted economic models and explored the impact of altering some of the key model assumptions as well as combining relevant sensitivity analyses. Three trials were used to inform the safety and efficacy aspects of this submission; however, one high-quality randomized controlled trial (RAISE study) was the principal source of evidence and was used to inform the economic model. Eltrombopag had greater odds of achieving the primary outcome of a platelet count between 50 × 10^⁹/L and 400 × 10^⁹/L during the 6-month treatment period than placebo (odds ratio [OR] 8.2, 99% CI 3.6, 18.7). In the eltrombopag group, 50/83 (60%) of non-splenectomized patients and 18/49 (37%) of splenectomized patients achieved this outcome. The median duration of response was 10.9 weeks for eltrombopag (splenectomized 6 and non-splenectomized 13.4) compared with 0 for placebo. Eltrombopag patients required less rescue medication and had lower odds of bleeding events for both the splenectomized and the non-splenectomized patients. For a watch-and-rescue strategy of care, the comparator was placebo and the ERG found that substantial reductions in the cost of eltrombopag are needed

  10. The Relationship between Self-esteem and Quality of Life of Patients with Idiopathic Thrombocytopenic Purpura at Isfahan's Sayed Al-Shohada Hospital, Iran, in 2013

    OpenAIRE

    Hemati, Zeinab; Kiani, Davood

    2016-01-01

    Background: Idiopathic thrombocytopenic purpura (ITP) is a chronic disease which is accompanied with hopelessness and loss of the sense of well-being due to its symptoms and treatment. It also affects patients' sense of social and spiritual well-being. This disorder decreases patients' self-esteem and their quality of life by changing their mental image and self-confidence. This study was performed to find the relationship between self-esteem and quality of life of patients with ITP. Subjects...

  11. [Detection, diagnosis and analysis of the first case of neonatal alloimmune thrombocytopenia purpura associated with anti-HPA-5b in China].

    Science.gov (United States)

    Zhou, Yan; Zhong, Zhou-Lin; Li, Li-Lan; Shen, Wei-Dong; Wu, Guo-Guang

    2014-04-01

    This study was aimed to investigate the detection and diagnosis of the neonatal alloimmune thrombocytopenia purpura (NAITP) caused by anti-HPA-5b antibody. The platelet count and clinical manifestation in the newborn were examined. The HPA-1-21bw genotypes of the newborn and her parents were detected by multiple-PCR and DNA sequencing. The HPA-specific antibody in the sera of newborn and her mother were detected and identified by flow cytometry (FCM) and monoclonal antibody-specific immobilization of platelet antigens (MAIPA). The results indicated that the clinical manifestations of the newborn were lighter. The HPA genotyping showed that the genotype of the newborn was HPA-5ab, while that of her mother and father were HPA-5aa and HPA-5ab, respectively. The antibody against the platelet of newborn's father existed in the newborn's mother sera. The HPA antibody of the mother was identified as anti-HPA-5b. It is concluded that the newborn with neonatal alloimmune thrombocytopenia purpura was caused by the antibody against HPA-5b.

  12. Partial splenic embolization combined with vincristine infusion for the treatment of refractory idiopathic thrombocytopenic purpura and Evans syndrome: observation of its long-term efficacy

    International Nuclear Information System (INIS)

    Objective: To observe the long-term efficacy of partial spleen embolization combined with vincristine infusion in treating refractory idiopathic thrombocytopenic purpura (ITP) and Evans syndrome. Methods: During the period of 2000-2007, partial spleen embolization together with vincristine infusion was carried out in 30 patients with refractory idiopathic thrombocytopenic purpura (n=24) or Evans syndrome (n=6). Vincristine infusion (2 mg) via splenic artery was performed before partial spleen embolization procedure. The long-term effectiveness was observed and analyzed. Results: One week after the treatment, the platelet count was increased from preoperative (10.23±8.28) × 109/L to (140.28±85.45) × 109/L in patients with ITP, while the platelet count was increased from preoperative (12±8) × 109/L to (210±60) × 109/L in patients with Evans syndrome. Meanwhile, the hemoglobin level showed an increase in different degrees, from preoperative (63.00±13.62) g/L to postoperative (123.00±13.14) g/L. The therapeutic effectiveness was 100%. During the follow-up time lasting for 3-5 years, recurrence was seen in 11 patients (36.7%) and the overall efficacy rate was 63.3%. Conclusion: For the treatment of refractory idiopathic thrombocytopenic purpura and Evans syndrome, partial spleen embolization combined with vincristine infusion carries reliable long-term efficacy. (author)

  13. Refractory thrombotic thrombocytopenic purpura associated with oral contraceptives and factor V Leiden: a case report

    Science.gov (United States)

    Tsirakis, George; Mantadakis, Elpis; Xylouri, Irini; Foudoulakis, Andreas; Vardaki, Eleftheria; Katsipi, Irene; Daphnis, Eugene; Samonis, George

    2009-01-01

    Introduction Thrombotic microangiopathies constitute a heterogeneous group of diseases characterised by microangiopathic haemolytic anaemia and thrombocytopaenia associated with platelet aggregation in the microcirculation responsible for ischaemic manifestations. Classically, thrombotic microangiopathies are described as encompassing two main syndromes: thrombotic thrombocytopaenic purpura and the haemolytic-uraemic syndrome Many cases of idiopathic thrombotic thrombocytopaenic purpura have, to date, been associated with severe ADAMTS13 metalloprotease deficiency while haemolytic uraemic syndrome usually occurs in the context of normal protease activity. Oestrogens and factor V Leiden have rarely been implicated in the pathogenesis of thrombotic microangiopathy. Case presentation We describe the case of a 17-year-old female with refractory thrombotic thrombocytopaenic purpura. The patient was receiving a new generation of oral contraceptives for dysmenorrhoea and had factor V Leiden. After undergoing prolonged and intense plasma exchange therapy for 40 days and high dose oral corticosteroids therapy for 90 days, our patient recovered fully. Conclusion Patients with refractory thrombotic thrombocytopaenic purpura should likely be evaluated for congenital thrombophilic disorders and for ingestion of drugs that have been associated with this rare form of thrombotic microangiopathy. Identification of these and as yet other unknown genetic and/or acquired risk factors may lead to more judicious treatment approaches. PMID:19829833

  14. Variant clinical courses in children with immune thrombocytopenic purpura: Sixteen year experience of a single medical center

    Directory of Open Access Journals (Sweden)

    Işın Yaprak

    2010-09-01

    Full Text Available Objective: Immune thrombocytopenic purpura (ITP is the most common cause of acquired thrombocytopenia in children. The objective of this study was to evaluate the presenting features, variation in the clinical courses, initial response rate to therapy, and long-term outcome in patients with ITP. Materials and Methods: Three hundred and fifty out of 491 newly diagnosed patients with ITP between the initial diagnosis ages of 6 months to 16 years were included in this retrospective, descriptive study. Patients with acute vs chronic ITP, acute vs recurrent ITP and chronic vs recurrent ITP were compared in terms of age at diagnosis, gender, initial platelet count, response rate to initial therapy, long-term outcome, and total duration of follow-up. Results: The clinical courses of the patients were determined as acute, chronic and recurrent in 63.8%, 29.1%, and 7.1%, respectively. Platelet count >20x109/L and initial diagnosis age >10 years were found to increase the probability of chronic outcome by at least two-fold. Conclusion: It is concluded that ITP in childhood is a common disease with low morbidity and mortality. In addition to the acute and chronic form, a rare recurrent form, which accounts for about 4-7% of all ITP patients, should be considered.

  15. Ticlopidine- and clopidogrel-associated thrombotic thrombocytopenic purpura (TTP): review of clinical, laboratory, epidemiological, and pharmacovigilance findings (1989–2008)

    Science.gov (United States)

    Zakarija, Anaadriana; Kwaan, Hau C.; Moake, Joel L.; Bandarenko, Nicholas; Pandey, Dilip K.; McKoy, June M.; Yarnold, Paul R.; Raisch, Dennis W.; Winters, Jeffrey L.; Raife, Thomas J.; Cursio, John F.; Luu, Thanh Ha; Richey, Elizabeth A.; Fisher, Matthew J.; Ortel, Thomas L.; Tallman, Martin S.; Zheng, X. Long; Matsumoto, Masanori; Fujimura, Yoshihiro; Bennett, Charles L.

    2012-01-01

    Thrombotic thrombocytopenic purpura (TTP) is a fulminant disease characterized by platelet aggregates, thrombocytopenia, renal insufficiency, neurologic changes, and mechanical injury to erythrocytes. Most idiopathic cases of TTP are characterized by a deficiency of ADAMTS13 (a disintegrin and metalloprotease, with thrombospondin-1-like domains) metalloprotease activity. Ironically, use of anti-platelet agents, the thienopyridine derivates clopidogrel and ticlopidine, is associated with drug induced TTP. Data were abstracted from a systematic review of English-language literature for thienopyridine-associated TTP identified in MEDLINE, EMBASE, the public website of the Food and Drug Administration, and abstracts from national scientific conferences from 1991 to April 2008. Ticlopidine and clopidogrel are the two most common drugs associated with TTP in FDA safety databases. Epidemiological studies identify recent initiation of anti-platelet agents as the most common risk factor associated with risks of developing TTP. Laboratory studies indicate that most cases of thienopyridine-associated TTP involve an antibody to ADAMTS13 metalloprotease, present with severe thrombocytopenia, and respond to therapeutic plasma exchange (TPE); a minority of thienopyridine-associated TTP presents with severe renal insufficiency, involves direct endothelial cell damage, and is less responsive to TPE. The evaluation of this potentially fatal drug toxicity can serve as a template for future efforts to comprehensively characterize other severe adverse drug reactions. PMID:19180126

  16. Does the site of platelet sequestration predict the response to splenectomy in adult patients with immune thrombocytopenic purpura?

    Science.gov (United States)

    Navez, Julie; Hubert, Catherine; Gigot, Jean-François; Navez, Benoit; Lambert, Catherine; Jamar, François; Danse, Etienne; Lannoy, Valérie; Jabbour, Nicolas

    2015-01-01

    Splenectomy is the only potentially curative treatment for chronic immune thrombocytopenic purpura (ITP) in adults. However, one-third of the patients relapse without predictive factors identified. We evaluate the predictive value of the site of platelet sequestration on the response to splenectomy in patients with ITP. Eighty-two consecutive patients with ITP treated by splenectomy between 1992 and 2013 were retrospectively reviewed. Platelet sequestration site was studied by (111)Indium-oxinate-labeled platelets in 93% of patients. Response to splenectomy was defined at last follow-up as: complete response (CR) for platelet count (PC) ≥100 × 10(9)/L, response (R) for PC≥30 × 10(9)/L and 100 versus <=100, 95% CI [0.025-0.493], p = 0.004) were significant predictors of recurrence-free survival in multivariate analysis. Response to splenectomy was independent of the site of platelet sequestration in patients with ITP. Pre-operative platelet sequestration study in these patients cannot be recommended. PMID:25275667

  17. Mathematical models for the study of the dynamics of indium-111-labelled platelets in idiopathic thrombocytopenic purpura

    Energy Technology Data Exchange (ETDEWEB)

    Savolainen, S.

    1992-01-01

    Platelet kinetics in patients with idiopathic thrombocytopenic purpura (ITP) was investigated by applying various models (compartmental and open models, and functional and uptake analyses) to data on indium-111 labelled platelets monitored with a gamma camera following intravenous injection of labelled platelets. The usefulness of the selected models was tested by relating kinetic data to pathophysiological phenomena. A comparison of the results of platelet and colloid kinetics showed that the splenic platelet kinetics in ITP patients does not seem to be primarily dependent on the reticuloendothelial system. Although closed three-compartmental analysis seemed to be superior to the other models applied, none of the methods of analysis tested in this study appears to provide a complete description of short-lived platelet dynamics, as for every model certain assumptions that are not entirely relevant have to be made; this stresses the importance of combining various methods for a comprehensive analysis of a complex phenomenon. Conclusions concerning the function of biological systems should be based on more than one dynamic model or calculation method, since applying only one model (or calculation method) may give artifactual results due to poor statistics of observed data or to inexactness of the assumptions concerning the model.

  18. Idiopathic thrombocytopenic purpura treatment of 80 cases%特发性血小板减少性紫癜80例治疗体会

    Institute of Scientific and Technical Information of China (English)

    王秋红

    2011-01-01

    目的 总结成人特发性血小板减少性紫癜(Idiopathic thromb0cytopeniC purpura,ITP)患者的临床特点.方法 对80例ITP患者的临床资料进行回顾性分析.结果 80例ITP患者经治疗后全部有效(100%),其中显效68例(85%),良效6例(7.5%),进步7例(7.5%).结论 ITP好发于中青年女性,多数对PRED治疗有反应,无反应者改用VCR多数仍可有反应,IVIG治疗疗效显著.%Objective To summarize the adult idiopathic thrombocytopenic purpura (idiopathic thrombocytopenic purpura, ITP) in patients with clinical features. Methods Totally 80 cases of ITP patients were retrospectively analyzed. Results Totally 80 ITP patients were all effective after treatment (100% ) , excellent in 68 cases( 85% ) ,good in 6 cases(7. 5% ) ,progress in 7 cases(7. 5% ) . Conclusions ITP occurs in young women, most of the PRED response to treatment, the majority of non - responders to switch to VCR can respond, IVIG therapy significantly.

  19. Rituximab in a child with autoimmune thrombotic thrombocytopenic purpura refractory to plasma exchange.

    Science.gov (United States)

    Narayanan, Parameswaran; Jayaraman, Aparna; Rustagi, Rashi S; Mahadevan, S; Parameswaran, Sreejith

    2012-07-01

    A nine-year-old girl presented with headache, purpura and mild left hemiparesis. Laboratory evaluation revealed thrombotic microangiopathy with ADAMTS13 deficiency, with auto-antibodies to ADAMTS13. She was treated with plasma exchange and steroids, following which she improved transiently, relapsing within 2 months. The relapse was refractory to conventional therapy and rituximab was tried. She had good response to rituximab and has been in remission for the past 12 months. Rituximab may be a promising option for children with acquired TTP refractory to plasma exchange and steroids.

  20. Thrombotic thrombocytopenic purpura in a postoperative patient taking cephalexin responding to plasmapheresis: A case report and review of cephalosporin-induced TTP.

    Science.gov (United States)

    Patel, Ritu; Amber, Kyle T

    2016-10-01

    The clinical presentation of thrombotic thrombocytopenic purpura (TTP) is often atypical delaying diagnosis and treatment. A number of drugs have been implicated in the development of TTP, including cyclosporine, tacrolimus, clopidogrel, and quinine. To our knowledge, only three cases of cephalosporin-induced TTP have been described, with two of these cases occurring with these use of cephalexin. We herein describe a case of TTP occurring in a postoperative patient taking cephalexin, requiring plasmapheresis. Following plasmapheresis, the patient's mental status and platelet count significantly improved. J. Clin. Apheresis 31:473-475, 2016. © 2015 Wiley Periodicals, Inc. PMID:26274019

  1. Randomized trial comparing intravenous immunoglobulin with methylprednisolone pulse therapy in acute idiopathic thrombocytopenic purpura. Danish I.T.P. Study Group.

    Science.gov (United States)

    Rosthøj, S; Nielsen, S; Pedersen, F K

    1996-08-01

    Forty-three children with newly diagnosed idiopathic thrombocytopenic purpura (ITP), platelet count (PC) below 20 x 10(9)l-1, and either continued bleeding or failure to show a spontaneous rise in the PC after a 3 day observation period were randomized to treatment with either intravenous immunoglobulin (IVIG) infusions 1 g kg-1 (n = 23) or intravenous methylprednisolone pulse therapy (MPPT) 30 mg kg-1 (n = 20) on two consecutive days. After 72 h, IVIG had induced greater platelet responses (mean PC 188 x 10(9) versus 77 x 10(9)l-1, 2p MPPT as the initial treatment for ITP. PMID:8863869

  2. TXRF analysis of low Z elements in serum of patients with idiopathic thrombocytopenic purpura using X-ray fluorescence

    Energy Technology Data Exchange (ETDEWEB)

    Canellas, Catarine G.L.; Leitao, Roberta G.; Lopes, Ricardo T., E-mail: catarine@lin.ufrj.b, E-mail: ricardo@lin.ufrj.b [Universidade Federal do Rio de Janeiro (PEN/COPPE/UFRJ), RJ (Brazil). Coordenacao dos Programas de Pos-Graduacao de Engenharia. Lab. de Instrumentacao Nuclear; Carvalho, Silvia M.F., E-mail: silvia@hemorio.rj.gov.b [State Institute of Hematology Arthur de Siqueira Cavalcanti (HEMORIO), Rio de Janeiro, RJ (Brazil); Bellido, Alfredo Victor B., E-mail: alfredo@ien.gov.b [Federal Fluminense University (UFF), Niteroi, RJ (Brazil). Chemistry Inst.; Anjos, Marcelino J., E-mail: marcelin@lin.ufrj.b [State University of Rio de Janeiro (UERJ), RJ (Brazil). Physics Inst.

    2011-07-01

    Idiopathic thrombocytopenic purpura (ITP) is a blood disorder characterized by an abnormal decrease in the number of platelets in the blood. ITP results from development of an antibody directed against a structural platelet antigen (an autoantibody). Platelets are also called thrombocytes, meaning cells that form clots. The cause of ITP is not known and their diagnosis requires that other disorders be excluded through selective tests. In this work, forty patients suffering from ITP and sixty healthy volunteers (Control Group) were analyzed. All the serum samples had been collected from people who live in the urban area of Rio de Janeiro City/Brazil. Blood was collected into vacutainers without additives. The measurements were performed at the X-ray fluorescence beamline at Brazilian National Synchrotron Light Laboratory (LNLS), in Campinas, Sao Paulo using a monochromatic beam with maximum energy of 20 keV for the excitation and an Ultra-LEGe detector with resolution of 148 eV at 5.9 keV. Standard solutions with Vanadium as internal standard were prepared for calibration system. It was possible to determine the elemental concentrations of the following six elements: Na, P, S, Cl, K and Ca. The Student's t-test was used to analyze significant differences ({alpha} = 0.05) between group of patients with ITP and control group. The elements that presented significant differences for the mean of their concentrations between each one of the ITP group and control group in {mu}g.g-1 were: phosphorous (136{+-}12 and 92{+-}12), Sulphur (1077{+-}97 and 847{+-}80), Chlorine (2905{+-}385 and 2266{+-}378), Potassium (137{+-}118 and 82{+-}15) and Calcium (64{+-}7 and 44{+-}6) respectively. These results will help the biomedical field with regard to early diagnosis and improved medical treatment. Thus, our findings indicate that these elements can be related to the important biochemical processes in ITP. (author)

  3. FATAL OUTCOME OF INFECTION BY DENGUE 4 IN A PATIENT WITH THROMBOCYTOPENIC PURPURA AS A COMORBID CONDITION IN BRAZIL

    Directory of Open Access Journals (Sweden)

    Frederico Figueiredo Amâncio

    2014-06-01

    Full Text Available Dengue is currently a major public-health problem. Dengue virus (DENV is classified into four distinct serotypes, DENV 1-4. After 28 years of absence, DENV-4 was again detected in Brazil in 2010 in Roraima State, and one year later, the virus was identified in the northern Brazilian states of Amazonas and Pará, followed by Rio de Janeiro and São Paulo. In Minas Gerais, the first confirmed case of DENV-4 occurred in the municipality of Frutal in 2011 and has now been isolated from a growing number of patients. Although DENV-2 is associated with the highest risk of severe forms of the disease and death due to the infection, DENV-4 has also been associated with severe forms of the disease and an increasing risk of hemorrhagic manifestations. Herein, the first fatal case of confirmed DENV-4 in Brazil is reported. The patient was an 11-year-old girl from the municipality of Montes Claros in northern Minas Gerais State, Brazil. She had idiopathic thrombocytopenic purpura as a comorbid condition and presented with a fulminant course of infection, leading to death due to hemorrhagic complications. Diagnosis was confirmed by detection of Dengue-specific antibodies using IgM capture enzyme-linked immunosorbent assay and semi-nested RT-PCR. Primary care physicians and other health-care providers should bear in mind that DENV-4 can also result in severe forms of the disease and lead to hemorrhagic complications and death, mainly when dengue infection is associated with coexisting conditions.

  4. The D173G mutation in ADAMTS-13 causes a severe form of congenital thrombotic thrombocytopenic purpura

    KAUST Repository

    Lancellotti, S.

    2015-08-13

    Congenital thrombotic thrombocytopenic purpura (TTP) is a rare form of thrombotic microangiopathy, inherited with autosomal recessive mode as a dysfunction or severe deficiency of ADAMTS-13 (A Disintegrin And Metalloprotease with ThromboSpondin 1 repeats Nr. 13), caused by mutations in the ADAMTS-13 gene. About 100 mutations of the ADAMTS-13 gene were identified so far, although only a few characterised by in vitro expression studies. A new Asp to Gly homozygous mutation at position 173 of ADAMTS-13 sequence was identified in a family of Romanian origin, with some members affected by clinical signs of TTP. In two male sons, this mutation caused a severe (< 3 %) deficiency of ADAMTS-13 activity and antigen level, associated with periodic thrombocytopenia, haemolytic anaemia and mild mental confusion. Both parents, who are cousins, showed the same mutation in heterozygous form. Expression studies of the mutant ADAMTS-13, performed in HEK293 cells, showed a severe decrease of the enzyme’s activity and secretion, although the protease was detected inside the cells. Molecular dynamics found that in the D173G mutant the interface area between the metalloprotease domain and the disintegrin-like domain significantly decreases during the simulations, while the proline-rich 20 residues linker region (LR, 285–304) between them undergoes extensive conformational changes. Inter-domain contacts are also significantly less conserved in the mutant compared to the wild-type. Both a decrease of the inter-domain contacts along with a substantial conformational rearrangement of LR interfere with the proper maturation and folding of the mutant ADAMTS-13, thus impairing its secretion.

  5. A murine model for human immune thrombocytopenic purpura and comparative analysis of multiple gene expression in bone marrow and spleen

    Institute of Scientific and Technical Information of China (English)

    Hong Wei; Xinchun Ding; Jiangong Ren; Ka Liu; Pingping Tan; Daquan Li; Runlin Z.Ma

    2008-01-01

    Homeostasis of platelet number in human and other mammals is well maintained for prevention of minor bleeding and for other im-munological functions, but the exact molecular mechanism responsible for immune thrombocytopenic purpura (ITP) has not been fullyunderstood. In an effort to identify genetic factors involved in initiation of platelet production in response to bleeding injury or plateletdestruction, we have successfully generated an animal model of human ITP via intraperitoneal injection of anti-platelet antibody into theBalb/c mouse. Platelet counts were dropped dramatically in animals that received antibody injection within 4 h, maintained at the mini-mum level for a period of 44 h, started to rebound after 48 h, and reached to the maximum at 144 h (6 days). Final homeostasis reached atapproximately 408 h (17 days), following a minor cycle of platelet number fluctuation. Using semi-quantitative RT-PCR, we assessed andcompared mRNA level of CD41, c-myb, c-mpl, caspase-3, caspase-9, GATA-1, and Bcl-xl in bone marrow and spleen. Alteration ofmRNA expression was correlated with the change of platelet level, and an inverse relationship was found for expression of the genes be-tween bone marrow and spleen. No transcription was detectable for any of the seven genes in bone marrow at the time when plateletnumber reached the maximum (144 h). In contrast, mRNA transcripts of the seven genes were found to be at the highest level in spleentissue. This is the first study of simultaneous detection of multiple platelet related genes in a highly reproducible ITP animal model. Ourresults provided the supportive evidence that expression of the above seven genes are more related to negative regulation of plateletnumber in spleen tissue, at least in the model animals.

  6. Generation of Anti-Murine ADAMTS13 Antibodies and Their Application in a Mouse Model for Acquired Thrombotic Thrombocytopenic Purpura.

    Science.gov (United States)

    Deforche, Louis; Tersteeg, Claudia; Roose, Elien; Vandenbulcke, Aline; Vandeputte, Nele; Pareyn, Inge; De Cock, Elien; Rottensteiner, Hanspeter; Deckmyn, Hans; De Meyer, Simon F; Vanhoorelbeke, Karen

    2016-01-01

    Thrombotic thrombocytopenic purpura (TTP) is a life-threatening thrombotic microangiopathy linked to a deficiency in the metalloprotease ADAMTS13. In the current study, a novel mouse model for acquired TTP was generated to facilitate development and validation of new therapies for this disease. Therefore, a large panel (n = 19) of novel anti-mouse ADAMTS13 (mADAMTS13) monoclonal antibodies (mAbs) of mouse origin was generated. Inhibitory anti-mADAMTS13 mAbs were identified using the FRETS-VWF73 assay. Four mAbs strongly inhibited mADAMTS13 activity in vitro (∼68-90% inhibition). Injecting a combination of 2 inhibitory mAbs (13B4 and 14H7, 1.25 mg/kg each) in Adamts13+/+ mice resulted in full inhibition of plasma ADAMTS13 activity (96 ± 4% inhibition, day 1 post injection), leading to the appearance of ultra-large von Willebrand factor (UL-VWF) multimers. Interestingly, the inhibitory anti-mADAMTS13 mAbs 13B4 and 14H7 were ideally suited to induce long-term ADAMTS13 deficiency in Adamts13+/+ mice. A single bolus injection resulted in full ex vivo inhibition for more than 7 days. As expected, the mice with the acquired ADAMTS13 deficiency did not spontaneously develop TTP, despite the accumulation of UL-VWF multimers. In line with the Adamts13-/- mice, TTP-like symptoms could only be induced when an additional trigger (rVWF) was administered. On the other hand, the availability of our panel of anti-mADAMTS13 mAbs allowed us to further develop a sensitive ELISA to detect ADAMTS13 in mouse plasma. In conclusion, a novel acquired TTP mouse model was generated through the development of inhibitory anti-mADAMTS13 mAbs. Consequently, this model provides new opportunities for the development and validation of novel treatments for patients with TTP. In addition, these newly developed inhibitory anti-mADAMTS13 mAbs are of great value to specifically study the role of ADAMTS13 in mouse models of thrombo-inflammatory disease. PMID:27479501

  7. Successful treatment of sepsis-induced disseminated intravascular coagulation in a patient with idiopathic thrombocytopenic purpura using recombinant human soluble thrombomodulin.

    Science.gov (United States)

    Koga, Tomohiro; Inoue, Daisuke; Okada, Akitomo; Aramaki, Toshiyuki; Yamasaki, Satoshi; Nakashima, Munetoshi; Kawakami, Atsushi; Eguchi, Katsumi

    2011-12-01

    Disseminated intravascular coagulation (DIC) may complicate a variety of disorders that contribute to mortality, particularly those related to bleeding. It is therefore very difficult to manage DIC in patients with known bleeding disorders. We treated a 62-year-old woman with idiopathic thrombocytopenic purpura (ITP) complicated with sepsis-induced DIC. She had been diagnosed with ITP 8 months prior to admission. Laboratory tests showed an elevation of d-dimer and endotoxin, while pyelonephritis was shown by abdominal computed tomography. Escherichia coli was detected by blood culture. Based on these findings, the patient was diagnosed with sepsis-induced DIC due to urinary tract infection. Thrombocytopenia was refractory despite the use of antibiotics and platelet transfusion, but it was promptly improved in response to recombinant human soluble thrombomodulin (rTM). We suggest that rTM provides a new therapeutic strategy for DIC patients with high hemorrhagic risk.

  8. [A Case of Thrombotic Thrombocytopenic Purpura in a Patient Undergoing FOLFOX6 plus Panitumumab Therapy for Unresectable Recurrent Rectal Cancer with a Rapidly Progressive Course].

    Science.gov (United States)

    Kato, Kuniyuki; Michishita, Yoshihiro; Oyama, Kenichi; Hatano, Yoshiaki; Nozawa, Tatsuru; Ishibashi, Masahisa; Konda, Ryuichiro; Sasaki, Akira

    2016-01-01

    A 71-year-old male patient began FOLFOX6 plus panitumumab treatment for unresectable recurrent rectal cancer. He developed thrombocytopenia after 2 courses of treatment and therefore a platelet transfusion was performed. The day after transfusion, the patient developed jaundice and hematuria. His lactate dehydrogenase levels had increased and a peripheral blood smear review revealed the presence of schistocytes. Anti-ADAMTS13 antibodies were present, and there was a reduction in ADAMTS13 activity. The patient was diagnosed with thrombotic thrombocytopenic purpura and treated with a plasma exchange. The day after the plasma exchange, his clinical condition rapidly worsened and he died. Thrombocytopenia due to chemotherapy often appears as myelosuppression. If conditions such as jaundice, indirect bilirubinemia, or hematuria appear during the course of chemotherapy, this condition must be considered as a differential diagnosis. PMID:26809542

  9. Anti-D (WinRho SD) treatment of children with chronic autoimmune thrombocytopenic purpura stimulates transient cytokine/chemokine production.

    Science.gov (United States)

    Semple, J W; Allen, D; Rutherford, M; Woloski, M; David, M; Wakefield, C; Butchart, S; Freedman, J; Blanchette, V

    2002-03-01

    Intravenous anti-D is often used in the treatment of autoimmune thrombocytopenic purpura (AITP), but little is known about its mechanisms of action. To investigate anti-D's potential in vivo mechanism(s) of action, a small group (N = 7) of children with chronic AITP was studied. The children initially received either 25 or 50 microg/kg of WinRho-SD in a four-cycle cross-over trial, and peripheral blood samples from the first and third cycles were assessed for cytokine levels at pre-treatment, 3 hr, 1 day, and 8 days post-treatment. Results showed that platelet counts significantly increased in all the children by day 8 post-treatment. Analysis of serum by ELISA showed that there was a significant but transient rise in both pro- and anti-inflammatory cytokine/chemokine levels (e.g., IL1RA, IL6, GM-CSF, MCP-1 alpha, TNF-alpha and MCP-1) by 3 hr post-treatment in both cycles which returned to baseline levels by 8 days post-treatment. These results suggest that anti-D administration may initially activate the RES in the form of cytokine/chemokine secretion, which is subsequently followed by an increase in platelet counts. It is possible that the induced cytokine/chemokine storm may have an effect on several physiological processes such as those mediating either adverse effects or potentially RES phagocytic activity. PMID:11891813

  10. Thrombotic thrombocytopenic purpura following salvage chemotherapy with paclitaxel, ifosfamide and cisplatin in a patient with a refractory germ cell tumor: A case report and review of the literature

    Science.gov (United States)

    ULAS, ARIFE; SILAY, KAMILE; AKINCI, SEMA; AKINCI, MUHAMMED BULENT; SENDUR, MEHMET ALI; DEDE, DIDEM SENER; POLAT, YUNUS HALIL; YALCIN, BULENT

    2015-01-01

    Thrombotic thrombocytopenic purpura (TTP) is a rare form of thrombotic microangiopathy that is characterized by microvascular thrombosis, thrombocytopenia, hemolysis and end organ damage. An extensive variety of drugs, including certain chemotherapeutic agents, have been associated with TTP. However, paclitaxel, cisplatin and ifosfamide regimen (TIP)-induced TTP has not previously been described. The present study reports the case of a 43-year-old patient with a refractory testicular germ cell tumor who developed acute TTP during TIP chemotherapy. Following the third cycle of TIP chemotherapy, the patient developed fever, anemia, thrombocytopenia and confusion. A diagnosis of TTP was established. Plasmapheresis was initiated as daily treatment in the first week, then continued every other day for 4 weeks. TIP chemotherapy was discontinued. The patient's clinical and neurological symptoms improved markedly after a week. Renal function and hemolysis improved, and the patient was discharged in a stable condition. The patient did not develop any complications and has been in remission for 5 months. The Naranjo adverse drug reaction probability scale indicated a likely association between TTP and the TIP chemotherapy regimen in this patient. This case is also investigated with regard to the associated literature to increase the awareness of TTP following chemotherapy. PMID:26622823

  11. Ticlopidine-, Clopidogrel-, and Prasugrel-Associated Thrombotic Thrombocytopenic Purpura: A 20-Year Review from the Southern Network on Adverse Reactions (SONAR)

    Science.gov (United States)

    Jacob, Sony; Dunn, Brianne L.; Qureshi, Zaina P.; Bandarenko, Nicholas; Kwaan, Hau C.; Pandey, Dilip K.; McKoy, June M.; Barnato, Sara E.; Winters, Jeffrey L.; Cursio, John F.; Weiss, Ivy; Raife, Thomas J.; Carey, Patricia M.; Sarode, Ravindra; Kiss, Joseph E.; Danielson, Constance; Ortel, Thomas L.; Clark, William F.; Rock, Gail; Matsumoto, Masanori; Fujimura, Yoshihiro; Zheng, X. Long; Chen, Hao; Chen, Fei; Armstrong, John M.; Raisch, Dennis W.; Bennett, Charles L.

    2013-01-01

    Thienopyridine-derivatives (ticlopidine, clopidogrel, and prasugrel) are the primary antiplatelet agents. Thrombotic thrombocytopenic purpura (TTP) is a rare drug-associated syndrome, with the thienopyridines being the most common drugs implicated in this syndrome. We reviewed 20 years of information on clinical, epidemiologic, and laboratory findings for thienopyridine-associated TTP. Four, 11, and 11 cases of thienopyridine-associated TTP were reported in the first year of marketing of ticlopidine (1989), clopidogrel (1998), and prasugrel (2010), respectively. As of 2011, the FDA received reports of 97 ticlopidine-, 197 clopidogrel-, and 14 prasugrel-associated TTP cases. Severe deficiency of ADAMTS-13 (a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13) was present in 80% and antibodies to 100% of these TTP patients on ticlopidine, 0% of the patients with clopidogrel-associated TTP (p < 0.05), and an unknown percentage of patients with prasugrel-associated TTP. TTP is associated with use of each of the three thienopyridines, although the mechanistic pathways may differ. PMID:23111862

  12. Recurrent Thrombotic Thrombocytopenic Purpura-Like Syndrome as a Paraneoplastic Phenomenon in Malignant Peritoneal Mesothelioma: A Case Report and Review of the Literature

    Directory of Open Access Journals (Sweden)

    Francisco Socola

    2012-01-01

    Full Text Available We report the case of an African American male with no significant past medical history presenting with recurrent, rapidly relapsing episodes of thrombotic thrombocytopenic purpura (TTP despite aggressive treatment with several lines of treatment. Incidentally, these episodes were associated with severe abdominal pain which eventually developed into acute abdomen and prompted exploratory laparotomy, revealing diffuse carcinomatosis with a tumor located on the left pelvis that was encasing the distal sigmoid colon. Pathology made a final diagnosis of peritoneal mesothelioma. TTP-like syndrome (TTP-LS has been described as a paraneoplastic phenomenon in several malignancies but never before in the setting of malignant mesothelioma. Paraneoplastic TTP-like syndrome has historically been associated with a dismal prognosis and particular clinical and laboratory abnormalities described in this paper. It is of utmost importance to make a prompt determination whether TTP is idiopathic or secondary to an underlying condition because of significant differences in their prognosis, treatment, and response. This paper also reviews the current literature regarding this challenging condition.

  13. Dieulafoy Lesion in the Ascending Colon Presenting with Gastrointestinal Bleeding and Severe Anemia Complicated by a Coexisting Severe Resistant Chronic Idiopathic Thrombocytopenic Purpura

    Directory of Open Access Journals (Sweden)

    Sherif Ali Eltawansy

    2014-01-01

    Full Text Available Background. GI (gastrointestinal bleeding can be due to a variety of etiologies ranging from being common like bleeding peptic ulcer disease or esophageal varices. One of the rarely documented causes is the Dieulafoy lesion which is known as an abnormally large ectatic artery that penetrates the gut wall, occasionally eroding through the mucosa causing massive bleeding. In addition to that, we refer to the uncommon presentation of Dieulafoy lesion itself as it is well known to be found in the stomach, esophagus, duodenum, and jejunum but not the ascending colon as in our case. The patient had a coexisting ITP (idiopathic thrombocytopenic purpura that was resistant to different therapies. Case Report. We report a case of a 48-year-old Egyptian female known for chronic ITP resistant to treatment. The patient presented with bright red bleeding per rectum and severe life threatening anemia. Endoscopic study showed a Dieulafoy lesion. Endoscopic clipping was successful in controlling the bleeding. Conclusion. Dieulafoy lesion is a rare reason for GI bleeding and can present in common or unexpected places. Also extreme caution should be used in patients with bleeding tendency due to different reasons, like ITP in our case.

  14. Veltuzumab, an anti-CD20 mAb for the treatment of non-Hodgkin's lymphoma, chronic lymphocytic leukemia and immune thrombocytopenic purpura.

    Science.gov (United States)

    Milani, Cannon; Castillo, Jorge

    2009-04-01

    Veltuzumab is a humanized, second-generation anti-CD20 mAb currently under development by Immunomedics Inc for the potential treatment of B-cell non-Hodgkin's lymphoma (NHL) and chronic lymphocytic leukemia (CLL). Licensee Nycomed is developing veltuzumab for the potential treatment of rheumatoid arthritis and immune thrombocytopenic purpura (ITP). Veltuzumab contains 90 to 95% human antibody sequences with identical antigen framework regions to epratuzumab (a humanized anti-CD22 mAb) and similar antigen-binding determinants to rituximab (chimeric, anti-CD20 mAb and the first-line treatment of aggressive and indolent NHL). In vitro studies have demonstrated that veltuzumab has enhanced binding avidities and a stronger effect on complement-dependent cytotoxicity compared with rituximab in selected cell lines. In dose-finding phase I/II clinical trials in patients with low-grade NHL, intravenous veltuzumab demonstrated a substantial rate of complete responses in concurrence with shorter and more tolerable infusions compared with rituximab. Currently there has been no evidence of an immune response to repeated administrations, and no serious adverse events related to veltuzumab treatment in patients with NHL. Veltuzumab is undergoing clinical trials using a low-dose subcutaneous formulation in patients with NHL, CLL and ITP. Prospective, randomized clinical trials are needed to clarify the role veltuzumab will play in a market where the therapy of B-cell lymphoproliferative disorders is dominated by rituximab. PMID:19330725

  15. Impact of chronic Immune Thrombocytopenic Purpura (ITP on health-related quality of life: a conceptual model starting with the patient perspective

    Directory of Open Access Journals (Sweden)

    George James N

    2008-02-01

    Full Text Available Abstract Background Immune thrombocytopenic purpura (ITP, a condition characterized by autoimmune-mediated platelet destruction and suboptimal platelet production, is associated with symptoms such as bruising, epistaxis, menorrhagia, mucosal bleeding from the gastrointestinal and urinary tracts and, rarely central nervous system bleeding. The aim of this research is to develop a conceptual model to describe the impact of ITP and its treatment on patients' health-related quality of life (HRQoL. Methods A literature search and focus groups with adult ITP patients were conducted to identify areas of HRQoL affected by ITP. Published literature was reviewed to identify key HRQoL issues and existing questionnaires used to assess HRQoL. Focus group transcripts were reviewed, and common themes were extracted by grouping conceptual categories that described the impact on HRQoL. Results The literature synthesis and themes from the focus group data suggest that decreased platelet counts, disease symptoms, and treatment side effects influence multiple domains of HRQoL for ITP patients. Key areas affected by ITP and its treatments include emotional and functional health, work life, social and leisure activities, and reproductive health. Conclusion ITP affects various areas of HRQoL. This conceptual model will help inform the evaluation of therapeutic strategies for ITP.

  16. DNA methyltransferase 3B (DNMT3B -579G>T) promotor polymorphism and the susceptibility to pediatric immune thrombocytopenic purpura in Egypt.

    Science.gov (United States)

    Khorshied, Mervat Mamdooh; El-Ghamrawy, Mona Kamal

    2012-12-10

    Idiopathic thrombocytopenic purpura (ITP) is an autoimmune disease characterized by increased platelet destruction. Although the etiology of ITP remains unclear, it is accepted that both environmental and genetic factors play an important role in the development of the disease. The present study aimed at exploring a novel molecular determinant that may influence the susceptibility and course of ITP in Egyptian children. To achieve our aim, genotyping of DNMT3B -579G>T promotor polymorphism by polymerase chain reaction restriction fragment length polymorphism (PCR-RFLP) assay. The current study was conducted on 140 ITP patients and 150 age and gender matched healthy controls. The results obtained revealed that DNMT3B -579 TT homotype was significantly higher in ITP patients and conferred almost three fold increased risk of ITP (OR=3.16, 95%CI=1.73-5.79). There was no statistically significant difference between ITP patients with wild or mutant genotypes as regards their clinical or laboratory data. Furthermore, there was no statistical difference in the distribution of DNMT3B -579G>T genotypes between acute and chronic ITP patients. In conclusion, DNMT3B -579G>T promotor polymorphism represents a novel genetic risk factor for ITP but not a predictor for tendency to chronicity in pediatric ITP in Egypt.

  17. Rituximab responsive immune thrombocytopenic purpura in an adult with underlying autoimmune lymphoproliferative syndrome due to a splice-site mutation (IVS7+2 T>C) affecting the Fas gene

    OpenAIRE

    Wei, Andrew; Cowie, Tiffany

    2007-01-01

    A 36 yr-old man of Israeli descent with a history of childhood splenectomy for severe thrombocytopenia and a family history of autoimmune lymphoproliferative syndrome (ALPS), presented with severe immune thrombocytopenic purpura refractory to standard therapy. He was found to possess a heterozygous mutation in the Fas gene (also termed TNFRSF6, CD95, Apo-1) affecting the donor splice site of intron 7 (IVS7+2 T>C). This frameshift mutation truncates the cytoplasmic domain of the Fas death rece...

  18. Efficacy, safety, and dose response of intravenous anti-D immune globulin (WinRho SDF) for the treatment of idiopathic thrombocytopenic purpura in children.

    Science.gov (United States)

    Freiberg, A; Mauger, D

    1998-01-01

    We analyzed data from 20 children treated for acute or chronic idiopathic (immune) thrombocytopenic purpura (ITP) at a single institution to determine the relationship between dose of intravenous anti-D immune globulin (WinRho SDF; Nabi, Boca Raton, FL), increase in platelet count, and decrease in hemoglobin in the therapy of ITP. Higher doses of anti-D were clearly associated with a greater therapeutic response in the platelet count, with no increase in hemolysis for both acute and chronic ITP. A significant correlation was found between dose and peak increase in platelet count measured in the 14 days following administration. This effect was present for both acute ITP (17 infusions, P = .0001) and chronic ITP (30 infusions, P = .038). Although hemolysis was seen in nearly all infusions, with a median hemoglobin fall of 1.9 g/dL (range, 0 to 4.2), the decrease in hemoglobin was greater than 2.5 for only three infusions, and the largest fall in hemoglobin (4.2) was in a child with an underlying hemolytic anemia. Furthermore, for both acute and chronic ITP there was no relationship between the decrease in hemoglobin and the dose given (P = .22), nor between the increase in platelet count and fall in hemoglobin (P = .27). This analysis supports the use of higher doses of anti-D for the treatment of ITP, and demonstrates the need for a trial of high-dose anti-D (>100 microg/kg) in acute and chronic ITP. PMID:9523746

  19. Evaluation of the effects of and earliest response rate to anti-D treatment in children with chronic idiopathic thrombocytopenic purpura: a pilot study.

    Science.gov (United States)

    Yetgin, Sevgi; Aytaç, Selin; Olcay, Lale; Tunç, Bahattin; Ozbek, Namik; Aydinok, Yeşim

    2010-01-01

    In this pilot study, 30 (14 male, 16 female; median age: 8 years, range: 2-18) chronic non-splenectomized idiopathic thrombocytopenic purpura (ITP) patients with Rh+ blood group and their 49 attacks were evaluated after intravenous (i.v.) anti-D (WinRho SDF, Cangene Corporation, Winnipeg, MB, Canada) treatment at a dose of 50 microg/kg x 3 days (n = 21 cases; 35 attacks) or a single dose of 75 microg/kg (n = 9 cases; 14 attacks) to define the hemostatic dose of anti-D. Five of 30 patients (22/49 attacks) were resistant to steroid, intravenous immunoglobulin (IVIG) and vincristine treatment. Hemoglobin (Hb), white blood cells (WBC), platelets (plt) and reticulocytes (ret) were evaluated before and after treatment during the follow-up in sequences on the 1st, 7th, 14th and 21st days after anti-D treatment if the patients had no symptom. All patients, even the resistant ones, experienced an increase in plt count to provide protection from bleeding (> or = 20 x 10(9)/L in patients with symptoms, > or = 10 x 10(9)/L in patients without symptoms). The plt responses of one resistant and five non-resistant patients treated with a single 75 microg/kg dose of i.v. anti-D in 8 attacks were monitored at the 2nd, 4th, 8th, 24th and 48th hours of the treatment. A protective plt level was attained within 2 hours in 6 attacks of five non-resistant cases and in 24 hours in the remaining 2 attacks of one resistant case. This pilot study suggests that anti-D treatment in ITP patients is effective and can increase plt to a level adequate enough to protect from hemorrhage within 2 hours, when given in a 75 microg/kg dose. A few adverse events (i.e. chills, hemolysis and hemoglobinuria) resolved without intervention. PMID:20560246

  20. Púrpura trombocitopênica trombótica associada à gravidez: relato de caso Thrombotic thrombocytopenic purpura associated with pregnancy: case report

    Directory of Open Access Journals (Sweden)

    Edson Silva Marques Filho

    2009-08-01

    Full Text Available A púrpura trombocitopênica trombótica (PTT é uma entidade rara em pacientes críticos. Relatamos um caso clínico de paciente gestante admitida em unidade de terapia intensiva obstétrica com quadro de alteração de sensório, atribuído inicialmente à doença hipertensiva da gravidez. Evoluiu com piora do quadro geral caracterizada por anemia e plaquetopenia grave, suscitando a investigação diagnóstica de púrpura trombocitopênica trombótica após o reconhecimento do perfil hematológico. Os autores enfatizam a importância do conhecimento da doença como marcador de prognóstico para pacientes obstétricas, em vista da semelhança com outras patologias comuns ao ciclo gravídico-puerperal e o fato do diagnóstico e tratamento precoce serem determinantes para o desfecho.Case report of a patient with 37-week gestational age admitted to an obstetric intensive care unit with an altered level of consciousness, related primarily to the pregnancy-induced hypertension. The patient presented a worsening clinical course characterized by, anemia and severe thrombocytopenia, Investigation led to a diagnostic of thrombotic thrombocytopenic purpura after the hematological profile was assessed. The authors emphasize the importance of the disease recognition as a prognostic marker for obstetric patients, in view of the similarity with other common morbidities during pregnancy and the importance of timely diagnosis and early treatment as determinant factors for the outcome.

  1. The European Medicines Agency review of eltrombopag (Revolade) for the treatment of adult chronic immune (idiopathic) thrombocytopenic purpura: summary of the scientific assessment of the Committee for Medicinal Products for Human Use.

    Science.gov (United States)

    Nieto, Maria; Calvo, Gonzalo; Hudson, Ian; Feldschreiber, Peter; Brown, David; Lee, Ching Cheng; Lay, Geoffrey; Valeri, Anna; Abadie, Eric; Thomas, Angela; Pignatti, Francesco

    2011-09-01

    On 11(th) March 2010, the European Commission issued a marketing authorization valid throughout the European Union for Revolade for the treatment of adult chronic immune (idiopathic) thrombocytopenic purpura. Revolade is an orphan medicinal product indicated for splenectomized patients with immune (idiopathic) thrombocytopenic purpura who are refractory to other treatments (e.g. corticosteroids, immunoglobulins) and as second-line treatment for non-splenectomized patients where surgery is contraindicated. The active substance of Revolade is eltrombopag (ATC code B02BX05). Eltrombopag increases platelet production through activation of the thrombopoietin receptor. The recommended oral dose is 50 mg once daily to achieve and maintain a platelet count of the 50×10(9)/L or more necessary to reduce or prevent the risk of bleeding. The benefit of Revolade is a durable response in maintaining platelet levels. The most common side effects include headache, nausea, hepatobiliary toxicity, diarrhea, fatigue, paresthesia, constipation, rash, pruritus, cataract, arthralgia and myalgia. The decision to grant the marketing authorization was based on the favorable recommendation of the Committee for Medicinal Products for Human Use of the European Medicines Agency. The objective of this paper is to describe the data submitted to the European Medicines Agency and to summarize the scientific review of the application. The detailed scientific assessment report and product information, including the summary of product characteristics, are available on the European Medicines Agency website (www.ema.europa.eu). PMID:21712542

  2. Intravenous anti-D treatment of immune thrombocytopenic purpura: analysis of efficacy, toxicity, and mechanism of effect.

    Science.gov (United States)

    Bussel, J B; Graziano, J N; Kimberly, R P; Pahwa, S; Aledort, L M

    1991-05-01

    The efficacy, toxicity, and mechanism of effect of intravenous Anti-D (Winrho) were studied in 43 Rh+ patients with immune thrombocytopenia purpura (ITP) who had not undergone splenectomy and in three already splenectomized patients. The mean platelet increase for the 43 nonsplenectomized patients was 95,000/microL (median 43,000/microL). Children had greater acute platelet responses than did adults. Human immunodeficiency virus status and duration of thrombocytopenia did not affect response. Maintenance treatment was given to patients as needed: the average interval between infusions was 24 days. The three splenectomized patients had no platelet response whatsoever. Toxicity was minimal; infusions were completed in less than 5 minutes. The generally accepted mechanism of effect of Anti-D has been Fc receptor blockade by substitution of antibody-coated red blood cells for antibody-coated platelets. Evidence is presented suggesting that the effect of IV Anti-D is not limited to Fc receptor blockade, including: (1) no correlation of parameters of hemolysis with platelet increase; (2) a 48- to 72-hour delay before platelet increase; (3) a tendency of the change in monocyte Fc receptor I expression to correlate with platelet increase; and (4) increased in vitro production of antibodies to sheep red blood cells following IV Anti-D infusion. PMID:1850307

  3. Clinical Observation on the Treatment of Childhood Refractory Idiopathic Thrombocytopenic Purpura with Dihuang Zhixue Capsule(地黄止血胶囊)

    Institute of Scientific and Technical Information of China (English)

    LIU Qing-chi; WU Wei-hai; WU Da-yong; FENG Xin-wang; MA Ya-hui; LI Jian-ying; PANG Yu-hui; SONG Shu-hua

    2008-01-01

    Objective:To observe the clinical effect of Dihuang Zhixue Capsule(地黄止血胶囊,DZC,a Chinese preparation for cooling blood and dispelling toxic substances) in the treatment of childhood refractory idiopathic thrombocytopenic purpura(RITP),with cyclosporin A(CsA)used as the control.Methods:Fody-one children of RITP were randomized into the treated group and the control group.The 21 patients in the treated group were orally given 2 to 3 DZC capsules each time,thrice a day and the 20 in the control group were given 3 mg/kg CsA per day,with 3 months as one therapeutic course.The therapeutic efficacy,platelet count and adverse reaction in the two groups were compared at the end of the course.Results:(1)In the treated group,1 (4.8%)patient was evaluated as cured,3(14.3%)as markedly effective,5(23.8%)as effective,5(23.8%)as improved,7(33.3%)as ineffective,with the total effective rate being 66.7%:while in the control group,the corresponding numbers were 0,2(10.0%),2(10.0%),3(15.0%),13(65.0%)and 35.0%,respectively,showing statistical significance in difference between the total effective rates of the two groups(X2=4.11,P=0.0426).(2)As compared with the baseline,the platelet count increased in both groups after 2 months'treatment(P<0.05).After 3 months'treatment,the platelet count was higher in the treated group than in the control group(P<0.05).(3)The improvement of hemorrhage in the treated group after 8 weeks'treatment was better than that in the control group (P<0.05).(4)No apparent adverse reaction was observed in fhe treated group,while in the control group,hirsutism was shown in 15 cases;gingival hyperplasia in 10;digestive reaction in 5,liver function impairment in 5.hypedension in 2 and renal impairment in 2.Conclusion:The therapeutic efficacy of DZC is better than that of CsA.and DZC shows good compliance but brings no obvious adverse reaction.

  4. A disease-specific measure of health-related quality of life for use in adults with immune thrombocytopenic purpura: Its development and validation

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    McMillan Robert

    2007-02-01

    Full Text Available Abstract Background No validated disease-specific measures are available to assess health-related quality of life (HRQoL in adult subjects with immune thrombocytopenic purpura (ITP. Therefore, we sought to develop and validate the ITP-Patient Assessment Questionnaire (ITP-PAQ for adult subjects with ITP. Methods Information from literature reviews, focus groups with subjects, and clinicians were used to develop 50 ITP-PAQ items. Factor analyses were conducted to develop the scale structure and reduce the number of items. The final 44-item ITP-PAQ, which includes ten scales [Symptoms (S, Bother-Physical Health (B, Fatigue/Sleep (FT, Activity (A, Fear (FR, Psychological Health (PH, Work (W, Social Activity (SA, Women's Reproductive Health (RH, and Overall (QoL], was self-administered to adult ITP subjects at baseline and 7–10 days later. Test-retest reliability, internal consistency reliability, construct and known groups validity of the final ITP-PAQ were evaluated. Results Seventy-three subjects with ITP completed the questionnaire twice. Test-retest reliability, as measured by the intra-class correlation, ranged from 0.52–0.90. Internal consistency reliability was demonstrated with Cronbach's alpha for all scales above the acceptable level of 0.70 (range: 0.71–0.92, except for RH (0.66. Construct validity, assessed by correlating ITP-PAQ scales with established measures (Short Form-36 v.1, SF-36 and Center for Epidemiologic Studies Depression Scale, CES-D, was demonstrated through moderate correlations between the ITP-PAQ SA and SF-36 Social Function scales (r = 0.67, and between ITP-PAQ PH and SF-36 Mental Health Scales (r = 0.63. Moderate to strong inter-scale correlations were reported between ITP-PAQ scales and the CES-D, except for the RH scale. Known groups validity was evaluated by comparing mean scores for groups that differed clinically. Statistically significant differences (p Conclusion Results provide preliminary evidence of

  5. Púrpura trombocitopênica idiopática e linfoma não-Hodgkin de células T na infância Idiopathic thrombocytopenic purpura and T-cell non-Hodgkin's lymphoma in childhood

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    Alessandra C. Borges

    2006-03-01

    Full Text Available Os linfomas representam 10% de todos os tumores malignos da infância e, destes, os linfomas não-Hodgkin são os mais freqüentes. Crianças com doenças auto-imunes apresentam maior probabilidade de desenvolver doenças linfoproliferativas, podendo ocorrer antes, durante ou após o aparecimento da neoplasia. A associação de púrpura trombocitopênica idiopática e linfomas é infreqüente (3%, principalmente na faixa etária pediátrica. Duas teorias tentam explicar a origem desta associação. Na primeira, a trombocitopenia seria decorrente da produção de auto-anticorpos antiplaquetas pelo clone tumoral. Na segunda, a PTI seria resultado de um estímulo antigênico persistente, secundário a uma desordem na proliferação linfóide. O objetivo do presente trabalho foi relatar a associação infreqüente na infância entre púrpura trombo-citopênica idiopática e linfoma não-Hodgkin de células T.Lymphomas represent 10% of all malignant tumors in childhood and from these non-Hodgkin's lymphomas are the most frequent. Children who have autoimmune diseases have a higher probability of developing lymphoproliferative diseases, which can happen before, during or after the appearance of the neoplasia. The association between idiopathic thrombocytopenic purpura and lymphomas is not common (3% especially in children. Two theories try to explain the origin of this association. In the first one, the thrombocytopenia would be a result of an autoantibody anti-blood platelet production by the tumoral clone. In the second one, the idiopathic thrombocytopenic purpura would be a result of a persistent antigenic stimulus subordinate to a disorder in the lymphoid proliferation. The aim of this work is to report the unusual association between idiopathic thrombocytopenic purpura and T-cell non-Hodgkin's lymphoma in childhood.

  6. Report of 2 cases with thrombotic thrombocytopenic purpura%血栓性血小板减少性紫癜2例报道

    Institute of Scientific and Technical Information of China (English)

    杨学敏; 董晨明; 王爱华

    2012-01-01

    目的 总结兰州大学第二医院2例血栓性血小板减少性紫癜(TTP)的所有实验检测资料.方法 回顾性分析与TTP诊断特征密切相关的实验室检测:血红蛋白(Hb)、血小板(PLT)、网织红细胞(RET)、裂片红细胞(FRC)、尿蛋白(PRO)、尿潜血(BLD)、乳酸脱氢酶(LDH)、总胆红素(TBil)、直接胆红素(DBil)、间接胆红素(IBil)、尿素氮(BUN),肌酐(Cr)、D二聚体(D-D)及骨髓检测,并归纳分析.结果 血浆、PLT、静丙输注无法改善PLT的严重减少;输注红细胞(RBC),Hb进行性下降;尿液PRO及BLD定性持续阳性;溶血检测中的RET、TBil、IBil及LDH持续增高;凝血检测中D-D持续不降;骨髓分析提示2例患者分别是增生性贫血与溶血性贫血并考虑Evans综合症.结论 对于TTP浅显认识导致临床诊治延误.回顾性总结TTP实验室检测及临床特征,发现有经典“五联征”,包括血小板减少症、微血管病性溶血性贫血、神经症状、肾脏损害及发热.%Objective To summarize the examination data of 2 cases with thrombotic thrombocytopenic purpura (TTP)in Lanzhou University Second Hospital. Methods The closely related diagnostic features of TTP were reviewed retrospectively:hemoglobin (Hb), platelet (PLT), reticulocyte (RET), fragments of red blood cell (FRC), urinary protein (PRO ), urinary occult blood (BLD ), lactate dehydrogenase (LDH), total bilirubin (Tbil), direct bilirubin (Dbil),indirect bilirubin (Ibil),urea nitrogen (BUN),creatinine (Cr),D-dimer (D-D)and bone marrow analysis. Results Intravenous infusing plasma, PLT and immunoglobulin could not improve the severe reducing of PLT. Intravenously infusing red blood cells (RBC), Hb declined progressively. PRO and BLD were persistently positive. RET,Tbil,Ibil and LDH of hemolysis test continued to rise. D-D of coagulation test continued not to reduce. Bone marrow analysis showed that 2 patients were proliferative anemia and hemolytic anemia with Evans

  7. 儿童特发性血小板减少性紫癜202例临床分析%Clinical characteristics of 202 cases with children idiopathic thrombocytopenic Purpura

    Institute of Scientific and Technical Information of China (English)

    周登余

    2011-01-01

    Objective To analyze the characteristics and therapy efficiency of children idiopathic thrombocytopenic purpura (ITP), and compare the differences between acute and chronic ITP. Methods 202 cases of idiopathic thrombo-cytopenic purpura who were diagnosed and treated in the First Affiliated Hospital of Anhui Medical University from 2008 to 2010 were reviewed. Results Among 202 cases (106 male and 96 female), acute ITP was 177 cases (87.62% ), chronic ITP (CITP) and refractory ITP (RITP) were 25 cases (12.38% ). Most of acute ITP in children were less than 6 year old (77.23%). 174 cases (86.14%) of ITP had severe thrombocytopenia and 81.68% of them showed mild bleeding. For acute ITP, the efficiency of different therapy had no significant difference in Pred group (89.66%), IVIG group (73.33%) and combination group (88.98% ), respectively. But it was lower in combination group in chronic/re-fractory ITP (54.55% ). Conclusions Childhood ITP mostly occur at the age of 1 to 6 year, the incidence of ITP is e-qual between men and women, the majority of the children's ITP cases are acute course with mild bleeding and recover fast.%目的 分析儿童特发性血小板减少性紫癜(idiopathic thrombocytopenic purpura,ITP)的临床表现及治疗情况,比较急、慢性ITP的不同点.方法 对安徽医科大学第一附属医院2008年1月~2010年12月期间,住院的202例ITP病例临床资料进行回顾性分析.结果 202例ITP患儿,男106例(52.48%),女96例(47.52%),急性ITP 177例(87.62%),慢性、难治性ITP 25例(12.38%),6岁及以下患儿156例(77.23%),6岁以上46例(22.77%);有174例(86.14%)ITP患儿发病时血小板呈重度、极重度减少,但临床多为轻度出血(81.68%);急性组单用激素治疗有效率89.66%,单用丙种球蛋白治疗有效率73.33%,两者联合治疗有效率为88.98%;慢性、难治性ITP激素联合丙种球蛋白治疗有效率54.55%.结论 儿童ITP多见于6岁及以下,男女发病机会均等,该病临床发病急

  8. Staphylococcus aureus septicemia presenting as disseminated intravascular coagulation - thrombotic thrombocytopenic purpura overlap and thrombus in inferior vena cava, right atrium and right ventricle: a case report

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    Khwaja Saifullah Zafar

    2015-01-01

    Full Text Available Staphylococcal sepsis following furunculosis and complicated by suspected deep vein thrombosis and septic inferior vena caval, right atrium, right ventricle emboli accompanied by disseminated intravascular coagulation (DIC - thrombotic thrombocytopenic overlap in a 65 years old lady is presented. She was managed successfully with antibiotics and anticoagulation. The case is reported for its rarity and brings to light the vivid manifestations of septicemia specially staphylococcal. [Int J Res Med Sci 2015; 3(1.000: 368-372

  9. Helicobacter pylori infection and idiopathic thrombocytopenic purpura in children%幽门螺杆菌感染与儿童特发性血小板减少性紫癜

    Institute of Scientific and Technical Information of China (English)

    刘巧

    2010-01-01

    Idiopathic thrombocytopenic purpura(ITP) is characterized by platelet destruction which is mediated by auto-antibodies. Recent researches show that helicobacter pylori infection is correlated with part of FTP in adult. Eradication of helicobacter pylori infection has brought dramatically platelet elevation. It was reported that cross reaction played a very important part in the helicobacter related FTP, but the exact mechanism is still unknown, whether the FTP in children needs helicobacter pylori eradication is under debate.%特发性血小板减少性紫癜(ITP)是以自身抗体介导的血小板破坏为特征的一组疾病,近年来许多研究显示部分成年人ITP的发生与幽门螺杆菌(Hp)感染密切相关,清除Hp后ITP患者的血小板数量有明显提高.研究认为Hp相关的ITP与抗原交叉反应有关,但其确切机制目前尚不明确;儿童ITP是否需要清除Hp治疗,国内外也存在争议.

  10. 特发性血小板减少性紫癜患儿病原感染的临床分析%Clinical analysis of pathogen infection in children with idiopathic thrombocytopenic purpura

    Institute of Scientific and Technical Information of China (English)

    王晓格; 胡姬婷

    2014-01-01

    目的:探讨特发性血小板减少性紫癜(ITP)患儿与病原感染的关系。方法:回顾性分析89例ITP患儿发病的诱因,应用ELISA法检测巨细胞病毒、EB病毒、幽门螺杆菌(Hp)、支原体,并分析与ITP的关系。结果:89例ITP患儿中,46.1%的患儿发病前1~3周有病毒感染,17.9%的患儿在发病前曾有预防接种史。其中 CMV 阳性10例(11.24%),EB 病毒阳性7例(7.86%),Hp 阳性12例(13.48%),MP 阳性2例(2.25%),总阳性率30.34%(27/89)。发病时血小板计数及治疗后血小板升至正常的时间在病原阳性组与阴性组比较,差异无统计学意义(P>0.5)。经过随访,急性转为慢性 ITP 4例(4.49%),各病原阳性组总的转慢率7.40%,病原阴性组转慢率3.22%,两组比较差异有统计学意义(P<0.05)。结论:病原感染是特发性血小板减少性紫癜发病的一个重要原因,其中以HP感染所占比例较高,Hp感染组ITP患儿易迁延不愈转为慢性。%Objective:To explore the relationship between pathogen infection and children with idiopathic thrombocytopenic purpura(ITP).Methods:Cause of the disease of 89 children with ITP were retrospectively analyzed.Cytomegalovirus,EB virus, helicobacter pylori,mycoplasma were detected using ELISA method.We analyzed the relationship between them and ITP.Results:In the 89 children with ITP,46.1% children were infected by virus in 1 to 3 weeks before the onset of disease,17.9% children had vaccination history before the onset of disease.The CMV was positive in 10 cases(11.24%),EB was positive in 7 cases(7.86%),HP was positive in 12 cases(13.48%),MP was positive in 2 cases(2.25%).The total positive rate was 30.34%(27/89).The platelet count at the time of onset and platelet rose to normal time after treatment between pathogen positive group and negative group were compared.There were no statistical significance(P>0.5).At follow-up,4 patients with acute ITP turned to chronic ITP(4.49%).In

  11. Relationship between idiopathic thrombocytopenic purpura and helicobacter pylori CagA protein in adults%成人特发性血小板减少性紫癜与幽门螺杆菌CagA蛋白的关系

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    张晋琳; 李慧; 王晓冬; 王春森; 吴良华

    2011-01-01

    目的了解成人特发性血小板减少性紫癜(idiopathic thrombocytopenic purpura,ITP)与幽门螺杆菌(helicobacter pylori,Hp)CagA蛋白的关系.方法 采用14C尿素呼吸试验对正常成人和ITP患者进行Hp检测后,对Hp阳性的ITP患者进行CagA蛋白抗体检测.依据急性ITP,慢性ITP,难治性ITP对ITP患者进行分类.结果 223例正常患者中123例Hp阳性,感染率为55.2%,而CagA蛋白抗体阳性为42例,占34.1%.278例ITP患者中Hp阳性176例,感染率63.3%,其中CagA蛋白抗体阳性83例,占47.2%,ITP患者的Hp感染率与正常人相似,但CagA蛋白抗体阳性率显著高于正常人(P0.05).结论 纳入研究的ITP患者的Hp感染率与正常人相似,但CagA蛋白抗体阳性率明显高于正常人,提示CagA蛋白在成人ITP的发生发展中具有一定作用.进一步分析也显示不同类型的ITP患者Hp感染率及CagA蛋白阳性率差异无统计学意义.

  12. Alteration in frequency and function of CD4⁺CD25⁺FOXP3⁺ regulatory T cells in patients with immune thrombocytopenic purpura.

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    Nargess Arandi

    2014-04-01

    Full Text Available Immune thrombocytopenic purpura (ITP is an autoimmune bleeding disorder characterized by production of auto-antibodies against platelet antigens. It is obvious that regulatory T cells (Tregs have a major role in controlling immune homeostasis and preventing autoimmunity.To investigate the frequency and functions of Tregs, twenty ITP patients and twenty age- and sex-matched healthy controls were recruited. The peripheral blood mononuclear cells were isolated and the proportion of Tregs was defined by flow cytometry method. The expression of immune-regulatory markers, cytotoxic T-lymphocyte associated antigen-4 (CTLA-4 and glucocorticoid induced tumor necrosis factor receptor (GITR were also assessed by quantitative Real-time PCR TaqMan method. For evaluation of Treg function, Tregs were enriched and their ability to inhibit proliferation of T cells was measured and levels of immune-regulatory cytokines IL-10 and TGF-β were also measured.Results showed that the frequency of Tregs and the mean fluorescence intensity of FOXP3 protein significantly decreased in ITP patients compared to those in healthy controls. In addition, there was a significant reduction in relative expression of both CTLA-4 and GITR mRNA in ITP patients (P=0.02 and P=0.006, respectively. The suppressive function of Tregs also diminished in ITP patients compared to that in controls. Both IL-10 and TGF-β cytokines were produced in lower amounts in ITP patients than controls.It could be concluded that alteration in Treg frequency and functional characteristics might be responsible for loss of self-tolerance and subsequently destructive immune responses observed in ITP patients.

  13. The role of tumor necrosis factor-alpha -308 G/A and transforming growth factor-beta 1 -915 G/C polymorphisms in childhood idiopathic thrombocytopenic purpura

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    Emel Okulu

    2011-09-01

    Full Text Available Objective: To increase our understanding of the etiology of idiopathic thrombocytopenic purpura (ITP some cytokine gene polymorphisms were analyzed for susceptibility to the disease. The aim of this study was to investigate the role of tumor necrosis factor-alpha (TNF-α -308 G/A and transforming growth factor-beta 1 (TGF-β1 –915 G/C polymorphisms in the development and clinical progression of childhood ITP.Materials and Methods: In all, 50 pediatric patients with ITP (25 with acute ITP and 25 with chronic ITP and 48 healthy controls were investigated via LightCycler® PCR analysis for TNF-α -308 G/A and TGF-β1 -915 G/C polymorphisms.Results: The frequency of TNF-α -308 G/A polymorphism was 20%, 16%, and 22.9% in the acute ITP patients, chronic ITP patients, and controls, respectively (p>0.05. The frequency of TGF-β1 -915 G/C polymorphism was 16%, 8%, and 8.3% in the acute ITP patients, chronic ITP patients, and controls, respectively (p>0.05. The risk of developing ITP and clinical progression were not associated with TNF-α -308 G/A (OR: 0.738, 95% CI: 0.275-1.981, and OR: 0.762, 95% CI: 0.179-3.249 or TGF-β1 -915 G/C (OR: 1.5, 95% CI: 0.396-5.685, and OR: 0.457, 95% CI: 0.076-2.755 polymorphisms. Conclusion: The frequency of TNF-α -308 G/A and TGF-β1 -915 G/C polymorphisms did not differ between pediatric ITP patients and healthy controls, and these polymorphisms were not associated with susceptibility to the development and clinical progression of the disease.

  14. The efficacy of tonsillectomy in treatment idiopathic thrombocytopenic purpura%扁桃体切除术治疗血小板减少性紫癜的临床观察

    Institute of Scientific and Technical Information of China (English)

    唐林甫; 简树财; 唐奕; 罗小莉; 唐萍; 范颖; 李晞晨

    2012-01-01

    To study the causality between chronic tonsillitis and idiopathic thrombocytopenic purpura(ITP), and evaluate the therapeutic effect of tonsillectomy. Methods There were 31 patients who were sick of chronic tonsillitis complicated ITP , all of them were treated by tonsillectomy. To observe the clinical symptom and platelet count in 2-year follow-up. Results 24 cases'symptom were disappeared, and the platelet count rised to normal level on the 1nt week ,3nd month, 1nt year and 2nd year,there was statistical significance compared with preoperative (P <0.01), 7 cases' symptom of chronic tonsillitis were disappeared,but the symptom of ITP still existed and the platelet count had no significant increased compared with preoperative (p< 0. 01). Conclusion Chronic tonsillitis is an important cause of ITP, and tonsillectomy may be an effective treatment for ITP.%目的 探讨慢性扁桃体炎与血小板减少紫癜(ITP)的因果关系及治疗方法.方法 31例慢性扁桃体炎合并ITP的患者行扁桃体切除术,术后随访观察血小板计数及临床症状,随访2年.结果 24例慢性扁桃体炎及ITP症状消失,术后1周、3个月、1年、2年血小板计数达到正常水平与术前比较差异有统计学意义(P<0.01),随访期内未见ITP复发.7例患者扁桃体炎症状消失,但皮肤、粘膜出血未见明显改善,血小板计数较术前差异无统计学意义(P>0.05).结论 慢性扁桃体炎是引起ITP的重要原因之一,扁桃体切除术是治疗ITP的一种有效方法.

  15. Epstein -barr virus infection in children with acute idiopathic thrombocytopenic purpura%儿童急性特发性血小板减少性紫癜与EB病毒感染的关系

    Institute of Scientific and Technical Information of China (English)

    白松婷; 赵晓明; 盛光耀; 方营旗

    2011-01-01

    Objective Acute idiopathic thrombocytopenic purpura (AITP) is the most common hemorrhagenic disease in children. It often appears to be related to the sensitization by some viral infections, such as Epstein - Barr virus ( EBV ), cytomegalovirus (CMV), Human parvovirus B19 ( HPVB19 ) and so on. The present study is to elucidate EBV infection in children with AITP. Methods The study cohorts consisted of 87 children with AITP and 40 healthy children. Specific viral antibody - EBV - IgM was detected by ELISA. The laboratory characteristics were analyzed in patients with and without EBV infection. Results Twenty - one (24. 14% ) of 87 children with AITP associated with EBV infection. Which was markedly higher than that of the controls (P <0. 01 ). In children with AITP,the platelet and magakaryocyte counts were similar in patients with and without EBV infection (P > 0. 05 ). However, in the group of EBV infection, the positive rate of antiplatelet antibodies (GPIIb/Ⅲa)in plasma and the numbers of variant lymphocytes in peripheral smear were both significantly increased compared with the group of non -EBV infection( P < 0. 01 ). Conclusions EBV should be the causative viral agent in some children with AITP. Its pathogenesis may initiate the autoimmune reaction.%目的 探讨儿童急性特发性血小板减少性紫癜(AITP)与EBV感染的关系.方法 用酶联免疫吸附法(ELISA)测定87例AITP患儿病毒特异性抗体EBV-IgM,做骨髓巨核细胞、外周血小板以及外周血异形淋巴细胞计数,采用改良MAIPA法检测血小板特异性抗体(GPⅡb/Ⅲa).对照组为40例健康体检的儿童.用统计学分析软件包SPSS13.0进行统计学分析.结果 87例AITP患儿中,EBV-IgM阳性者21例,阳性率24.14%,明显高于对照组(P0.05).结论 EBV感染确实与部分儿童AITP的发病有关.EBV感染引起ITP的机制可能与引发机体自身免疫反应有关.

  16. The intervention of amifostine in idiopathic thrombocytopenic purpura mouse%氨磷汀对免疫性血小板减少性紫癜动物模型小鼠的干预作用

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    周国坚; 王桂明; 蔡小连

    2013-01-01

      目的探讨氨磷汀对免疫性血小板减少性紫癜(ITP)动物模型小鼠血小板、脾脏系数、骨髓巨噬细胞的影响。方法通过获取BALB/C 小鼠血小板抗体,接种到豚鼠身上,获取豚鼠抗小鼠血小板抗体(APS)。将APS腹腔注射BALB/C 小鼠建立免疫性血小板减少性紫癜动物模型,使用氨磷汀腹腔注射进行治疗,观察氨磷汀对模型小鼠的血小板、脾脏系数、骨髓巨噬细胞的影响。结果氨磷汀给药后均使ITP模型小鼠血小板计数上升、脾脏系数减少、骨髓成熟巨核细胞增多,与模型组比较差异有统计学意义(P<0.01)。结论氨磷汀对免疫性血小板减少性紫癜有较为明显的治疗效果。%Objective To study the effect of amifostine on mice models of idiopathic thrombocytopenic purpura(ITP). Methods Guinea pig anti mice platelet serum(GP-APS) was diluted with saline(1:4),and intravitreal injected into mice once in 2 days for 10 days.Two dosage of amifostine was intravitreal injected into mice in 10 days since mice models of ITP were established. Results Amifostine can enhance the amount of platelets,promote the mature of megakaryocyte of mice significantly and improve the histological pathology of spleen. Conclusion Amifostine is an effective and safety medicine in treating ITP, and can be used widely in clinic.

  17. Preliminary Screening of PRO Scale Items of Idiopathic Thrombocytopenic Purpura%特发性血小板减少性紫癜PRO量表条目的初步筛选

    Institute of Scientific and Technical Information of China (English)

    龙远雄; 马利; 何丽云; 闫世艳; 王彬; 王双连

    2015-01-01

    目的:筛选特发性血小板减少性紫癜(ITP)PRO量表条目,形成适合我国ITP患者疗效评价的PRO量表。方法:采用专家评分法、因子分析法、离散程度法、克朗巴赫系数(Cronbach Alpha)法、相关系数法(包括2种),从条目的重要性大、敏感性高、独立性强、代表性好、确定性等方面进行筛选。结果:终选量表共54个条目,分为生理、心理和社会3个领域,其中生理领域包括出血、疼痛与不适、睡眠与休息三个维度,最终保留条目数为36条,心理领域保留12条,社会保留6条。结论:筛选后的量表离推广运用还有一定差距,还需在其他人群中进行信度和效度检验。%Objective:To screen PRO scale items of idiopathic thrombocytopenic purpura and form the PRO scale suitable for the evaluation of therapeutic efficiency of ITP patients in China. Methods: The expert grading method, factor analysis method, discrete degree method, Cronbach Alpha method and correlation coefficient method (including two methods) are used to screen from the important, high-sensitivity, strong-independence, representative and certain entries. Results:The ultimate scale has a total of 54 items, divided into physiological, psychological and social area. Among them, the physical area includes three dimensions, which are bleeding, pain and discomfort, and sleep and rest. There are a total of 36 final reserved entries, 12 reserved entries in the psychological area and 6 social reserved entries. Conclusion: After the screening the scale still cannot be popularized and applied. We still need to do the reliability and validity test in other groups.

  18. 重组人白细胞介素-11联合长春新碱治疗激素无效特发性血小板减少性紫癜临床观察%Investigation of the Efficacy of Recombinant Human Interleukin-11 in Combination with Vincristine in Hormone Invalid Idiopathic Thrombocytopenic Purpura

    Institute of Scientific and Technical Information of China (English)

    滕志

    2012-01-01

    目的 探讨重组人白细胞介素-Ⅱ (rhIL-Ⅱ)联合长春新碱治疗激素无效特发性血小板减少性紫癜(ITP)患者的临床疗效及安全性.方法 选取2008年12月~2011年12月在我院血液内科住院治疗的112例激素无效ITP患者为研究对象,应用随机数字表法将患者分为对照组和观察组.对照组患者给予长春新碱,而观察组患者则加用rhIL-Ⅱ进行治疗,比较对照组和观察组患者临床疗效及药物不良反应发生情况.结果 观察组患者治疗总有效率(85.71%)明显高于对照组的(69.64%),比较差异有统计学意义(P<0.05).两组患者不良反应发生率比较差异无统计学意义(P>0.05).结论 rhIL-Ⅱ联合长春新碱治疗激素无效ITP具有较好的疗效和安全性,值得在经济条件落后地区及基层医院推广.%Objective The purpose of this study is To investigate the Clinical efficacy and safety of recombinant human interleukin-II combined with vincristine in hormone invalid idiopathic thrombocytopenic purpura. Methods 112 patients with hormone invalid idiopathic thrombocytopenic purpura in our hospital from December 2008 to December 2011 were randomly divided into control group and observation group. Patients in the control group were treated with vincristine. Patients in the observation group were treated with recombinant human interleukin- II combined with vincristine. The efficacy and side effect were analyzed in the control group and observation group. Results The total efficient rate was higher in the observation group than control group (85.71% vs 69.64%,P0.05). Conclusion Recombinant human interleukin- II combined with vincristine obtained satisfactory efficacy and safety for hormone invalid idiopathic thrombocytopenic purpura. It is worth expanding in hospitals of regional basic-level economic conditions

  19. Efficacy of vincristine in combination with interleukin-11 treatment of refractory idiopathic thrombocytopenic purpura%长春新碱联合白介素11治疗难治性特发性血小板减少性紫癜疗效观察

    Institute of Scientific and Technical Information of China (English)

    刘国锋

    2013-01-01

    目的探讨长春新碱联合重组人白介素11治疗难治性血小板减少性紫癜的疗效方法。方法通过分析我院2006年3月~2011年2月间符合23例难治性血小板减少性紫癜的临床资料,采用长春新碱联合重组人白介素11进行治疗。结果显效8例,良9例,进步2例,无效4例,总有效率达73.9%(17/23)。结论长春新碱联合重组人白介素11治疗难治性ITP疗效切实可行,不良反应小。%Objective:Vincristine recombinant human interleukin 11 in the treatment of refractory thrombocytopenic purpura efficacy. Methods :By analyzing our hospital from March 2006 to February 2011 in line with the clinical data of 23 patients with refractory thrombocytopenic purpura, vincristine recombinant human interleukin-11 treatment. Results: Effective in 8 cases, good in 9 cases, progress in the two cases, 4 cases, the total effective rate of 73.9% (17/23). Conclusion: Vincristine recombinant human interleukin-11 treatment of refractory ITP efficacy practicable adverse reactions.

  20. 成人特发性血小板减少性紫癜淋巴细胞亚群研究%Study on Changes and Clinical Significance of Lymphocyte Subtypes in Adult Patients with Idiopathic Thrombocytopenic Purpura

    Institute of Scientific and Technical Information of China (English)

    孙雄飞; 陈卫布; 林海清; 伍娟

    2012-01-01

    目的 研究淋巴细胞亚群在成人特发性血小板减少性紫癜(ITP)治疗前后及正常对照人群中的表达变化,探讨其在ITP发病机制中的作用及临床意义.方法 应用流式细胞术检测23例ITP患者治疗前、后及23例正常对照者外周血淋巴细胞亚群的表达水平,包括CD 3+、CD3+CD4+、CD 3+CD 8+、CD 19+淋巴细胞百分比及CD 3+CD4+/CD 3+CD 8+比值.结果 ITP患者治疗前CD3+T淋巴细胞百分比、CD3+CD4+T淋巴细胞百分比及CD 3+CD4+/CD 3+CD 8+比值均较正常对照组及ITP治疗后组减低(P<0.05),CD 3+ CD 8+T淋巴细胞百分比及CD 19+B淋巴细胞百分比较正常对照组及ITP治疗后组升高(P<0.05),正常对照组与治疗后ITP组四项指标无明显统计学差异(P<0.05).结论 T、B淋巴细胞亚群异常参与了ITP的发病,对其检测可能对ITP的诊断及治疗有帮助.%Objective To study the changes in lymphocyte subsets in patients with idiopathic thrombocytopenic purpura (1TP) before and after therapy and healthy controls ,and then to explore the clinical significance of the related factors envolved in pathlgenesis of ITP. Methods The percentages of lymphocyte subsets in the perpheral blood of 23 patients with ITP and 23 heaithy controls were detected by flow cytometry, including CD 3+ , CD 3+ CD 4+, CD 3 + CD 8 VCD 19+ lymphocytes and CD 3 + CD 4+/ VCD 3 + CD 8+. Results The percentages of CD 3 + T lymphocyte, CD 4 + T lymphocyte and the ratio of CD 4 VCD 8 + in patients with ITP were obviously lower than those in healthy controls and ITP after immunosuppressive therapy ( P < 0. 05 ) , but the percentages of CD 3 + CD 8 + T lymphocyte and CD 19 + B lymphocyte was much higher ( P < 0.05) than those in heaithy controls and ITP afrer immunosuppressive therapy. There were no significant difference between the healthy controls and ITP afrer therapy (P <0.05) . Conclusions The aberrant changes in T and B lymphocyte subsets are involved in the pathogenesis of ITP

  1. 幽门螺杆菌感染与特发性血小板减少性紫癜关系的荟萃分析%Relationship between Helicobacter pylori infection and idiopathic thrombocytopenic purpura: a meta-analysis

    Institute of Scientific and Technical Information of China (English)

    谭勇; 施瑞华; 赵福军

    2009-01-01

    目的:评价特发性血小板减少性紫癜(ITP)与幽门螺杆菌(H pylori )感染的相关性.方法:检索PubMed、EMBase和CNKI数据库,获取ITP与H pylori 感染的病例-对照研究, 将入选的病例分成4组:H pylori 阳性并成功清除组; H pylori 阳性但清除不成功组; H pylori 阳性未清除治疗组; H pylori 阴性组. 每组均记录进入实验时各患者的基础血小板计数和随访时的血小板计数, 并在组内行荟萃分析.结果:检索文献中纳入21篇病例-对照研究, 其中国外17篇, 国内4篇. 在随访前后, H pylori阳性并成功清除组P LT计数有统计学意义(WMD 61.70, 95%CI:47.58-75.81); H pylori阳性但清除不成功组PLT计数无统计学意义(WMD 11.41, 95%CI:-0.07-22.88); H pylori阳性未清除治疗组P LT计数无统计学意义(WMD 15.77, 95%CI:-7.99-39.54); H pylori阴性组PLT计数有统计学意义(WMD 24.24,95%CI:8.54-39.93).结论:H pylor i 阳性的ITP患者在成功清除H pylori 后PLT计数上升, 但H pylori 感染并不是ITP发生的唯一因素.%AIM: To investigate the relationship between Helicobacter pylori (H pylori) infection and idiopathic thrombocytopenic purpura (ITP). METHODS: The databases of PubMed, EMBase and CNKI were searched for published case-control studies on association between H pylori infection and ITP. We focused on the difference in the platelet count between the experimental arm (H pylori-infected patients who responded to eradication therapy) and each control arm (H pylori-infected patients who failed to respond to eradication therapy; H pylori-infected patients without eradication therapy and H pylori-negative patients). Data were extracted using a standardized form and the meta-analysis was performed. RESULTS: Twenty-one eligible studies, including 17 studies carried by foreigners, and 4 by Chinese researchers, were included in the metaanalysis. There was a statistically significant difference in platelet count between patients who responded

  2. 病毒感染与免疫性血小板减少性紫癜的临床观察%Clinical observation of the relationship between immune thrombocytopenic purpura and virus infection

    Institute of Scientific and Technical Information of China (English)

    张永卓

    2013-01-01

    Objective To investigate the relationship between immune thrombocytopenic purpura (ITP) and virus infection.Methods A retrospectively analysis was based on the clinical records.ELSIA tests of EBV and CMV antibody were performed at 50 children suffered from ITP.The clinical characteristics and therapeutic effects in viral infection group and non-viral infection group were compared.Results Thirty-eight children in 50 cases who suffered from ITP were infected by EBV or CMV.Between viral infection group and non viral infection group,there was no significant difference in clinical features,hemoglobin and platelet (P > 0.05).But,it showed that the effects of viral infection group were better than those of non viral infection group at the time of two months and six months after treatment (P < 0.05).There were 18 cases ineffective in the viral infection group for six months treatment.There was the higher chronic tendency incidence in viral infection group (47.3%) than that in the non viral infection group (8.3%).Conclusions Most ITP patients infected by virus.Virus associated ITP had poor treatment effect and is easy to prolong course.%目的 探讨免疫性血小板减少性紫癜(ITP)与病毒感染的关系.方法 回顾性分析50例ITP儿童的临床资料,采用ELISA法检测50例ITP患儿血清病毒抗体(EB病毒及巨细胞病毒等),比较病毒抗体检测在病毒感染组及非病毒感染组ITP患儿中的临床特点及治疗效果.结果 病毒血清学检测:病毒感染38例,非病毒感染12例.病毒感染组与非病毒感染组比较:①两组临床表现、Hb及血小板计数比较差异均无统计学意义(P>0.05);②治疗后2个月效果比较差异有统计学意义(P<0.05),病毒感染组初始疗效较非病毒感染组治疗有效率低;③治疗后6个月两组疗效比较差异有统计学意义(P<0.05),治疗后6个月病毒感染组无效18例(47.4%),且呈慢性倾向,明显高于非病毒感染组(8.3%).结论

  3. Púrpura trombocitopênica trombótica na apresentação de pacientes com lúpus eritematoso sistêmico juvenil Thrombotic thrombocytopenic purpura at presentation of juvenile systemic lupus erythematosus patients

    Directory of Open Access Journals (Sweden)

    Lucia M. A. Campos

    2013-02-01

    Full Text Available Púrpura trombocitopênica trombótica (PTT é uma alteração hematológica rara e com risco de morte, caracterizada por trombocitopenia, anemia hemolítica microangiopática e alterações neurológicas e/ou renais. A PTT foi descrita em raros pacientes com lúpus eritematoso sistêmico juvenil (LESJ e, até onde se sabe, a prevalência dessa manifestação em uma população de lúpus pediátrico ainda não foi estudada. Assim, entre janeiro de 1983 e dezembro de 2010, revisamos os prontuários de 5.508 pacientes acompanhados na Unidade de Reumatologia Pediátrica do nosso hospital universitário. Foram identificados 279 (5,1% casos de LESJ que preencheram os critérios de classificação do American College of Rheumatology. Dois destes (0,7% apresentavam PTT, ambos no início do LESJ, e foram aqui descritos. Os dois pacientes tinham febre, anemia hemolítica microangiopática (com esquizócitos no sangue periférico e trombocitopenia. O paciente do gênero masculino apresentava hemiparesia e proteinúria, e a paciente do gênero feminino tinha cefaleia persistente e hematúria. Ambos foram tratados com metilprednisolona endovenosa e plasmaferese quando do diagnóstico de PPT. Após tratamento, não houve recidiva da PTT, e hematócritos, contagens de plaquetas e níveis de desidrogenase lática permaneceram normais. Em conclusão, a PTT é uma rara e grave manifestação no início do LESJ. Os casos relatados reforçam a importância de um diagnóstico precoce e de uma terapia agressiva em pacientes com PTT, devido à sua alta morbidade.Thrombotic thrombocytopenic purpura (TTP is a rare and life-threatening hematological abnormality characterized by thrombocytopenia and microangiopathic hemolytic anemia, with neurological abnormalities and/or renal disease. TTP has been rarely reported in juvenile systemic lupus erythematosus (JSLE patients and, to our knowledge, its prevalence in a paediatric lupus population has not been studied. Therefore

  4. 儿童急性特发性血小板减少性紫癜125例临床分析%Clinical analysis of 125 cases of children with idiopathic thrombocytopenic purpura

    Institute of Scientific and Technical Information of China (English)

    刘德新; 廖宁

    2012-01-01

    Objective To discuss the clinical features of children with idiopathic thrombocytopenic purpura (FTP). Methods The clinical data of 125 children with ITP and 3 different strategies of therapy were retrospectively analyzed. Results (1)lt occurred more commonly in small children, accounting for 60% of all cases, of which 37 cases (29.6% ) ranged from one month to one year old, 38 cases (30.4% ) ranged from one year to 3 years old. (2) Among all cases, 67 (53.6% ) had unclear causes, 58 (46.4% ) had clear causes, and 12 cases had vaccination histories within one month. (3)The bleeding symptoms could be effectively controlled by ACTH. The PLT counts increased dramatically after 3-day and 7-day treatment( P <0.01). Conclusions (1 )Childhood FTP occurred commonly in younger children. (2) More than half cases had prodromal infection histories. The incidence rate of FTP caused by vaccine injection was higher than previous reports. (3)The treatment of ACTH was effective for ITP, including oral prednisone, oral prednisone plus intravenous IVIG and intravenous DXM plus IYIG.%目的 探讨儿童特发性血小板减少性紫癜(ITP)的临床特征.方法 回顾性分析125例ITP住院患儿的临床资料,并对其发病年龄、诱因、出血情况及3种不同治疗方案的治疗效果进行分析.结果 (1)婴幼儿发病多见,其中年龄1个月~1岁者37例(29.6%),~3岁者38例(30.4%),≤3岁占所有病例数的60%;(2)起病诱因不明者67例(53.6%),诱因明确58例(46.4%),12例(9.6%)发病前1个月内有预防接种史;(3)以肾上腺糖皮质激素治疗为主的综合治疗可使出血症状很快消失;治疗后3、7d的血小板值与治疗前比较差异有显著性(P<0.01).结论 (1)儿童ITP发病年龄以婴幼儿期多见;(2)约半教患儿有前驱感染史,疫苗接种后ITP发生率高于以往报道;(3)泼尼松、泼尼松+静脉注射用人免疫球蛋白治疗以及地塞米松+静脉注射用人免疫球蛋白3

  5. 老年特发性血小板减少性紫癜患者的临床观察%Clinical study on the elderly patients with idiopathic/immune thrombocytopenic purpura

    Institute of Scientific and Technical Information of China (English)

    范芸; 常乃柏; 邢宝利; 裴蕾; 李元明; 顾惜春; 许晓东

    2008-01-01

    目的 总结老年特发性血小板减少性紫癜(ITP)患者的发病特点、治疗及临床转归.方法 回顾分析1992-2007年我院住院治疗的老年ITP患者的临床资料,并与同期住院的非老年患者的临床资料进行对照.结果 老年患者(老年组)43例,男性16例,女性27例;随访时间1个月~15年,存活35例.43例患者中,7例血小板持续(30~50)×109/L,出血不显著,未予以治疗;36例首选泼尼松治疗,敏感型25例(69.4%),以完全反应或部分反应健康存活;脾切除或栓塞4例,3例血小板恢复正常;对于泼尼松治疗不敏感者分别使用免疫抑制剂,其中硫唑嘌呤21例,环孢A23例,长春新碱3例及环磷酰胺9例,硫唑嘌呤、环孢A疗效优于长春新碱及环磷酰胺.进展为难治性ITP5例,难治率为13.9%;进展为未定性单克隆免疫球蛋白增多症(MGUS)和淋巴瘤各1例.死亡8例,死于外伤感染引发的心肺功能衰竭4例,肿瘤3例,脑出血1例.结论 老年ITP患者临床表现不典型,致命性出血的风险低,对免疫抑制剂的反应与非老年组近似,治疗宜个体化.%Objective To explore the clinical characteristics,therapy reactions and prognosis of the elderly patients with idiopathic thrombocytopenic purpura(ITP). Methods A total of 43elderly ITP patients(age≥60 years old)including 16 men and 27 women were reviewed and further followed up for 1 month to 15 years. Results Until June 2007,35 elderly ITP patients survived,platelet counts were sustained(30-50)×109/L in 7 cases,but no significant bleeding was found.Thirty-six patients had adrenocorticosteroid therapy first, 25 patients were sensitive to adrenocorticosteroid therapy,4 patients underwent splenectomy,and 3 patients achieved a normal platelet count. Immunosuppressive agents(vinscristine,cyclophosphamide, azathioprine and Cyclosporin A)treatments were held in 5 6 case-times,Cyclosporin A and azathioprine were more effective than vinscristine and cyclophosphamide

  6. 特发性血小板减少性紫癜患儿HLA-DRB1基因多态性%HLA-DRB1 polymorphism of idiopathic thrombocytopenic purpura in children

    Institute of Scientific and Technical Information of China (English)

    李晓红; 盛光耀

    2011-01-01

    Aim: To estimate the relation between human leukocyte antigen-DRBl ( HLA-DRB1 ) and idiopathic thrombocytopenic purpura (ITP) in children. Methods: Peripheral blood samples and stools were collected from 200 children with ITP and 200 normal children. GICA and ABC-ELISA were used to detect the Helicobacter pylori (Hp) antibody in blood and Hp antigen in stool. PCR specific sequence primers( PCR-SSP) was used to identify HLA-DRB1 gene. Results: The frequency of HLA-DRB1 * 07 significantly decreased in ITP children compared with controls(x2 = 5.444,P =0.020). Compared with acute ITP( AITP) , the frequency of HLA-DRB1 * 11 significantly increased in children with chronic ITP (CITP)(x2 = 6.224,P=0.013).The frequencies of HLA-DRB1 * 14 and HLA-DRB1 * 11 in ITP children with Hp infection were higher than those without Hp infection(x2 =8.602 and 8.794,all P were 0.003). Conclusion:HLA-DRB1 *07 seems to be aprotective factor to against ITP. HLA DRB1 * 11 may play an important role in CITP. HLA-DRB1 * 11 and HLA-DRB1 * 14 seems to predict susceptibility to Hp infection in ITP children.%目的:探讨人类白细胞抗原DRB1(HLA-DRB1)基因多态性与儿童特发性血小板减少性紫癜(ITP)的关系.方法:分别采用胶体金法和ABC-ELISA法检测200例ITP患儿和200例对照全血幽门螺杆菌(Hp)-1gG抗体和粪便Hp抗原;序列特异性引物PCR(PCR-SSP)法对HLA-DRB1基因进行分型.结果:①ITP组与对照组HLA-DRB1*07基因频率分别为6.5%和13.5%,差异有统计学意义(χ2=5.444,P=0.020).②急性ITP(AITP)与慢性ITP(CITP)患儿HLA-DRB1*11基因频率分别为9.3%和22.0%,差异有统计学意义(χ2=6.224,P均为0.003).③Hp阳性与Hp阴性ITP患儿HLA-DRB1*14和HLA-DRB1*11基因频率分别为16.0%和3.8%及22.3%和7.5%,差异有统计学意义(χ2=8.602和8.794,P均=0.003).结论:①HLA-DRB1*07可能对儿童ITP的发病具有保护作用.②具有HLA-DRB1*11基因型的患儿易发展为CITP.③HLA-DRB1*14和HLA-DRB1*11基因可能是儿童ITP患儿Hp感染的易感因素.

  7. Idiopathic Thrombocytopenic Purpura Misdiagnosed as Hereditary Angioedema

    DEFF Research Database (Denmark)

    Andersen, Michelle Fog; Bygum, Anette

    2015-01-01

    Hereditary angioedema is a rare, but potentially life-threatening genetic disorder that results from an autosomal dominant trait. It is characterized by acute, recurrent attacks of severe local edema, most commonly affecting the skin and mucosa. Swelling in hereditary angioedema patients does...... of hereditary angioedema. The case illustrates how clinicians can have difficulties in handling patients with rare diseases, especially in the emergency care setting....

  8. Immune thrombocytopenic purpura following Varicella zoster infection

    Directory of Open Access Journals (Sweden)

    Alper Dai

    2011-03-01

    Full Text Available Although thrombocytopenia is a rarely observed complicationfollowing chickenpox, it can lead to serious bleedingproblems. In order to underline rare hematologiccomplications of varicella infection and the importanceof vaccination, here we reported a seven year old boywho developed severe thrombocytopenia duringvaricella infection and gave good response to intravenousimmunoglobulin therapy. J Clin Exp Invest 2011;2(1: 85-87

  9. The geoepidemiology of immune thrombocytopenic purpura.

    Science.gov (United States)

    Deane, Sean; Teuber, Suzanne S; Gershwin, M Eric

    2010-03-01

    First described in 1735 (Watson-Williams et al., 1958), immune-mediated platelet destruction is a phenomenon of protean associations that has historically varied in its definition. Recently, consensus guidelines were proposed for a standardized system of nomenclature that preserves the acronym "ITP" but encompasses a number of causes of immune-mediated thrombocytopenias, including both primary immune thrombocytopenia as well as such entities as thrombocytopenia associated with connective tissue diseases or cancer. In this paper, we will focus on current aspects of geoepidemiology, pathophysiology, diagnosis and management of adult and pediatric primary immune thrombocytopenia. It is clear that both genetic and extrinsic factors exist for ITP and are likely different between children and adults. Immune thrombocytopenia remains a challenging problem but our understanding of its pathophysiology has greatly improved. PMID:19945546

  10. Moxifloxacin (Avelox) Induced Thrombotic Thrombocytopenic Purpura

    OpenAIRE

    Surana, Sikander P.; Zahily Sardinas; Multz, Alan S.

    2012-01-01

    We report a case of a 66-year-old African-American female who presented with complaints of progressively worsening weakness, shortness of breath on minimal exertion, lethargy for the last few days, and short episodes of aphasia lasting 20–30 seconds. Prior to presentation, she was treated with two courses of moxifloxacin for sinusitis. Laboratory examination was remarkable for anemia and thrombocytopenia with elevated lactate dehydrogenase and no evidence of renal failure. Peripheral smear sh...

  11. Microangiopatias trombóticas: púrpura trombocitopênica trombótica e síndrome hemolítico-urêmica Thrombotic microangiopathies: thrombotic thrombocytopenic purpura / hemolytic uremic syndrome

    Directory of Open Access Journals (Sweden)

    Maria Goretti Polito

    2010-09-01

    complemento. Uma série de mutações e polimorfismo em genes que codificam proteínas reguladoras do complemento sozinhas ou em combinação podem levar a SHU atípica. Aproximadamente 60% dos casos de SHU atípica têm mutações do tipo "perda da função" em genes que codificam as proteínas reguladoras do complemento, as quais protegem as células hospedeiras da ativação do complemento: fator H do complemento (FHC, fator I (FIC e proteína cofator de membrana (PCM ou CD46, ou mutações do tipo "ganho da função" em genes que codificam o FHC ou C3. Além disso, aproximadamente 10% dos pacientes com SHU atípica têm deficiência na função do FHC devido a anticorpos anti-FHC. Mesmo que as MATs sejam condições altamente heterogêneas, um terço dos pacientes tem deficiência severa da ADA-MTS13. Transfusões de plaquetas são contraindicadas nesses pacientes. Infusão de plasma ou plasma exchange (PE é o único tratamento eficiente.Thrombotic microangiopathies (TMAs are pathological conditions characterized by generalized microvascular occlusion by platelet thrombi, thrombocytopenia, and microangiopathic hemolytic anemia. Two typical phenotypes of TMAs are hemolytic- uremic syndrome (HUS and thrombotic thrombocytopenic purpura (TTP. Other disorders occasionally present with similar manifestations. Depending on whether renal or brain lesions prevail, two pathologically indistinguishable but somehow clinically different disorders have been described: HUS and TTP. Injury to the endothelial cell is the central and likely inciting factor in the sequence of events leading to TMA. Loss of physiological thromboresistance, leukocyte adhesion to damaged endothelium, complement consumption, abnormal von Willebrand factor release and fragmentation, and increased vascular shear stress may then sustain and amplify the microangiopathic process. Intrinsic abnormalities of the complement system and of the von Willebrand factor pathway may account for a genetic predisposition to the

  12. Esplenectomia vídeo-laparoscópica para púrpura trombocitopênica imune: técnica e resultados Laparoscopic splenectomy for immune thrombocytopenic purpura: technique and results of a prospective study

    Directory of Open Access Journals (Sweden)

    Ricardo Zorrón

    2004-08-01

    ópica é segura e efetiva, tornando-se o tratamento de escolha para PTI com indicação cirúrgica.BACKGROUND: Immune thrombocytopenic purpura (ITP is a common indication for splenectomy. In order to evaluate the results of Laparoscopic Splenectomy, 17 patients with ITP were submitted to this procedure in a prospective study. METHODS: Using three trocars through a posterior approach and simple inabsorbable ligatures, without using hemoclips and vascular stapplers, splenectomy was carried out in a prospective series of 17 patients. RESULTS: All patients were successfully managed laparoscopically, with no conversion to open surgery. Complications ocurred in three patients: one wound haematoma, one residual splenic tissue requiring reoperation, and one pancreatic pseudocyst that was treated by conservative measures. An additional fourth trocar was needed in four patients. Mean operative time was 132.9min, mean postoperative stay was 2.53 days. Intraoperative platelet transfusion was needed in two patients (11.8% and accessory spleen was detected in four (23.5%. Favourable sustained response to splenectomy was obtained in 13 patients (76.5%, with partial or no response in four (23.5%. CONCLUSION: Careful anatomical dissection technique and search for accessory tissue is needed to avoid splenosis and therapy failure. Detection of accessory spleens by this method is precise and reliable. Patients with PTI have the same remission rates of open surgery, with less complications and shortened postoperative stay. The results suggest that Laparoscopic Splenectomy is effective and safe, and has become the golden standard for the treatment of ITP with surgical indication.

  13. Trombotisk trombocytopenisk purpura hos barn med lavt ADAMTS13 enzymniveau

    DEFF Research Database (Denmark)

    Spangenberg, Katrine Bredsdorff; Clasen-Linde, Erik; Poulsen, Anja

    2014-01-01

    Thrombotic thrombocytopenic purpura (TTP) is a rare condition, but important to consider in case of thrombocytopenia and haemolysis. It is imperative to proceed with the correct treatment, in order to ensure a satisfactory outcome. TTP is either acquired or idiopathic. This case report shows that...

  14. Clinical observation and nursing of Chinese medicine combined with recombinant interleukin-11 in treating refractory idiopathic thrombocytopenic purpura%中药联合重组白介素-11治疗难治性特发性血小板减少性紫癜的临床观察和护理

    Institute of Scientific and Technical Information of China (English)

    李洪琴; 陈波; 江桂林; 张静静; 石焕玉; 霍长亮

    2014-01-01

    目的:探讨中药联合重组白介素-11治疗难治性特发性血小板减少性紫癜的临床疗效和护理措施。方法将52例肾性贫血难治性特发性血小板减少性紫癜患者随机分成2组,治疗组26例给予重组人白介素-11(巨和粒)治疗,皮下注射,每天1次,连用14 d,每个月为1个疗程,并口服中药汤剂(由黄芪、生地、仙鹤草、水牛角、丹皮、紫珠草等组成),日1剂,早晚分服;对照组26例只给予重组人白介素-11(巨和粒)治疗。2组均配合专科护理干预措施,均治疗2个月。结果治疗组总有效率为88.5%,对照组为65.4%,2组比较差异有统计学意义(P <0.05)。结论中药联合重组白介素-Ⅱ对难治性特发性血小板减少性紫癜有显著疗效。%ABSTRACT:Objective To explore the clinical curative effect and nursing of Chinese medicine combined with recombinant interleukin-2 in treating refractory idiopathic thrombocytopenic purpu-ra.Methods 52 renal anemia refractory idiopathic thrombocytopenic purpura patients were ran-domly divided into treatment group and control group.The treatment group with 26 cases was given subcutaneous injection of recombinant human interleukin-2(Juhe Granule)for 1 time daily,con-tinuous treatment lasted for 14 days per month and 1 months was a course of treatment.Besides, Chinese medicine decoction(composed of radix astragali,radix rehmanniae,herba agrimoniae,buf-falo horn,cortex moutan,callicarpa macrophylla vahl,etc)was orally taken for 1 agent daily for 2 times in the morning and evening.The control group with 26 cases was given only recombinant hu-man interleukin-2 (Juhe Granule).The course of the treatment in the two groups was lasted for 2 months and nursing intervention measures were conducted during the treatment.Results The ef-fective rate was 88.5% in the treatment group and 65.4% in the control group.Comparison of the two groups showed that the

  15. Purpura fulminans

    DEFF Research Database (Denmark)

    Jordan, Karina; Kristensen, Kim

    2010-01-01

    Varicella-associated purpura fulminans (PF) is a rare complication to varicella infection. The condition is due to autoantibodies directed against protein S which forms part of the anticoagulation system. Lack of protein S leads to disseminated intravascular coagulation in the small vessels, which...

  16. Platelet alloimmunization after transfusion

    DEFF Research Database (Denmark)

    Taaning, E; Simonsen, A C; Hjelms, E;

    1997-01-01

    BACKGROUND AND OBJECTIVES: The frequency of platelet-specific antibodies after one series of blood transfusions has not been reported, and in multiply transfused patients is controversial. MATERIALS AND METHODS: We studied the frequency of alloimmunization against platelet antigens in 117 patients...... who received a single series of blood transfusions. They received mostly saline-adenine-glucose+mannitol red blood cell components (poor in leukocytes and platelets) in connection with cardiac surgery. Platelet-specific antibodies were detected with the platelet ELISA and the monoclonal...... (17.9%), of whom 18 (15.4%) had had no detectable antibodies before transfusion. There was a positive correlation between the transfused load of immunogenic materials and the frequency of alloimmunization against HLA antigens. In one third of the immunized patients, there was no history of previous...

  17. Progress of CD4 + CD25 + regulatory T cells in pathogenesis of idiopathic thrombocytopenic purpura%CD4+CD25+调节性T细胞在特发性血小板减少性紫癜发病机制中的作用

    Institute of Scientific and Technical Information of China (English)

    刘小群

    2011-01-01

    CD4 + CD25 + regulatory T cells(Treg) are thought to be a subgroup of mature CD4 + T cells.Forkhead winged helix transcription factor-3 (Foxp3)is specifically expressed on them and plays a key role in their development and function. CD4 + CD25 + Treg cells can maintain the stabilization of internal environment by two principal pathways to suppress the immunological function: the direct suppression of the target cells by cell-contact and the secretion of suppressor cytokines. At present, it has been considered that decreased number and dysfunction of CD4+ CD25+ Treg cells are closely related to pathogenesis of autoimmune disease. Recent findings show that CD4+ CD25+ Treg cells play an important role in pathogenesis of idiopathic thrombocytopenic purpura.%CD4+ CD25+调节性T细胞(regulatory T cell,Treg)是一种成熟的CD4+T细胞亚群,而叉头翼状螺旋转录因子3(forkhead winged helix transcription factor-3,Foxp3)特异性表达于该细胞上,对其发育和功能起关键作用。CD4+ CD25+ Treg细胞主要通过直接接触和分泌抑制性细胞因子两大途径发挥免疫抑制功能,维持机体内环境的稳定。目前认为其数目减少和功能障碍与自身免疫性疾病的发生密切相关。近年来研究表明CD4+ CD25+ Treg细胞在特发性血小板减少性紫癜的发病中起重要作用。

  18. Preoperative embolization of the splenic artery in patients that underwent splenectomy for immune thrombocytopenic purpura Embolização pré-operatória da artéria esplênica em pacientes submetidos à esplenectomia por púrpura trombocitopênica immune

    Directory of Open Access Journals (Sweden)

    PlínioCarlos Baú

    2007-12-01

    Full Text Available Transfusion of platelets, red blood cells, or both is usually necessary immediately after splenic artery ligature in patients with immune thrombocytopenic purpura who undergo splenectomy. PURPOSE: To investigate whether preoperative embolization of the splenic artery reduced the need for transfusion of platelets, red blood cells, or both. METHODS: Twenty- seven consecutive patients that underwent splenectomy for purpura between October 1999 and March 2006 performed by the same surgical team were enrolled. The first 17 patients did not undergo embolization and were compared with the next 10 patients, who composed the embolization group. RESULTS: The platelet count in the embolization group rose from a mean 7000 u/µl before to 75000 u/µl after the procedure. There was no need for platelet or red blood cell transfusion in the embolization group; in the group without preoperative embolization, 11 patients (p=0.001 required platelet transfusion and 8 (p=0.01, red blood cell transfusion. CONCLUSION: Embolization of the splenic artery before splenectomy is a safe method to avoid blood transfusions in patients with ITP.A transfusão de plaquetas e ou hemácias geralmente é realizada em pacientes submetidos a esplenectomia por Purpura Trombocitopênia Imune (PTI. OBJETIVO: Investigar se a embolização pré-operatória da artéria esplênica é eficaz na redução da necessidade de transfusão de hemácias ou plaquetas. MÉTODOS: Vinte e sete pacientes foram submetidos a esplenectomia por PTI de Outubro de 1999 a Março de 2006 pela mesma equipe cirúrgica. Os primeiros 17 pacientes não foram submetidos a embolização e foram comparados com os outros 10 individuos nos quais a embolização foi realizada. RESULTADOS: A contagem de plaquetas no grupo em que a embolização foi realizada subiu de uma média de 7000u/µl antes do procedimento, para 75000 u/µl após. Não foi necessário transfundir plaquetas ou glóbulos vermelhos no grupo submetido a

  19. The Role of Amifostine in Treating Idiopathic Thrombocytopenic Purpura%氨磷汀在治疗特发性血小板减少性紫癜中的作用

    Institute of Scientific and Technical Information of China (English)

    靳英; 李明阳; 刘波; 张伟; 朱宏丽

    2012-01-01

    特发性血小板减少性紫癜(ITP)并不少见,该病诊断不难,治疗方法多.但本例患者高龄、既往治疗出现副作用,用药受限,常规药物效果不佳,经氨磷汀治疗而使患者得以痊愈,目前已随访观察5个月无复发.结合文献,氨磷汀对高龄、难治性病例安全有效,提示氨磷汀可能是ITP治疗中一种较有潜力的药物.%This is to discuss Amifostine mechanism by analyzing one case with Idiopathic Thromhocytopenic Purpura (ITP). ITP is not difficult to diagnozed. Many drugs are used to treat it. One 92 —year—old male patient with 1TP was not ameliorated by routine treatment. Finally he was cured by Amifostine . Then he was followed —up for 5 months without recurrence. We can say that Amifostine is safe and effective, further more it is possible a potentional medicine to cure elderly serious ITP.

  20. Association of human parvovirus B19 infection and childhood idiopathic thrombocy-topenic purpura: a meta analysis of Chinese literatures%人微小病毒B19感染与儿童特发性血小板减少性紫癜关系的meta分析

    Institute of Scientific and Technical Information of China (English)

    张耀东; 胡群; 刘双又; 刘爱国; 王冠玲; 熊昊; 孙燕

    2009-01-01

    Objective To study the relationship between human parvovirus B19 infection and childhood idiopathic thrombocytopenic purpura ( FTP) by the principle of evidence based medicine. Methods Papers related to the relationship between human parvovirus B19 infection and childhood ITP published between 1994 and 2008 were retrieved electronically from the Chinese Journals Full-text Database and the Wanfang Data. These relevant papers on case-control trials were statistically studied by meta analysis. Results Eight papers that met the inclusion criteria were included for this meta analysis. Five hundred and sixteen cases of childhood ITP and 246 healthy controls were enrolled. The meta analysis showed that the incidence of human parvovirus B19 infection in the ITP group was significantly higher than that in the control group ( OR = 13. 71, 95% CI = 7. 07-26. 59, Z = 7. 75, P < 0. 01). Conclusions Human parvovirus B19 infection is closely associated with childhood ITP.%目的 综合分析人微小病毒B19感染与儿童特发性血小板减少性紫癜(ITp)的关系.方法 计算机检索中国期刊全文数据库、万方数据库,检索时间从1994~2008年,纳入人微小病毒B19感染与儿童ITP关系的病例对照研究,对文献数据进行meta分析.结果 共纳入8个研究,其中病例组516例,对照组246例.meta分析显示人微小病毒B19感染在病例组的表达高于对照组(OR=13.71,95% CI=7.07~26.59,Z=7.75,P<0.01).结论 人微小病毒B19感染与儿童ITP的发病有密切相关性.

  1. Fetal/Neonatal Alloimmune Thrombocytopenia: Pathogenesis, Diagnostics and Prevention.

    Science.gov (United States)

    Brojer, Ewa; Husebekk, Anne; Dębska, Marzena; Uhrynowska, Małgorzata; Guz, Katarzyna; Orzińska, Agnieszka; Dębski, Romuald; Maślanka, Krystyna

    2016-08-01

    Fetal/neonatal alloimmune thrombocytopenia (FNAIT) is a relatively rare condition (1/1000-1/2000) that was granted orphan status by the European Medicines Agency in 2011. Clinical consequences of FNAIT, however, may be severe. A thrombocytopenic fetus or new-born is at risk of intracranial hemorrhage that may result in lifelong disability or death. Preventing such bleeding is thus vital and requires a solution. Anti-HPA1a antibodies are the most frequent cause of FNAIT in Caucasians. Its pathogenesis is similar to hemolytic disease of the newborn (HDN) due to anti-RhD antibodies, but is characterized by platelet destruction and is more often observed in the first pregnancy. In 75 % of these women, alloimmunization by HPA-1a antigens, however, occurs at delivery, which enables development of antibody-mediated immune suppression to prevent maternal immunization. As for HDN, the recurrence rate of FNAIT is high. For advancing diagnostic efforts and treatment, it is thereby crucial to understand the pathogenesis of FNAIT, including cellular immunity involvement. This review presents the current knowledge on FNAIT. Also described is a program for HPA-1a screening in identifying HPA-1a negative pregnant women at risk of immunization. This program is now performed at the Institute of Hematology and Transfusion Medicine in cooperation with the Department of Obstetrics and Gynecology of the Medical Centre of Postgraduate Education in Warsaw as well as the UiT The Arctic University of Norway. PMID:26564154

  2. Safety and Efficacy Study of Romiplostim to Treat ITP in Pediatric Subjects

    Science.gov (United States)

    2016-01-13

    Idiopathic Thrombocytopenic Purpura; Thrombocytopenia; Thrombocytopenia in Pediatric Subjects With Immune (Idiopathic) Thrombocytopenic Purpura (ITP); Thrombocytopenia in Subjects With Immune (Idiopathic) Thrombocytopenic Purpura (ITP); Thrombocytopenic Purpura; Immune Thrombocytopenia

  3. Púrpura trombocitopênica trombótica - remissão completa em paciente com mau prognóstico após tratamento com plasmaférese terapêutica e rituximabe Successful outcome in poor-prognostic acute thrombotic thrombocytopenic purpura treated with plasma exchange and rituximab

    Directory of Open Access Journals (Sweden)

    Cesar de Almeida Neto

    2008-02-01

    Full Text Available A púrpura trombocitopênica trombótica (PTT é uma doença rara e fatal que deve ser diagnosticada e tratada prontamente a fim de se obter melhor resposta terapêutica. Apresentamos um caso de PTT aguda grave tratada com plasmaférese e rituximabe. Ao diagnóstico, a paciente apresentava anemia hemolítica microangiopática, icterícia, febre, convulsões, seguidas por coma e choque hipovolêmico. Os exames laboratoriais iniciais mostravam DHL=2.860 IU/L, contagem de plaquetas de 37 x 10(9/L, hemoglobina de 5,1 g/dL e no esfregaço de sangue periférico havia a presença de esquizócitos. Iniciado tratamento para PTT com pulsoterapia com metilprednisolona e plasmaféreses terapêuticas diárias com troca de uma volemia plasmática e substituição com plasma fresco congelado. Após cinco sessões de plasmaférese, houve piora no quadro neurológico, acompanhado por aumento importante de DHL, ALT, AST e a contagem de plaquetas era de 72 x 10(9/L. Iniciamos o uso de rituximabe na dose padrão de 375mg/m²/semana/4 semanas e passamos a utilizar plasma pobre em crioprecipitado como reposição durante as plasmaféreses. Dois dias após a mudança na conduta terapêutica, houve importante melhora do quadro neurológico, estabilização da contagem de plaquetas e queda acentuada de DHL. Após 23 procedimentos de plasmaférese e quatro doses de rituximabe, a paciente apresentou remissão completa, mantida há 34 meses. A plasmaférese terapêutica com plasma pobre em crioprecipitado e o uso concomitante de rituximabe foi uma estratégia útil no tratamento deste caso de PTT aguda grave. Porém, ensaios clínicos prospectivos e randomizados são necessários para confirmar estes achados.Thrombotic thrombocytopenic purpura (TTP is a rare severe disease that must be diagnosed and treated promptly for a successful outcome. We report a case of severe acute TTP treated with plasma exchange and rituximab. The patient presented at diagnosis with severe

  4. 巨细胞病毒感染与儿童免疫性血小板减少性紫癜的关系探讨%Exploration of the relationship between human cytomegalovirus infection and immune thrombocytopenic purpura in children

    Institute of Scientific and Technical Information of China (English)

    颜慕霞; 张力; 林涛

    2011-01-01

    Objective To explore the relationship between human cytomegalovirus (HCMV) infection and immune thrombocytopenic purpura (ITP) in children. Methods HCMV DNA in serum samples from 154 cases with ITP(ITP groups)and 50 healthy children (control groups) was detected by Real-time PCR. HCMV IgM and HCMV IgC were tested by ELISA simultaneously. Urine specimens from 105 cases in ITP group and 50 children in control group were collected for the detection of HCMV DNA by Real-time PCR. At the same time, amount of platelet (PLT) in HCMV DNA positive children was compared with that of the HCMV DNA negative ones for the ITP groups. Results The positive rates of HCMV DNA, HCMV IgM and HCMV IgG in serum samples in the ITP group were higher than those in the control group, and the HCMV DNA in urine specimens was also found in more cases in the ITP group. There was significant difference between both groups (P<0.01). There were significant difference of PLT between HCMV DNA positive children and the negative ones in the ITP group (P<0.05).Conclusion HCMV infection may be an important pathogenic factor in ITP. This finding is important for the treatment and prevention of ITP.%目的 探讨人巨细胞病毒(HCMV)感染与儿童免疫性血小板减少性紫癜(ITP)的关系.方法 采集154例ITP患儿(ITP组)和50例健康儿童(对照组)的血清样本,用Real-time PCR检测HCMV DNA,ELISA方法检测HCMV IgM、IgG抗体;其中105例ITP患儿和50例健康对照儿童采集了尿液标本,用Real-time PCR检测HCMV DNA.并比较ITP组HCMV DNA阳性患儿与阴性患儿的血小板数量的差异.结果 ITP患儿血清HCMV DNA、HCMV IgM及IgG抗体和尿HCMV DNA阳性率均明显高于健康对照儿童,两组比较差异均有统计学意义(P<0.01);ITP组HCMV DNA阳性患儿的血小板数量(29.72±14.54)x10~9/L与阴性患儿(41.28±18.35)x10~9/L比较差异有统计学意义(P<0.05).结论 儿童感染HCMV可能是发生ITP的重要致病因素之一,这对指导临床有

  5. 特发性血小板减少性紫癜患者血清白细胞介素11水平、淋巴细胞亚群及NK细胞变化的临床意义%Clinical significance of the serum level interleukin-11, lymphocyte subsets and NK cells in idiopathic thrombocytopenic purpura patients

    Institute of Scientific and Technical Information of China (English)

    张红梅; 李艳敏

    2011-01-01

    目的 检测特发性血小板减少性紫癜(ITP)患者血清白细胞介素11(IL-11)水平、淋巴细胞亚群及NK细胞的变化,探讨相关因素在ITP发病中的作用.方法 应用酶联免疫吸附法(ELISA)、流式细胞术分别检测50例ITP(ITP组)和30例健康体检者(对照组)血清IL-11水平、淋巴细胞亚群及NK细胞的变化.结果 ITP组的血小板计数[(30.21±19.40)×109/L]明显低于对照组[(207.21±31.55)×109/L](P<0.05),ITP组患者血清IL-11水平[(255.72±163.43)ng/L]明显高于对照组[(40.60±5.57)ng/L](P<0.05),相关分析表明ITP患者血清IL-11水平与血小板计数呈负相关(r=-0.557,P<0.05);ITP组患者CD3+、CD4+T淋巴细胞百分比及CD4+/CD8+明显低于对照组(P<0.05),CD8+T淋巴细胞百分比明显高于对照组(P<0.05);CD3-CD(16+56)+NK细胞百分比明显低于对照组(P<0.05).结论 IL-11水平、淋巴细胞亚群及NK细胞变化与ITP的发病密切相关,且IL-11水平与血小板计数可能存在负反馈调节作用.%Objective To detect the serum level of interleukin (IL)-1 1, lymphocyte subsets and NK cells in patients with idiopathic thrombocytopenic purpura (ITP), and explore the related factors in the pathogenesis of ITP. Methods The serum level of IL-11, lymphocyte subsets and NK cells were detected by double antibody sandwich enzyme linked immunosorbent assay (ELISA) and flow cytometry in 50 ITP patients (ITP group) and 30 controls (control group). Results The platelet in ITP group [ (30.21 ± 19.40) ×109/L] was lower than that in control group [ (207.21 ± 31.55 ) × 109/L] obviously (P < 0.05 ); the serum level of IL-11 in ITP group [(255.72 ± 163.43) ng/L] was significantly higher than that in control group [ (40.60 ± 5.57 ) ng/L ] (P < 0.05 ). The correlation analysis indicated that the blood serum levels of IL- 11 had negative relationship with the platelet (r = -0.557 ,P < 0.05). The percentage of CD3+ and CD4+ T lymphocyte percentage, CD4+/CD8+ in ITP

  6. IL-23及其相关细胞因子在慢性特发性血小板减少性紫癜中的表达与作用探讨%Expression and Significance of Interleukin-23 and Its Related Cytokines in Chronic Idiopathic Thrombocytopenic Purpura

    Institute of Scientific and Technical Information of China (English)

    黄颖; 李永志; 魏彩霞; 黎承平; 李维佳; 杨弘

    2011-01-01

    The aim of this study was to investigate the expression and immunologic regulation function of interleukin23 and its related cytokines in chronic idiopathic thrombocytopenic purpura(ITP) patients. Levels of cytokines in peripheral blood mononuclear cells(PBMNC) were detected by reverse-transcription real-time polymerase chain reaction in 30 patients with chronic ITP and 15 healthy volunteers. The quantity of IL-23, IL-12, IL-17 in serum was detected by enzyme-linked immunosorbent assay(ELISA). The results showed that low detectable mRNA levels of IL-23p19 ,IL-12p35,IL-27 and IL-12p40 were found in all patients and healthy persons. Trace of IL-17 mRNA were expressed in PBMNC of part of patients and normal controls. Levels of IL-12p35, IL-27, IL-17 mRNA between healthy persons and chronic ITP patients were not statistically different. Compared with normal controls, patients showed the lower expression levels of IL23p19 and IL-12p40 mRNA (p <0.01 ). The IL-12 levels of chronic ITP patients were significantly higher than that of normal controls (p<0.01). The IL-23 and IL-17 levels of chronic ITP patients were same to that of normal controls. It is concluded that the imbalance of T cell subsets in ITP patients mainly associated with IL-12, but not with IL-23 and IL-17.%本研究旨在探讨IL-23及其它的IL-12家族成员在慢性特发性血小板减少性萦癜(ITP)的表达及其免疫调节功能.运用反转录实时PCR方法检测30例慢性ITP患者和15例正常对照者外周血单个核细胞(PBMNC)中IL-23p19、IL-12p35、IL-12p40、IL-27、IL-17 mRNA的表达水平,用ELISA方法检测血浆IL-23、IL-12、IL-17含量并分析其在慢性ITP中的表达规律及与T亚群的关系.结果表明,慢性ITP患者及正常人PBMNC均低水平表达IL-23p19、IL-12p35、IL-27、IL-12p40 mRNA;部分患者及正常人PBMNC微量表达IL-17 mRNA.IL-12p35、IL-27、1L-17mRNA的表达水平与正常对照相比无显著差异,IL-23p19、IL-12p40 mRNA的表达水

  7. Expression of Transforming Growth Factor-β1 and Its Receptors in Peripheral Blood of Patients with Immune Thrombocytopenic Purpura%转化生长因子-β1及其受体在免疫性血小板减少性紫癜症患者中的表达及意义

    Institute of Scientific and Technical Information of China (English)

    方治; 张翼鷟; 蔡挺; 李克强; 余静; 罗央清; 赵海丰

    2012-01-01

    本研究通过检测外周血转化生长因子( TGF-β1)及其受体((TGF-βR)的表达探讨其在免疫性血小板减少性紫癜症(ITP)发病中的作用机制.以ITP患者为研究对象,健康人为对照,通过实时PCR方法检测外周血中TGF-β1及其受体(TGF-βRⅠ、TGF-βRⅡ和TGF-βRⅢ)的表达量,分析两组之间的差异.结果表明,ITP患者组TGF-β1和TGF-βRⅡmRNA的表达明显高于正常对照组,差异有统计学意义,而TGF-βR Ⅰ mRNA的表达明显低于正常对照组,差异有统计学意义.TGF-βRⅢmRNA的表达在两组间无统计学差异.结论:TGF-β1及其受体TGF-βR Ⅰ和TGF-pRⅡ在ITP患者中表达异常,表明TGF-β1信号通路在ITP患者发病中可能具有一定的作用.%This study was purposed to detect the expression of transforming growth factor β1 (TGF-β1 ) and its receptors (TGF-pR) and to investigate their roles in pathogenesis of immune thrombocytopenic purpura (ITP). The expressions of TGF-β1 and their receptors TGF-βR I , TGF-pR H and TGF-pR i in the peripheral blood of patients with FTP and healthy persons were detected by the real-time PCR, and differences of their expression levels were analysed. The results showed that the expression of TGF-&1 and TGF-βR II mRNA in ITP patients was significantly higher than that in the healthy controls, while the TGF-pR I mRNA expression in ITP patient was significantly lower than that in the controls. The expression of TGF-βR HI was not statistically different between the two groups. It is concluded that TGF-βl and its receptors including TGF-pR I and TGF-βR II express abnormally in the peripheral blood of ITP patients, which suggests that the TGF-p signaling pathway probably play a vital role in the pathogenesis of the ITP.

  8. 地塞米松冲击疗法治疗成人原发免疫性血小板减少症的疗效观察%The Efficacy Observation of Dexamethasone Impact Therapy in the Treatment of Immune Thrombocytopenic Purpura

    Institute of Scientific and Technical Information of China (English)

    张付华; 刘珍

    2014-01-01

    目的:观察地塞米松冲击疗法治疗成人原发免疫性血小板减少症(ITP)的临床疗效。方法:自2006年7月-2013年9月间笔者所在医院收治成人ITP患者38例,患者均给予胸腺肽α1联合大剂量维生素C治疗,其中19例同时应用4 d地塞米松冲击治疗,观察临床症状、血小板计数和不良反应。结果:胸腺肽α1+大剂量维生素C治疗组总有效率78.9%,平均起效时间(16.8±3.2)d;胸腺肽α1+大剂量维生素C联合地塞米松冲击治疗组总有效率84.2%,平均起效时间(6.7±2.7)d。两种治疗方案的不良反应均较轻微。结论:胸腺肽α1联合大剂量维生素C与冲击量地塞米松联合应用治疗成人ITP疗效较好,副作用少,可缩短起效时间。%Objective:To observe the curative effect of Dexamethasone impact therapy to the immune thrombocytopenic purpura(ITP).Method:38 ITP patients were all given thymosin alpha 1(Tα1)combined high-dose vitamin C,among them 19 patients were companied with flushing dose dexamethasone during the first 4 medicative days.The clinic syndrome,platelet counts and side effects were observed.Result:The total effective rate in Tα1 combined high-dose vitamin C therapy group reached 78.9%,the average onset time were(16.8±3.2)d;the total effective rate in Tα1 combined high-dose vitamin C and flushing dose dexamethasone therapy group was 84.2%,the average onset time were(6.7±2.7)d.The side effects of the two groups were slight.Conclusion:Tα1 combined high-dose vitamin C and flushing dose dexamethasone has good curative effect and fewer side effects on adult ITP;it can also act fsatly.

  9. 吸附无细胞百白破灭活脊髓灰质炎和b型流感嗜血杆菌(结合)联合疫苗相关婴儿血小板减少性紫癜%Thrombocytopenic purpura caused by acellular pertussis,diphtheria,tetanus,inactivated poliomye-litis,haemophilus influenza type b conjugate vaccine in an infant

    Institute of Scientific and Technical Information of China (English)

    孙琦; 张亚明

    2015-01-01

    A 88-day-old boy was vaccinated with the acellular pertussis,diphtheria,tetanus, inactivated poliomyelitis,hemophilus influenza type b conjugate vaccine. Twenty-five days later,scattered red rash occurred on his face,both legs and hip,which had asymmetric distribution and not faded when pressed. His platelet count(PLT)was 18 × 109 / L. Idiopathic thrombocytopenic purpura was diagnosed. He was given oral prednisone 5 mg thrice daily. The next day,the PLT was 3 × 109 / L. He was treated with IV infusions of dexamethasone 5 mg,human immunoglobulin 5 g and vitamin C 1 g,then an intravenous injection of vitamin K1 5 mg. Three days later,the purpura basically subsided,the PLT was 290 × 109 / L. Follow up of 1. 5 years showed that his PLT had no abnormal changes.%1例出生后88 d 男婴接种吸附无细胞百白破灭活脊髓灰质炎和 b 型流感嗜血杆菌(结合)联合疫苗后25 d,面部、双下肢及臀部出现散在暗红色皮疹,不对称分布,压之不褪色,血小板计数(PLT)为18×109/ L。诊断:特发性血小板减少性紫癜。给予泼尼松5 mg 口服,3次/ d。次日复查, PLT 3×109/ L。给予地塞米松5 mg、人免疫球蛋白5 g、维生素 C 1 g 静脉滴注,维生素 K15 mg 静脉注射。3 d 后,患儿紫癜基本消退,PLT 290×109/ L。随访至1.5岁,患儿 PLT 未见异常。

  10. Fireogfyrre svangerskaber med idiopatisk trombocytopenisk purpura

    DEFF Research Database (Denmark)

    Clausen, Tine Dalsgaard; Jønsson, Viggo; Wiik, Allan;

    2002-01-01

    INTRODUCTION: The aim of this project was to describe the course of pregnancy with idiopathic thrombocytopenic purpura (ITP) and to estimate risk factors and indications for treatment. MATERIAL AND METHODS: Birth, haematological, and neonatal files were examined retrospectively. RESULTS: Forty......-eight ITP women with 55 pregnancies gave birth to 61 children, 59 live-born. The first singleton pregnancy in the observation period (the index pregnancy) was used for statistics, namely 44 index pregnancies. A maternal platelet fall from the first trimester to delivery was seen, as was a platelet rise...... with thrombocytopenia, severe maternal thrombocytopenia, male gender. The nadir platelet count in the newborn infants was seen up to seven days after delivery. The presence of an older sibling with neonatal ITP is a risk factor for neonatal ITP in subsequent pregnancies. A significant association was found between...

  11. [Multi-facetted clinical presentation of thrombotic thrombocytopenic purpura

    DEFF Research Database (Denmark)

    Niemann, C.U.; Jurlander, J.; Daugaard, G.;

    2009-01-01

    smears. Determination of the ADAMTS13-activity is now becoming available as a routine analysis. We present two cases that illustrate the multi-facetted clinical presentation under which TTP occurs. The importance of access to ADAMTS13 measurements is stressed Udgivelsesdato: 2009/1/26...

  12. Pulmonary Hyalinizing Granuloma Associated with Idiopathic Thrombocytopenic Purpura

    OpenAIRE

    Christopher Coleman; Aziza Nassar; Barbara McComb

    2014-01-01

    Pulmonary hyalinizing granuloma (PHG) is a rare, benign lung disease of unknown etiology. It manifests as discrete, rounded nodules within the lung parenchyma. A 39-year-old woman presented for investigation after pulmonary nodules were found incidentally. Chest computed tomography showed multiple, discrete, non-enhancing pulmonary nodules bilaterally. Positron emission tomography (PET) was negative. Biopsy demonstrated a non-specific lymphoplasmacytic infiltrate. Open resection yielded two n...

  13. Supraclavicular lymph node tuberculosis presenting with immune thrombocytopenic purpura

    Institute of Scientific and Technical Information of China (English)

    LU Hua; WANG Yong-ren; JI Ou; XU Wei; ZHANG Jian-fu; FAN Qin-he; LI Jian-yong

    2007-01-01

    @@ Tuberculosis (TB) differs from many other infectious maladies in having particular social and geographic distributions. The disease was under control in developed nations and being brought under control in developing countries, as in China.

  14. Ludwig's angina after severe thrombocytopenic purpura associated with dengue fever

    Directory of Open Access Journals (Sweden)

    Maria Antonia Campos

    2014-01-01

    Full Text Available Here, we report a case of Ludwig's angina, which required surgery because of toothache. The patient had dengue and severe thrombocytopenia as confirmed by clinical and laboratory diagnoses. However, dengue is not included among the predisposing factors for Ludwig's angina.

  15. Ny markør ved trombotisk trombocytopenisk purpura

    DEFF Research Database (Denmark)

    Gøtze, Jens Peter; Hillarp, Andreas; Lindblom, Anders;

    2008-01-01

    Thrombotic microangiopathy can be caused by several conditions which are difficult to diagnose from the clinical presentation alone. Deficient enzyme activity of a newly-discovered enzyme, ADAMTS-13, can lead to thrombotic thrombocytopenic purpura (TTP). Lack of ADAMTS-13 activity causes increased...... concentrations of high molecular weight von Willebrand factor forms and increased platelet aggregation. Measurement of ADAMTS-13 activity is useful for the diagnosis of TTP and may also be relevant as a prognostic test for recurrent TTP. Udgivelsesdato: 2008-Aug-11...

  16. Thrombosis warning in children suffering from Henoch-Schonlein Purpura

    Directory of Open Access Journals (Sweden)

    Luan Li

    2013-01-01

    Full Text Available Henoch-Schonlein Purpura (HSP is one of the most common types of vasculitis in children. The characteristic clinical manifestations include non-thrombocytopenic purpura, arthritis or arthralgia, abdominal pain, gastrointestinal hemorrhage and renal abnormalities. Thrombosis has been reported as, a rare complication of HSP. We present the case of a 14-year-old boy who was diagnosed with HSP and suspected superior mesenteric vein thrombosis. We reviewed the relevant literature and found eight similar reported cases. HSP is associated with thrombosis and HSP itself and some risk factors may result in thrombosis. We suggest that physicians should monitor patients with HSP who are at a higher risk of developing thrombosis more closely.

  17. Comparison of dexamethasone and Anti-D Immune globulin for immune thrombocytopenia purpura in children

    OpenAIRE

    Abdollah Banihashem; Hamid Farhangi; Mojtaba Mousavi Bazaz; Zahra Badiee; Ali Ghasemi; Sara Hesari

    2014-01-01

    Different therapeutic options in children with immune thrombocytopenic purpura include observation alone, periodic treatment with corticosteroids, intravenous immunoglobulin (IVIG) or anti-D, chronic administration of immunosuppressive agents, and splenectomy. Preference of the type of therapy depends on the degree of thrombocytopenia and clinical bleeding manifestations. Dexamethasone is safe but its side effects are the main disadvantages for its usage. Anti-D is more expensive than dexamet...

  18. 特发性血小板减少性紫癜患儿血清IL-6,sIL-2R的变化及意义%Changes of serum IL-6 and sIL-2R levels in patients with idiopathic thrombocytopenic purpura and their clinical significance

    Institute of Scientific and Technical Information of China (English)

    张国利; 张剑白; 蒋丽鑫

    2009-01-01

    Objective To study the changes and the clinical significance of serum IL-6 and sIL-2R levels in children with idiopathic thrombocytopenic purpura( ITP). Methods The levels of serum IL-6 and sIL-2R were measured in 37 ITP children with sandwich ELSIA as well as in 20 healthy children. At the same time, PAIb' s qualitative determination was checked in 25 ITP children. And the relationships of PLT counts,IL-6 and sIL-2R's level in ITP children' s peripheral blood were analyzed. Results The levels of ITP children' s IL-6 and sIL-2R were significantly higher than those of control group(P <0. 01). The levels of IL-6 and sIL-2R were dramatically higher in the group of ITP children before treatment responsive to after treatment(P <0.01 ). The levels of severe hemorrhage group' s IL-6, sIL-2R were significantly higher than those of hyporrhea group' s( P < 0.05 ). Nine of 25 ITP children' s PAIb was completely positive(9/25) ,the levels of ITP children's IL-6, sIL-2R in completely positive group were significantly higher than non-positive group' s( P < 0. 05 ). The levels of ITP children' s IL-6, sIL-2R ( before treating) were negative correlation with PLT counts. The level between ITP children' s IL-6 and sIL- 2R was positive correlation. Conclusion PIL-6 and sIL-2R participate in ITP' s procedure. The levels change with the pathogenetic condition and the course of disease. To sum up, cell mediated immunity plays a role in ITP' s procedure as well as humoral-mediated immunity.%目的 探讨特发性血小板减少性紫癜(ITP)患儿血清中细胞因子白介素6(IL-6)、可溶性白介素2受体(sIL-2R)的变化及临床意义.方法 采用双抗体夹心酶联免疫(ELISA)定量测定方法检测37例ITP患儿和20例正常健康儿童血清中IL-6、sIL-2R水平,同时对其中25例ITP患儿进行血小板相关抗体PAIb(IgG、IgA、IgM)的定性测定,并对ITP患儿外周血中血小板计数、血清IL-6、sIL-2R水平进行相关分析.结论 ITP患儿血清IL-6

  19. Expression of peripheral Blood lymphocyte subpopulation in patients with acute and chronic idiopathic thrombocytopenic purpura%急慢性特发性血小板减少性紫癜患者外周血淋巴细胞亚群的表达

    Institute of Scientific and Technical Information of China (English)

    张红; 刘庆华; 田芳

    2014-01-01

    目的:检测急慢性特发性血小板减少性紫癜(ITP)患者外周血淋巴细胞亚群,并探讨其在发病机制中的作用。方法选择74例ITP患者及30例健康查体人员的外周血,采用流式细胞术检测淋巴细胞亚群(CD3+, CD3+CD4+, CD3+CD8+, CD4+/CD8+, CD19+)。结果①ITP患者淋巴细胞亚群CD3+CD4+为(34.15±8.55)%,正常对照组为(36.74±3.65)%, t=2.166, P=0.033;ITP患者CD4+/CD8+为(1.24±0.44)%,正常对照组为(1.48±0.25)%, t=3.582, P=0.001;ITP患者CD3+CD8+为(30.63±7.72)%, CD19+为(18.23±7.67)%,均显著高于正常对照组(P=0.000)。②急性ITP患者外周血CD19+细胞比例为(22.6±7.25)%,慢性ITP患者为(14.14±5.57)%,两者差异有统计学意义(t=5.677, P=0.000);而慢性ITP患者的CD3+细胞的比例为(76.02±11.00)%,急性ITP患者为(66.82±10.95)%, t=3.583, P=0.001。结论 ITP的发生不仅存在B淋巴细胞异常,也存在T淋巴细胞的异常。急性ITP患者以体液免疫功能亢进为主,慢性患者则表现为细胞免疫功能亢进为主。%Objective To detect the expression of peripheral blood lymphocyte subpopulation in patients with acute and chronic idiopathic thrombocytopenic purpura (ITP), and to investigate its influence on pathogenesis. Methods The expression of lymphocyte subpopulation (CD3+, CD3+CD4+, CD3+CD8+, CD4+/CD8+, CD19+) in the peripheral blood was analyzed using flow cytometry in 74 patients with ITP and 30 healthy people. Results ①CD3+CD4+of lymphocyte subpopulation in patients with ITP was (34.15±8.55)%, while that of the control group was (36.74±3.65)%(t=2.166, P=0.033). CD4+/CD8+of lymphocyte subpopulation in patients with ITP was (1.24±0.44)%, and that of the control group was (1.48±0.25)% (t=3.582, P=0.001). CD3+CD8+ and CD19+of lymphocyte subpopulation in patients with ITP were (30.63±7.72)%and (18.23±7.67)%, and they were all obviously higher than the control group (P=0.000).②The proportion of CD19+B cells in peripheral Blood of acute

  20. 特发性血小板减少性紫癜患儿血小板膜糖蛋白表达及临床意义%Expression of platelet membrane glycoproteins in pediatric idiopathic thrombocytopenic purpura and its clinical significance

    Institute of Scientific and Technical Information of China (English)

    王娜; 柴忆欢; 何海龙; 李建琴; 曹岚; 曹丽娟

    2013-01-01

    Objective To explore the relationship between the expression of platelet membrane glycoprotein in pediatric idiopathic thrombocytopenic purpura (ITP) and its clinical significance.Methods A modified monoclonal antibody immobilization of platelet antigen (MAIPA) method was used to detect the positive expression rates of 4 platelet membrane glycoproteins (GP Ⅰ b/Ⅸ,GP Ⅰ b,GP Ⅲ a,and GP Ⅰ b) in 80 pediatric patients with ITP.The correlation was explored between the GP positive rate and the clinical efficacy in pediatric ITP.Trying to observe the correlationship between the GP positive rate of pediatric ITP in the total,the different gender,the acute and chronic and the treatment response rate in pediatric ITP respectively.Results There was a significant difference in curative rate statistically between the GP positive group and the GP negative group(x2 =8.535,P < 0.01) in 80 pediatric ITP patients,but no statistic difference in curative rate existed between the 36 female and 44 male(x2 =0.013,P >0.05).Markedly statistic difference was found in the female(x2 =4.433,P < 0.05),the same to the male (x2 =4.156,P < 0.05).Meanwhile,there was an extremely statistic difference between 67 acute and 13 chronic patients(x2 =23.513,P < 0.001).Apparently statistic difference also occurred in the acute (x2 =4.157,P < 0.05),but not in the chronic cases (x2 =0.410,P > 0.05).Conclusions The clinical response rate is significantly correlated with the GP positive rate in pediatric ITP,but not correlated with gender.The GP positive rate can reflect the disease status of pediatric ITP to a certain extent and be used as an indicator for judging the efficacy and monitor prognosis of pediatric ITP.%目的 检测儿童特发性血小板减少性紫癜(ITP)血小板膜糖蛋白(GP)的表达并探讨其临床意义.方法 采用改良单克隆抗体特异性俘获血小板抗原(MAIPA)法检测80例ITP患儿GP Ⅰ b/Ⅸ、GPⅡb、GPⅢa、GPⅠ b的阳性表达率.分别

  1. Changes and Signiifcance of Peripheral BloodγδT Cells and IL-17 and IL-23 Levels in Patients With Idiopathic Thrombocytopenic Purpura%成人特发性血小板减少性紫癜患者外周血γT细胞及IL-17、IL-23水平的变化和意义

    Institute of Scientific and Technical Information of China (English)

    杨国雷; 张复华; 凌奕文; 牛国敏

    2015-01-01

    目的:通过检测ITP患者外周血γδT细胞、IL-17与IL-23水平的变化,进一步探讨ITP的发病机制,为ITP的防治新途径提供理论依据。方法采用流式细胞术检测外周血γδT细胞;酶联免疫吸附试验方法检测 IL-17、IL-23。实验组分正常对照组和ITP组;ITP组分为治疗前组与治疗后组(有效组、无效组);ITP组初始均予地塞米松10 mg/d,此后口服强的松维持并逐渐减量,总疗程1个月。结果正常对照组外周血γδT、IL-17、IL-23分别为(6.41±1.76)%,(43.04±6.10)pg/ml,(34.03±3.64)pg/ml;ITP治疗前组:γδT、IL-17、IL-23分别为(14.90±2.41)%,(76.67±11.86)pg/ml,(63.06±12.08)pg/ml;治疗有效组:γδT、IL-17、IL-23分别为(7.19±1.29)%,(53.95±11.97)pg/ml,(37.12±3.89)pg/ml;治疗无效组:(12.87±2.11)%,(62.51±10.11)pg/ml,(58.42±10.08)pg/ml。结论 ITP患者外周血γδT细胞、IL-17、IL-23水平较正常人高,γδT细胞、IL-17、IL-23参与了ITP的发生发展;ITP患者治疗有效者γδT细胞、IL-17、IL-23水平下降。%Objective To explore the pathogenesis of idiopathic thrombocytopenic purpura (ITP), and provide theoretical basis for new pathways to the prevention and control of ITP, through testing γδT cells, IL-17 and IL-23 in peripheral blood of ITP patients. Methods γδT cells in peripheral blood was testing by flow cytometry. IL-17 and IL-23 in peripheral blood was testing by enzyme-linked immunosorbent assay. Experiment group was divided into normal control group and ITP patient group. ITP patient group was divided into pre-treatment group and treatment group (effective and non-effective group). ITP patient group was initially given dexamethasone 10 mg/d, following oral prednisone (reduced gradually), total course lasting a month. ResultsγδT cells, IL-17 and IL-23 in peripheral blood of normal control group were (6.41±1.76)%, (43.04± 6

  2. Comparison of anti-D immunoglobulin and high-dose intravenous immunoglobulin therapy in childhood acute idiopathic thrombocytopenic purpura:a systematic review and meta-analysis%抗-D免疫球蛋白与大剂量静脉丙种球蛋白治疗儿童急性特发性血小板减少性紫癜的meta分析

    Institute of Scientific and Technical Information of China (English)

    泥永安; 刘壮; 廖必才

    2013-01-01

    Objectives To compare the effectiveness and safety of intravenous anti-D immunoglobulin (IV anti-D) with high-dose intravenous immunoglobulin (IVIG) as initial treatments of acute idiopathic thrombocytopenic purpura (ITP) in children. Methods Randomized controlled trials comparing anti-D with high-dose IVIG in the treatment of childhood acute ITP were systematically reviewed from PubMed, Embase and Cochrane Central Register of Controlled Trials and hand-searched reference lists. The number of patients with a platelet count>20 × 109/L at 72 hours after treatment initia-tion, and the decrease in hemoglobin were the primary outcomes. The meta-analysis was performed by RevMan 5.1. Results A total of 771 relevant articles were retrieved, and ifve studies were included. The RR (anti-D versus high dose IVIG) of achieving a platelet count>20×109/L at 72 hours was 0.90 (95%CI:0.82~0.98). However, subgroup analysis suggested no signiifcant difference between anti-D at a dose of 50μg/kg and high-dose IVIG (RR 0.98, 95% CI: 0.84~1.13), as well as between anti-D at a dose of 75μg/kg and high-dose IVIG (RR 0.88, 95%CI:0.75~1.03). Hemoglobin drop was greater in the anti-D group. No patients, however, required transfusions of erythrocyte suspensions. Conclusions IV anti-D may be as ef-fective as high-dose IVIG in the treatment of childhood acute ITP at 72 hours after therapy. The side effects of anti-D were tolerated and acceptable.%  目的比较静脉注射抗-D免疫球蛋白(anti-D immunoglobulin,anti-D)与大剂量静脉注射免疫球蛋白(IVIG)治疗儿童急性特发性血小板减少性紫癜(ITP)的有效性及安全性。方法计算机检索PubMed、Embase和Cochrane Central Register of Controlled Trials。手工查阅计算机检索到的文献的参考文献目录。选取治疗72 h后血小板计数>20×109/L的百分率和血红蛋白下降值作为主要测量指标。采用RevMan 5.1对纳入文献进行meta分析。

  3. 升血小板胶囊联合激素治疗特发性血小板减少性紫癜有效性的系统评价%Effectiveness of Platelet Increasing Capsule Combined with Hormone in Treating Idiopathic Thrombocytopenic Purpura: A Systematic Review

    Institute of Scientific and Technical Information of China (English)

    叶华觅; 郝建萍; 赵芳; 王欢; 郭新红

    2013-01-01

    Objective To systematically evaluate the effectiveness of platelet increasing capsule combined with hormone in treating idiopathic thrombocytopenic purpura (ITP).Methods Randomized controlled trials (RCTs) of ITP treated by platelet increasing capsule combined with hormone were electronically searched from PubMed (1966 to 2012),The Cochrane Library (CENTRAL,Issue 3,2012),CBM (1978 to 2012),CNKI (1979 to 2012),WanFang Data (1998 to 2012),and VIP (1991 to 2012).References of included studies were also retrieved.The literature was independently screened according to exclusion and inclusion criteria by two researchers independently and meta-analysis was conducted using RevMan 5.1 software after data extraction and quality assessment.Results 10 RCTs were included involving 588 patients.The results meta-analysis showed that,the group which was treated by platelet increasing capsule combined with hormone was superior to the hormone alone group in the aspects of overall effectiveness rate (RR=1.18,95%CI 1.06 to 1.32,P=0.003),the significant effectiveness rate (RR=1.57,95%CI 1.29 to 1.91,P<0.000 01),blood platelet count (MD=21.54,95%CI 13.85 to 29.23,P<0.000 01),and the recurrence rate (RR=0.49,95%CI 0.34 to 0.69,P<0.000 01) which was lower.Conclusion Current evidence has showed that platelet increasing capsule combined with hormone in treating ITP is better than hormone alone.However,more high quality RCTs are needed to verify the above conclusion in future.%目的 系统评价升血小板胶囊联合激素治疗特发性血小板减少性紫癜(ITP)的有效性.方法 计算机检索PubMed (1966~2012)、CENTRAL(2012年第3期)、CBM (1978~2012)、CNKI (1979~2012)、Wanfang Data (1998~2012)、VIP (1991~2012)等数据库,收集升血小板胶囊联合激素治疗特发性血小板减少性紫癜的随机对照试验(RCT),并追溯纳入研究的参考文献.由两位研究者按照纳入与排除标准独立筛选文献、提取资料和评价质

  4. Atypical course of Henoch-Schonlein purpura in an adult patient.

    Science.gov (United States)

    Bielewicz-Zielińska, Agnieszka; Górnikiewicz-Brzezicka, Bożena; Brzezicki, Jan; Rymko, Marcin; Jeka, Sławomir

    2015-01-01

    Henoch-Schonlein purpura is vasculitis of small blood vessels characterized by deposits of IgA immune complexes and also non-thrombocytopenic purpura, abdominal pain, arthritis and renal involvement. It affects people of all ages, but most cases occur in children between 2 and 11 years old, more frequently in boys. The disease is much less common in adults, who often have a more severe course of the disease. The paper presents the case of a 38-year-old female patient with insidious course of the disease, initially dominant skin symptoms and joint pain without signs of inflammation. Symptoms of the disease were initially uncharacteristic and mild, and did not suggest severe and rapid course of the disease. The patient required hospitalization in several departments before final diagnosis of Henoch-Schonlein purpura. The patient was treated with corticosteroids and immunosuppression. Despite the rapid course of the disease, treatment was effective. The patient was discharged in good condition.

  5. 婴幼儿与3岁以上儿童特发性血小板减少性紫癜的临床对比%Comparison study of idiopathic thrombocytopenic purpura in the children below 3 years old with above 3 years old

    Institute of Scientific and Technical Information of China (English)

    刘巧; 宪莹; 戴碧涛; 徐酉华; 肖剑文; 王世一; 陆玲玲; 于洁

    2009-01-01

    小板上升速度快于单用激素治疗.%Objective To study the clinical features of pediatric patients with idiopathic thrombocytopenic purpura (1TP) from infancy to adolescence, and compare their treatment response and the effects. Methods The data of the ITP children diagnosed and administrated between 2006~2008 were collected and analyzed by soft ware SPSS 12.0. The patients were divided into infant group ( 3 years old). The gender, demographic information, platelet count at the diagnosis, bleeding and anemia, virus infection and megakaryocytes, and most importantly, the treatment response were evaluated. Results (1 )The percentage of the male in infant group (66%) was statistically higher than that (50.6% ) in the elder group ( P = 0.031). (2) The incidence of epistaxis in the infant group (9.7% , n - 10) was statistically lower than that (43.7%) in the elder group (P < 0.001). The incidence of bleeding-caused anemia in the infant group (11.7%) was statistically lower than that (43.7%) in the elder children (P = 0.001). (3) The incidence of viral enteritis as a motivation in the infant group (14.56% ) was statistically higher than that (1.15%) in the elder group (P = 0.001); the incidence of vaccine inoculation as a motivation in the infant group (8.74%) was statistically higher than that (1.15%) in the elder group (P = 0.045). About 32.32% was cytomegalovi-rus-positive in the infant group while 2.35% in the elder group (P < 0.001). (4)The percentage of EB virus positive in infant group (4.04%) was statistically lower than that (14.12% ) in the elder patients(P - 0.016). (5) Both the median and the increase ratio of megakrocytes in infant group ( 144,81.7%) were statistically lower than that (280,96.6%) in the elder group (P = 0.005 ; P = 0.002). (6) Un-der the treatment of steroids only, the time for the platelet to become normal in infant group(7.1 ±2.6) was statistically shorter than that( 10.3±3.2) in the elder children(P< 0.001). Under the treatment

  6. 大剂量地塞米松对免疫性血小板减少性紫癜患者浆样树突状细胞功能及Toll样受体9表达的影响%Effect of high-dose dexamethasone on the function and TLR-9 expression of plasmacytoid dendritic cells in patients with immune thrombocytopenic purpura

    Institute of Scientific and Technical Information of China (English)

    王莉; 张连生; 柴晔; 曾鹏云; 吴重阳

    2012-01-01

    To investigate the effect of high-dose dexamethasone on the function and TLR-9 expression of plasmacytoid dendritic cells in the patients with immune thrombocytopenic purpura. Method: Fifteen newly diagnosed patients with immune thrombocytopenic purpura received high dose(HD)DXM at single daily doses of 40 mg for 4 consecutive days. The peripheral blood plasmacytoid dendritic cells isolated from 13 remission patients and 15 normal controls were separated by immunomagnetie beads and then induced by CpG-OND2216 for 24 hours. The levels of IFN-α,IL-6 and TNF-αin the supernatant were detected by enzyme linked immunosorbent assay. The expression of TLR9 mRNA of pDCs was detected by Real-time quantitative PCR. Result: In ITP patients, the levels of IFN-α.IL-6 and TNF-αproduced by pDCs were significantly higher compared with those in normal controls and in treated group(P0. 05). The expression of TLR9 mRNA of pDCs in untreated group were significantly higher than control group(P0. 05). Conclusion: pDCs may play important role in ITP by their Toll-like receptor 9 and cytokines secretion (Dexamethasone may reduce the expression of TLR9, inhibit pDC function,and thus play a therapeutic role.%目的:研究大剂量地塞米松对免疫性血小板减少性紫癜(ITP)患者浆样树突状细胞(pDCs)功能及Toll样受体9表达的影响.方法:15例初诊的ITP患者给予地塞米松40 mg/d,连用4d.采用免疫磁珠分离法体外分离15例正常对照及13例治疗有效患者治疗前后外周血中浆细胞样树突状细胞(pDCs),用CPG-ODN 2216刺激外周血pDCs并与之共培养24 h,采用酶标记免疫吸附(ELISA)方法检测上清中IFN-α、IL-6、TNF-α的含量;实时定量聚合酶链反应(RT-PCR)检测pDCs的TLR9 mRNA表达量.结果:①治疗前pDCs产生IFN-α、IL-6、TNF-α细胞因子的水平[(960.83±164.65)pg/ml,(156.15土39.89) pg/ml,(137.31土35.44)pg/ml]明显高于正常对照组[(616.67±105.98) pg/ml,(89.13±21.48) pg/ml,(88.53±25

  7. Purpura Fulminans following Thermal Injury

    Directory of Open Access Journals (Sweden)

    Jiongyu Hu

    2013-01-01

    Full Text Available Purpura fulminans is a rare syndrome of intravascular thrombosis and hemorrhagic infarction of the skin, which is an unusual cutaneous manifestation of disseminated intravascular coagulation. It often occurs in small children and babies due to infection and/or sepsis, rarely in adults in clinic. We report the first case of deadly purpura fulminans following thermal injury in a 64-year-old Chinese woman. The purpura developed sharply and aggravated multiple organ dysfunction. The patient died of purpura fulminans, disseminated intravascular coagulation, and multiple organ dysfunction syndrome.

  8. Fatal purpura fulminans and Waterhouse-Friderichsen syndrome from fulminant Streptococcus pneumoniae sepsis in an asplenic young adult.

    Science.gov (United States)

    Hale, Andrew J; LaSalvia, Mary; Kirby, James E; Kimball, Allison; Baden, Rachel

    2016-01-01

    Asplenic patients are at increased risk for sepsis and fulminant infection. Sepsis in these patients is typically secondary to encapsulated bacteria, with Streptococcus pneumoniae being the most frequent pathogen. Rare complications of severe sepsis include purpura fulminans and bilateral adrenal hemorrhage (Waterhouse-Friderichsen syndrome). We present the case of a 36-year-old woman, healthy except for splenectomy years prior for idiopathic thrombocytopenic purpura treatment, who presented with fever. Upon presentation to our hospital, three hours after symptoms onset, she had purpura fulminans and shock. Despite timely antimicrobials and maximal resuscitative efforts, her disease progressed and she expired 12 hours after symptoms onset. Autopsy revealed bilateral adrenal hemorrhage; acute adrenal crisis likely contributed to her refractory shock. Prior to her presentation, she had not received guideline-based post-splenectomy care. Sepsis in asplenic patients can be fulminant and rapidly fatal. Streptococcus pneumoniae remains the most frequent cause, despite decreasing rates in recent years related to widespread pneumococcal vaccination. Guideline-based vaccinations and "pill-in-pocket" therapy can be life-saving for asplenic patients. Purpura fulminans represents an extreme manifestation of disseminated intravascular coagulation, is more common in asplenic patients, and portends a poor prognosis. Waterhouse-Friderichsen syndrome can be seen concurrently with purpura fulminans and further portends a poor prognosis; pre-mortem diagnosis requires a high index of suspicion.

  9. Fatal purpura fulminans and Waterhouse-Friderichsen syndrome from fulminant Streptococcus pneumoniae sepsis in an asplenic young adult.

    Science.gov (United States)

    Hale, Andrew J; LaSalvia, Mary; Kirby, James E; Kimball, Allison; Baden, Rachel

    2016-01-01

    Asplenic patients are at increased risk for sepsis and fulminant infection. Sepsis in these patients is typically secondary to encapsulated bacteria, with Streptococcus pneumoniae being the most frequent pathogen. Rare complications of severe sepsis include purpura fulminans and bilateral adrenal hemorrhage (Waterhouse-Friderichsen syndrome). We present the case of a 36-year-old woman, healthy except for splenectomy years prior for idiopathic thrombocytopenic purpura treatment, who presented with fever. Upon presentation to our hospital, three hours after symptoms onset, she had purpura fulminans and shock. Despite timely antimicrobials and maximal resuscitative efforts, her disease progressed and she expired 12 hours after symptoms onset. Autopsy revealed bilateral adrenal hemorrhage; acute adrenal crisis likely contributed to her refractory shock. Prior to her presentation, she had not received guideline-based post-splenectomy care. Sepsis in asplenic patients can be fulminant and rapidly fatal. Streptococcus pneumoniae remains the most frequent cause, despite decreasing rates in recent years related to widespread pneumococcal vaccination. Guideline-based vaccinations and "pill-in-pocket" therapy can be life-saving for asplenic patients. Purpura fulminans represents an extreme manifestation of disseminated intravascular coagulation, is more common in asplenic patients, and portends a poor prognosis. Waterhouse-Friderichsen syndrome can be seen concurrently with purpura fulminans and further portends a poor prognosis; pre-mortem diagnosis requires a high index of suspicion. PMID:27583208

  10. Alloimmunization among transfusion-dependent thalassemia patients

    OpenAIRE

    Sadeghian Mohammad; Keramati Mohammad; Badiei Zahra; Ravarian Mehrangiz; Ayatollahi Hossein; Rafatpanah Houshang; Daluei Mohammad

    2009-01-01

    Background: Thalassemia is a common hemoglobin disorder in Iran and one of the major public health problems. Although blood transfusions are lifesavers for thalassemia patients, they may be associated with some complications especially erythrocyte alloimmunization. The purpose of this study was to investigate the prevalence of red blood cell alloantibodies and to determine types of these antibodies among multiple-transfused thalassemic patients. Materials and Methods: A total of 313 thalas...

  11. Systemic Lupus Erythematosus Presenting as Thrombotic Thrombocytopenia Purpura: How Close Is Close Enough?

    Directory of Open Access Journals (Sweden)

    Cesar A. Perez

    2011-01-01

    Full Text Available Thrombotic thrombocytopenic purpura (TTP is an uncommon life-threatening disease characterized by microangiopathic hemolytic anemia and thrombocytopenia, commonly associated with infections, malignancy, drugs, and autoimmune diseases. We report a case of 19-year-old previously healthy female that presents with anemia and thrombocytopenia diagnosed with thrombotic thrombocytopenic purpura that was treated successfully with plasmapheresis and corticosteroids. Laboratory findings also revealed antinuclear antibodies and antibodies to double-stranded DNA. Two weeks after presentation developed inflammatory arthritis, fulfilling diagnostic criteria for systemic lupus erythematosus (SLE. Prompt diagnosis and treatment with plasma exchange and corticosteroids should be instituted as soon as the diagnosis of TTP is suspected, even if other diagnoses, including lupus, are possible. When present, the coexistence of these two etiologies can have a higher mortality than either disease alone. An underlying diagnosis of SLE should be considered in all patients presenting TTP and the study of this association may provide a better understanding of their immune-mediated pathophysiology.

  12. Effect of High Dose Dexamethasone on Function and TLR-9 mRNA Expression of Plasmacytoid Dendritic Cells in Patients with Immune Thrombocytopenic Purpura%大剂量地塞米松对免疫性血小板减少性紫癜患者浆细胞样树突状细胞功能及Toll样受体9mRNA表达的影响

    Institute of Scientific and Technical Information of China (English)

    王莉; 张连生; 柴晔; 曾鹏云; 吴重阳

    2012-01-01

    本研究探讨大剂量地塞米松对免疫性血小板减少性紫癜(ITP)患者外周血浆细胞样树突状细胞(pDC)功能及Toll样受体9(TLR9)表达的影响.15例初诊的ITP患者给予地塞米松40 mg/d,连用4d,采用免疫磁珠分离法体外分离15例正常对照及13例治疗有效患者治疗前后外周血中pDC;用CpG-ODN 2216刺激外周血pDC并与之共培养24h,采用ELISA方法检测上清中IFN-α、IL-6、TNF-α的含量;用实时定量聚合酶链反应(RT-PCR)检测pDC的TLR9 mRNA表达量.结果表明,①治疗前pDC产生IFN-α、IL-6、TNF-α水平[(960.83±164.65)pg/ml,(156.15±39.89)pg/ml,(137.31±35.44) pg/ml)]明显高于正常对照组[(616.67±105.98) pg/ml,(89.13±21.48) pg/ml,(88.53±25.81) pg/ml,P<0.05];治疗后pDC产生IFN-α、IL-6、TNF-α水平分别降至(678.46±128.88) pg/ml,(97.77±26.31) pg/ml,(103.08±26.42) pg/ml,与治疗前比较差异有统计学意(P<0.05),与正常对照组相比差异无统计学意义(P>0.05);②治疗前pDC的TLR9 mRNA的表达水平高于正常对照组(P<0.05);治疗后pDC的TLR9 mRNA的表达水平低于治疗前(P<0.05),与正常对照组比较差异无统计学显著性(P>0.05).结论:pDC分泌的细胞因子及其表达的TLR9在ITP发病中起重要作用;地塞米松可能通过下调TLR9的表达,抑制pDC分泌细胞因子的功能,而对ITP起到治疗作用.%This study was purposed to investigate the effect of high-dose dexamethasone ( DXM) on function and Toll like receptor 9 (TLR-9) expression of plasmacytoid dendritic cells ( pDC) in peripheral blood of patients with immune thrombocytopenic purpura( ITP). 15 newly diagnosed patients with ITP received high dose DXM at single daily doses of 40 mg for 4 consecutive days. The peripheral blood plasmacytoid dendritic cells from 13 remission patients and 15 nor-mal controls were separated by immunomagnetie beads and then induced by CpG-OND2216. 24 h later, the levels of IFN-α,IL-6 and TNF-α in the

  13. 自身免疫性血小板减少性紫癜患者外周血中CD4+CD25+调节性T细胞、sFas和sFasL的表达及临床意义%Expression and Clinical Significance of CD4 + CD25 + Treg Cells, sFas and sFasL in Peripheral Blood of Patients with Autoimmune Thrombocytopenic Purpura

    Institute of Scientific and Technical Information of China (English)

    贾瑞萍; 赵雪芸

    2011-01-01

    本研究通过检测成人自身免疫性血小板减少性紫癜(AITP)外周血中CI4+CD25+调节性T细胞(Treg)、sFas和sFasL的表达,探讨它们在AITP发病机制中的作用及临床意义,为寻求AITP治疗的有效方法提供理论依据.采用流式细胞术分别检测30例AITP患者和18例正常对照者外周血CD4+T、Treg、CD4+ CD25-T细胞表达率及Treg/CD4+T比值;用酶联免疫吸附法(ELISA)检测AITP患者治疗前后及对照组外周血sFas、sFasL表达水平.结果表明,AITP组外周血中CD4+T细胞表达率低于对照组(p<0.05),Treg细胞表达率及Treg/CD4+T比值明显低于正常对照组(p<0.0l).AITP组患者治疗前外周血中sFas和sFasL水平明显高于治疗后和正常对照组(p<0.01),AITP组治疗后与正常对照组外周血sFas、sFasL水平差异无统计学意义(p>0.05).AITP患者治疗前Treg细胞表达率、Treg/CD4+T细胞比值与血小板计数呈正相关;AITP患者外周血中sFas和sFasL水平呈正相关;CD4+T细胞、CD4+CD25 -T细胞表达率,sFas、sFasL浓度与血小板计数无明显相关;Treg细胞表达率和sFas、sFasL浓度间没有明显相关性.结论:Treg在AITP的发病机制中发挥一定作用;Treg细胞水平与AITP病情的严重程度有关;sFas和sFasL水平异常参与了AITP的免疫病理过程.%This study was aimed to detect the expression of CD4 * CD25 * regulatory T cells (Treg) , sFas and sFasL in patients with autoimmune thrombocytopenic purpura ( AITP) , and to explore their roles in the pathogenesis of AITP and clinical significance, so as to provie a theoretical basis for effective treatment for AITP. The expressions of CD4 * T, Treg, CD4 * CD25" T, Treg /CD4 * T in peripheral blood of 30 the patients with AITP and 18 controls were detected by flow cytometry, and enzyme-linked immunosorbent assay (ELISA) was used to detect the levels of sFas and sFasL in peripheral blood of controls and the patients with AITP before and after treatment. The

  14. Prevention and management of transfusion-induced alloimmunization: current perspectives

    Directory of Open Access Journals (Sweden)

    Hauck-Dlimi B

    2014-08-01

    Full Text Available Barbara Hauck-Dlimi, Susanne Achenbach, Julian Strobel, Reinhold Eckstein, Robert Zimmermann Department of Transfusion Medicine and Haemostaseology, University Hospital Erlangen, Erlangen, Germany Abstract: Transfusion of blood components, transplantations, and exchange of blood between mother and child during pregnancy or at birth can lead to alloimmunization. Because of its clinical relevance, this review brings into focus alloimmunization against red blood cells, human platelet antigens, human leukocyte antigens, and human neutrophil antigens. In principle, an individual is able to develop antibodies after exposure to a nonautogenous antigen, but these cells actually induce alloimmunization only for a minority of patients. An individual producing alloantibodies after having contact with foreign antigens depends on various factors, such as genetic predisposition, underlying diseases, the patient's immune status, and clinical immune modulation. When alloimmunization has occurred, it could lead to problems for future transfusions or transplantations. Keywords: transfusion, alloimmunization, prevention

  15. Maternal anti-HLA class I antibodies are associated with reduced birth weight in thrombocytopenic neonates.

    Science.gov (United States)

    Dahl, J; Husebekk, A; Acharya, G; Flo, K; Stuge, T B; Skogen, B; Straume, B; Tiller, H

    2016-02-01

    In this comparative cross-sectional study, possible associations between maternal anti-HLA class I antibodies and birth weight in neonatal thrombocytopenia are explored. Although commonly detected in pregnancies and generally regarded as harmless, it has been suggested that such antibodies might be associated with fetal and neonatal alloimmune thrombocytopenia (FNAIT). As a link between FNAIT due to human platelet antigen 1a-specific antibodies and reduced birth weight in boys has previously been demonstrated, we wanted to explore whether maternal anti-HLA class I antibodies might also affect birth weight. To examine this, suspected cases of FNAIT referred to the Norwegian National Unit for Platelet Immunology during the period 1998-2009 were identified. Pregnancies where the only finding was maternal anti-HLA class I antibodies were included. An unselected group of pregnant women participating in a prospective study investigating maternal-fetal hemodynamics at the University Hospital North Norway during the years 2006-2010 served as controls. Twenty-nine percent of controls had anti-HLA class I antibodies. The thrombocytopenic neonates had a significantly lower adjusted birth weight (linear regression, P=0.036) and significantly higher odds of being small for gestational age (OR=6.72, P<0.001) compared with controls. Increasing anti-HLA class I antibody levels in the mother were significantly associated with lower birth weight and placental weight among thrombocytopenic neonates, but not among controls. These results indicate that maternal anti-HLA class I antibodies in thrombocytopenic neonates are associated with reduced fetal growth. Further studies are needed to test if placental function is affected.

  16. Case report: solid-phase platelet crossmatching to support the alloimmunized patient.

    Science.gov (United States)

    O'Connell, B A

    1995-01-01

    Platelet crossmatching by a solid-phase red cell adherence assay was used to provide compatible platelets for two alloimmunized patients with leukemia. In this study, a successful platelet transfusion was defined as giving a corrected count increment (CCI) of >7,500 in a posttransfusion sample. For patient A, a total of 205 random platelet concentrates (PCs) were crossmatched. Eleven were considered compatible. These 11 PCs were transfused during five transfusion episodes. Four of the five transfusions produced CCIs of >7,500 and were considered successful. Individually, eight of the eleven units were considered in vivo compatible, and five of the eight donors of these units agreed to become apheresis donors. Platelets from three of these five apheresis donors gave CCIs of >7,500. For patient B, 1,074 random PCs were crossmatched, and 332 were considered compatible. These units were administered during 78 different transfusions. Seventy-one of these transfusion episodes resulted in CCIs of >7,500. In addition, 19 apheresis donors were identified by platelet crossmatching, and they provided platelets for 38 of 39 successful transfusions for Patient B. Platelet crossmatching should therefore be considered when a blood bank is called upon to support a refractory thrombocytopenic patient.

  17. A Non-Invasive Strategy for Neonatal Alloimmune Thrombocytopenia Diagnosis: Newborn Platelet Genotyping with Buccal Swabs

    Directory of Open Access Journals (Sweden)

    Gérald Bertrand

    2016-07-01

    Full Text Available Neonatal alloimmune thrombocytopenia results from the maternal immune response against fetal-specific antigens inherited from the father. The diagnosis is ascertained only when the maternal alloantibody and the offending antigen present in the newborn are identified. Up until now most laboratories perform DNA extraction for neonatal genotyping from newborn blood samplings. In order to avoid such an invasive procedure, two protocols of DNA extraction from buccal swabs were developed: a manual protocol using the QIAamp mini blood kit (Qiagen, and an automated procedure with the MagNA Pure Compact instrument (Roche. Both EDTA-blood and buccal swabs from thrombocytopenic newborns were genotyped manually (14 samples, automatically (15 samples or both manually and automatically (two samples. Human Platelet Antigen (HPA genotyping was performed using the BeadChip assay (BioArray, Immucor. Concordant genotypings were obtained for all samples except for one swab with the manual method. The automated DNA extraction from newborn buccal swabs with the MagNA Pure Compact instrument was chosen as the first-line strategy, with a significant gain of time in processing buccal swabs.

  18. Neonatal management and outcome in red cell alloimmunization

    NARCIS (Netherlands)

    Smits-Wintjens, Vivianne Elise Huberta Johanna

    2012-01-01

    In this thesis, several studies on neonatal red cell alloimmune hemolytic disease are presented, including various management options, associated complications and co-morbidities and the short-term and long-term outcome of children with Rhesus hemolytic disease.

  19. ACOG Practice Bulletin No. 75: Management of alloimmunization during pregnancy.

    Science.gov (United States)

    2006-08-01

    When any fetal blood group factor inherited from the father is not possessed by the mother, antepartum or intrapartum fetal-maternal bleeding may stimulate an immune reaction in the mother Maternal immune reactions also can occur from blood product transfusion. The formation of maternal antibodies, or"alloimmunization,' may lead to various degrees of transplacental passage of these antibodies into the fetal circulation. Depending on the degree of antigenicity and the amount and type of antibodies involved, this transplacental passage may lead to hemolytic disease in the fetus and neonate. Undiagnosed and untreated, alloimmunization can lead to significant perinatal morbidity and mortality. Advances in Doppler ultrasonography have led to the development of noninvasive methods of management of alloimmunization in pregnant women. Together with more established protocols, Doppler ultrasound evaluation may allow for a more thorough and less invasive workup with fewer risks to the mother and fetus. Prevention of alloimmunization is addressed in another Practice Bulletin (1). PMID:16880320

  20. Responder individuality in red blood cell alloimmunization.

    Science.gov (United States)

    Körmöczi, Günther F; Mayr, Wolfgang R

    2014-11-01

    Many different factors influence the propensity of transfusion recipients and pregnant women to form red blood cell alloantibodies (RBCA). RBCA may cause hemolytic transfusion reactions, hemolytic disease of the fetus and newborn and may be a complication in transplantation medicine. Antigenic differences between responder and foreign erythrocytes may lead to such an immune answer, in part with suspected specific HLA class II associations. Biochemical and conformational characteristics of red blood cell (RBC) antigens, their dose (number of transfusions and pregnancies, absolute number of antigens per RBC) and the mode of exposure impact on RBCA rates. In addition, individual circumstances determine the risk to form RBCA. Responder individuality in terms of age, sex, severity of underlying disease, disease- or therapy-induced immunosuppression and inflammation are discussed with respect to influencing RBC alloimmunization. For particular high-risk patients, extended phenotype matching of transfusion and recipient efficiently decreases RBCA induction and associated clinical risks. PMID:25670932

  1. Factors Influencing RBC Alloimmunization: Lessons Learned from Murine Models

    OpenAIRE

    Ryder, Alex B.; Zimring, James C.; Hendrickson, Jeanne E

    2014-01-01

    Red blood cell (RBC) alloimmunization may occur following transfusion or pregnancy/delivery. Although observational human studies have described the immunogenicity of RBC antigens and the clinical significance of RBC alloantibodies, studies of factors influencing RBC alloimmunization in humans are inherently limited by the large number of independent variables involved. This manuscript reviews data generated in murine models that utilize transgenic donor mice, which express RBC-specific model...

  2. An insidious presentation of thrombotic thrombocytopenic purpura:A case report and brief literature review

    Institute of Scientific and Technical Information of China (English)

    Shafeek Kiblawi; Elie Harmouche; Ralph Bou Chebl; Gilbert Abou Dagher

    2014-01-01

    Thrombotic thrombocytopenic purpura(TTP) is a rare thrombotic microangiopathy with an estimated incidence of11 cases/million population per year.Early treatment is essential and is curative in this disease where lack of treatment results in90% mortality.We describe an atypical case of a patient withTTP who presented to theEmergencyDepartment for headache, and was found to have thrombocytopenia but only mild anemia that was explained by another disease process.Case:A44-year-old female presented to theEmergencyDepartment for worsening headache and weakness over the last week.She had no fever and no focal neurological deficits but was pale and complained of severe headache.A blood test showed her to be anemic and thrombocytopenic.She explained that she had been having prolonged heavy menses over the last year.She was treated with blood and platelet transfusions, and seen by theGynecology service who treated her for uterine fibroids after which she was discharged.She returned1 week later with the same complaint, and was found to have a stable hemoglobin level but recurrence of thrombocytopenia.ATTP diagnosis was entertained and the workup confirmed it.The patient was treated with plasmapheresis and discharged home with no sequalae.Conclusion:Emergency physicians should keepTTP in mind when approaching cases of thrombocytopenia with mild anemia, even if an alternative diagnosis exists.

  3. Forskelle i behandling af børn med idiopatisk trombocytopenisk purpura

    DEFF Research Database (Denmark)

    Edslev, Pernille Wendtland; Kjærsgaard, Mimi; Rosthøj, Steen

    2010-01-01

    INTRODUCTION: Idiopathic thrombocytopenic purpura (ITP) is a rare immune-mediated bleeding disorder that usually takes a self-limiting and benign course. Due to the risk of intracranial haemorrhage, treatment regimens tend to be active. We present treatment data from 17 paediatric departments...... in Denmark (1998-2000), focusing on regional differences in treatment strategy. MATERIAL AND METHODS: As part of a prospective Nordic study, clinical findings and treatment were recorded for 109 children with newly diagnosed ITP. The course in the following six months was reported for 91 children. Results...... transfusion was given to 24%, 0% and 4%, respectively. There were no differences in remission rates or frequency of mucosal bleeding during follow-up, but treatment rates were 6.3, 4.7, and 3.0 per patient-year with severe thrombocytopenia. Chronic ITP lasting more than six months developed in 26%, 33...

  4. Idiopathic thromobocytopenic purpura in two mothers of children with DiGeorge sequence: A new component manifestation of deletion 22q11?

    Energy Technology Data Exchange (ETDEWEB)

    Levy, A.; Philip, N. [Hopital d`Enfants de la Timone, Marseilles (France); Michel, G. [Hopital d`Enfants de la Timone, Marseilles (France)] [and others

    1997-04-14

    The phenotypic spectrum caused by the microdeletion of chromosome 22q11 region is known to be variable. Nearly all patients with DiGeorge sequence (DGS) and approximately 60% of patients with velocardiofacial syndrome exhibit the deletion. Recent papers have reported various congenital defects in patients with 22q11 deletions. Conversely, some patients have minimal clinical expression. Ten to 25% of parents of patients with DGS exhibit the deletion and are nearly asymptomatic. Two female patients carrying a 22q11 microdeletion and presenting with idiopathic thrombocytopenic purpura are reported. Both had children with typical manifestations of DGS. 12 refs., 4 figs., 1 tab.

  5. Role of RBCs alloimmunization in multiple transfused thalassaemia patients

    Directory of Open Access Journals (Sweden)

    Amita Sagar Patel

    2016-03-01

    Results: Out 50 patients of thalassemia major, 4 patients (8% developed red cell alloantibodies respectively. The red cell alloantibodies were against Rh, Kidd, Kell, Duffy, Lewis, MNS and P system. Results of this study (P value >0.05 indicate low frequency of RBCs alloimmunization. Conclusions: Low alloimmunization rate implies that there is homogeneity of red cell antigens in blood donors and recipients. RBCs alloantibody formation was not influenced by gender, age at start of transfusions and number of packed cells received. Already alloimmunized patients get benefits from leucodepleted packed cells. Specific recommendation given on routine pre-transfusion antibody screening to ensure safer transfusion. [Int J Res Med Sci 2016; 4(3.000: 822-828

  6. Prostanoids modulate inflammation and alloimmune responses during graft rejection

    Directory of Open Access Journals (Sweden)

    P.N. Rocha

    2005-12-01

    Full Text Available Acute rejection of a transplanted organ is characterized by intense inflammation within the graft. Yet, for many years transplant researchers have overlooked the role of classic mediators of inflammation such as prostaglandins and thromboxane (prostanoids in alloimmune responses. It has been demonstrated that local production of prostanoids within the allograft is increased during an episode of acute rejection and that these molecules are able to interfere with graft function by modulating vascular tone, capillary permeability, and platelet aggregation. Experimental data also suggest that prostanoids may participate in alloimmune responses by directly modulating T lymphocyte and antigen-presenting cell function. In the present paper, we provide a brief overview of the alloimmune response, of prostanoid biology, and discuss the available evidence for the role of prostaglandin E2 and thromboxane A2 in graft rejection.

  7. Crystallographic Structure of the Human Leukocyte Antigen DRA, DRB3*0101: Models of a Directional Alloimmune Respone and Autoimmunity

    Energy Technology Data Exchange (ETDEWEB)

    Parry,C.; Gorski, J.; Stern, L.

    2007-01-01

    We describe structural studies of the human leukocyte antigen DR52a, HLA-DRA/DRB3*0101, in complex with an N-terminal human platelet integrin {alpha}II{sub B}{beta}III glycoprotein peptide which contains a Leu/Pro dimorphism. The 33:Leu dimorphism is the epitope for the T cell directed response in neonatal alloimmune thrombocytopenia and post-transfusion purpura in individuals with the {alpha}II{sub B}{beta}III 33:Pro allele, and defines the unidirectional alloimmune response. This condition is always associated with DR52a. The crystallographic structure has been refined to 2.25 {angstrom}. There are two {alpha}{beta} heterodimers to the asymmetric unit in space group P4{sub 1}2{sub 1}2. The molecule is characterized by two prominent hydrophobic pockets at either end of the peptide binding cleft and a deep, narrower and highly charged P4 opening underneath the beta 1 chain. Further, the peptide in the second molecule displays a sharp upward turn after pocket P9. The structure reveals the role of pockets and the distinctive basic P4 pocket, shared by DR52a and DR3, in selecting their respective binding peptide repertoire. We observe an interesting switch in a residue from the canonically assigned pocket 6 seen in prior class II structures to pocket 4. This occludes the P6 pocket helping to explain the distinctive '1-4-9' peptide binding motif. A {beta}57 Asp {yields} Val substitution abrogates the salt-bridge to {alpha}76 Arg and along with a hydrophobic {beta}37 is important in shaping the P9 pocket. DRB3*0101 and DRB1*0301 belong to an ancestral haplotype and are associated with many autoimmune diseases linked to antigen presentation, but whereas DR3 is susceptible to type 1 diabetes DR52a is not. This dichotomy is explored for clues to the disease.

  8. Evaluation of a prototype electronic personal health record for patients with idiopathic thrombocytopenic purpura

    OpenAIRE

    Chiche, Laurent

    2012-01-01

    Laurent Chiche,1 Alessandra Brescianini,1 Julien Mancini,2 Hervé Servy,3 Jean-Marc Durand11Service de Médecine Interne, Centre de Compétence pour la prise en charge des Cytopénies Auto-immunes, Hôpital de la Conception, Marseille, 2Service de Santé Publique, Hôpital de la Timone, Marseille, 3Association AIMSU, Maison des Associations, La Ciotat, FranceBackground: Patients with rare diseases often lack information about...

  9. Management of chronic immune thrombocytopenic purpura: targeting insufficient megakaryopoiesis as a novel therapeutic principle

    Directory of Open Access Journals (Sweden)

    Andreas Rank

    2010-05-01

    Full Text Available Andreas Rank, Oliver Weigert, Helmut OstermannMedizinische Klinik III – Grosshadern, Klinikum der Ludwig Maximilians-Universitaet Munich, Munich, GermanyAbstract: Traditionally, anti-platelet autoantibodies accelerating platelet clearance from the peripheral circulation have been recognized as the primary pathopysiological mechanism in chronic immune thrombocytopenia (ITP. Recently, increasing evidence supports the co-existence of insufficient megakaryopoiesis. Inadequate low thrombopoietin (TPO levels are associated with insufficient proliferation and differentiation of megakaryocytes, decreased proplatelet formation, and subsequent platelet release. Recently two novel activators of TPO receptors have been made available: romiplostim and eltrombopag. In several phase III studies, both agents demonstrated increase of platelet counts in about 80% of chronic ITP patients within 2 to 3 weeks. These agents substantially broaden the therapeutic options for patients with chronic ITP although long-term results are still pending. This review will provide an update on the current conception of underlying mechanisms in ITP and novel, pathophysiologically based treatment options.Keywords: immune thrombocytopenia, romiplostim, eltrombopag, megakaryopoiesis

  10. Thrombotic thrombocytopenic purpura and its look-alikes: a single institution experience.

    Science.gov (United States)

    Bellone, Michael; Chiang, Jason; Ahmed, Tahmeena; Galanakis, Dennis; Senzel, Lisa

    2012-02-01

    At presentation, variant or "look-alike" conditions can resemble TTP. We reviewed charts of 26 consecutive patients treated for presumed TTP. Of 15 classic TTP patients, 11 were tested for ADAMTS13; all showed severe deficiency, and inhibitor levels correlated with probability of relapse. The variant TMA group consisted of 8 patients who had active clinical disorders which overlapped with TTP. Variant TMA patients had higher creatinine and worse prognosis than classic TTP patients. "Look-alike" disorders included ITP with intravascular hemolysis following administration of WinRho™, and human granulocytic anaplasmosis. These conditions had not been previously described as TTP look-alikes. PMID:22154043

  11. How we use WinRho in patients with idiopathic thrombocytopenic purpura.

    Science.gov (United States)

    Stotler, Brie A; Schwartz, Joseph

    2015-11-01

    Primary immune thrombocytopenia (ITP) is an autoimmune disease that affects children and adults. WinRho SDF is a D immune globulin product that is Food and Drug Administration approved for the treatment of ITP in D+ pediatric and adult patients. WinRho is a plasma-derived biologic product dispensed from blood banks. Transfusion medicine physicians serve as a resource to health care providers regarding blood component and derivative usage and, as such, should be familiar with the use of WinRho for ITP, including the dosage, administration, and contraindications. This report details the transfusion medicine consultation practice and guidelines at a tertiary care academic medical center for the usage of WinRho SDF in patients with ITP. PMID:26094894

  12. Pathogenicity of Anti-ADAMTS13 Autoantibodies in Acquired Thrombotic Thrombocytopenic Purpura

    Directory of Open Access Journals (Sweden)

    Mari R. Thomas

    2015-08-01

    Conclusions: Anti-spacer domain autoantibodies are the major inhibitory antibodies in acquired TTP. However, depletion of ADAMTS13 antigen (rather than enzyme inhibition is a dominant pathogenic mechanism. ADAMTS13 antigen levels at presentation have prognostic significance. Taken together, our results provide new insights into the pathophysiology of acquired TTP.

  13. Management of chronic immune thrombocytopenic purpura: targeting insufficient megakaryopoiesis as a novel therapeutic principle

    OpenAIRE

    Rank, Andreas

    2010-01-01

    Andreas Rank, Oliver Weigert, Helmut OstermannMedizinische Klinik III – Grosshadern, Klinikum der Ludwig Maximilians-Universitaet Munich, Munich, GermanyAbstract: Traditionally, anti-platelet autoantibodies accelerating platelet clearance from the peripheral circulation have been recognized as the primary pathopysiological mechanism in chronic immune thrombocytopenia (ITP). Recently, increasing evidence supports the co-existence of insufficient megakaryopoiesis. Inadequate low throm...

  14. Thrombocytopenic thrombotic purpura: severe clinic with no CT, minor MRI, but a SPECT correlate

    Energy Technology Data Exchange (ETDEWEB)

    Baron, Y. [Radiologische Abteilung, Staedtisches Krankenhaus Kiel, Chemnitzstr. 33, Kiel 24116 (Germany); Bargemann, T. [Abteilung Internistische Intensivmedizin und Dialyse am Staedtischen Krankenhaus Kiel, Chemnitzstr. 33, Kiel 24116 (Germany); Harten, P. [Zweite Medizinische Universitaetsklinik und Poliklinik der Christian-Albrechts-Universitaet zu Kiel, Chemnitzstr. 33, Kiel 24116 (Germany); Gutschmidt, H.-J. [Abteilung Internistische Intensivmedizin und Dialyse am Staedtischen Krankenhaus Kiel, Chemnitzstr. 33, Kiel 24116 (Germany)

    1999-07-01

    A 28-year-old woman with primarily therapy refractory TTP was followed neuroradiologically over 6 months. Despite pronounced neurological and neuropsychiatric symptoms including hemiparesis and aphasia she had unremarkable CT scans on two occasions. Three MRI exams showed no correlate for her neurological symptoms except a small petechial cortical hemorrhage in the right parietooccipital gyrus which may account for her TTP-related anxiety disorder. A cerebral HMPAO-SPECT showed long-standing right-sided hypoperfusion compatible with residual vasculature changes. The possible causes for the clinico-neuroradiological discrepancies are discussed in view of the literature.

  15. Rituximab chimeric anti-CD20 monoclonal antibody treatment for adult refractory idiopathic thrombocytopenic purpura

    DEFF Research Database (Denmark)

    Braendstrup, Peter; Bjerrum, Ole W; Nielsen, Ove J;

    2005-01-01

    . Recent studies have shown that rituximab, a chimeric anti-CD20 monoclonal antibody, is useful in the treatment of these patients, with overall response rates of about 50%. Most published reports have included a small number patients including case reports. The present study reports the results...... of a retrospective Danish multicenter study of rituximab in the treatment of adult patients with refractory ITP. Thirty-five patients (median age 52 years, range 17-82 years, 17 males) were included. One patient had immune thrombocytopenia and neutropenia. All patients had received prednisolone (Pred). Next to Pred...... of rituximab. The large majority of patients also received Pred and, in some cases, other concomitant immunosuppressive treatment during part of their rituximab treatment. A complete response (CR) was defined as a rise in the platelet count > 100 x 10(9)/L, a partial response (PR) as a rise in the platelet...

  16. Intravenous immunoglobulin therapy of idiopathic thrombocytopenic purpura in childhood and adolescence.

    Science.gov (United States)

    Bussel, J B; Hilgartner, M W

    1987-09-01

    Intravenous immunoglobulin is not only a dramatic clinical therapy, but it is also extremely interesting in regard to mechanism of action. The high cost of therapy limits its application, yet it appears to be equal to or perhaps slightly more effective than corticosteroids as a treatment of ITP and is far less toxic with prolonged use. The appropriate place for its exact use remains to be determined but probably includes patients urgently requiring rapid platelet increases (in conjunction with steroids), treatment of immunocompromised patients, and treatment of chronic patients, either children to avoid splenectomy or adults with severe disease after splenectomy. Controlled trials to resolve these clinical questions are urgently needed. Existing studies on its mechanisms of actions are very interesting and have furthered our understanding of the pathophysiology of ITP. Although future work may lead to further applications, initial enthusiasm for the use of IVGG in the treatment of other autoimmune diseases with the exception of myasthenia gravis has been limited by subsequent clinical experience. PMID:2452151

  17. Spontaneous Tumor Lysis Syndrome and Secondary Thrombotic Thrombocytopenic Purpura in Early Stage Colorectal Cancer

    Institute of Scientific and Technical Information of China (English)

    Saad Usmani; Joel Appel; Zainab Shahid; Husain Saleh

    2008-01-01

    @@ Acute tumor lysis syndrome (ATLS) is a well-described oncological emergency that is usually associated with hematological malignan-cies complicated by treatment. It is typically related to a high tumor burden, rapidly growing and chemosensitive malignancies.

  18. Current understanding of alloimmunity of the intestinal graft

    NARCIS (Netherlands)

    Trentadue, Guido; Dijkstra, Gerard

    2015-01-01

    Purpose of review This review focuses on the known mechanisms of alloimmunity that occur after transplantation and what is being done in order to improve graft and patient survival, particularly in the long term. Recent findings The presence of mismatched antigens and epitopes might relate directly

  19. [Purpura: primary systemic amyloidosis manifestation].

    Science.gov (United States)

    Lestre, Sara; Gonçalves, Andreia; João, Alexandre; Ferreira, Ana; Apetato, Margarida

    2009-01-01

    Primary Systemic Amyloidosis (AL) is the most frequent form of systemic amyloidosis and its morbilility is associated with immunoglobulin light chains deposition in vital organs. The mucocutaneous manifestations occur in about 30-40% of the cases and are important in diagnostic suspicion, once they appear in early stages of disease. We report a 71-years-old female patient, with disseminated purpura and cutaneous fragility with 6 months of evolution, accompanied by recent complaints of dysphagy. The first laboratory evaluation didn't show any alterations. The histological and immunohistochemical study of subcutaneous abdominal fat and skin biopsy showed lambda type amyloid protein. In the systemic work-up, we highlight a proteinúria > 1g/24h with Bence Jones proteins and the presence of monoclonal immunoglobulin light chain (lambda type) in serum immunoelectrophoresis. With the diagnosis of primary systemic amyloidosis, treatment with prednisolone and melphalan was started. PMID:19686633

  20. ALLOIMMUNIZATION IN SICKLE CELL DISEASE: CHANGING ANTIBODY SPECIFICITIES AND ASSOCIATION WITH CHRONIC PAIN AND DECREASED SURVIVAL

    Science.gov (United States)

    Telen, Marilyn J.; Afenyi-Annan, Araba; Garrett, Melanie E.; Combs, Martha Rae; Orringer, Eugene P.; Ashley-Koch, Allison E.

    2014-01-01

    BACKGROUND: Alloimmunization remains a significant complication of transfusion and has been associated with multiple factors, including inflammation, an important pathophysiologic mechanism in sickle cell disease (SCD). We explored whether alloimmunization is associated with disease severity in SCD. STUDY DESIGN AND METHODS Adult SCD patients were enrolled in a study of outcome modifying genes in SCD. Historical records of patients with SCD at two participating institutions were reviewed for data on antigen phenotype and alloimmunization. Differences in demographic, clinical and laboratory findings, end organ damage, and overall disease severity were then compared between alloimmunized and non-alloimmunized patients. RESULTS Of 319 patients, 87 (27%) were alloimmunized. Alloantibody specificities differed from those previously described, especially due to the significantly higher frequency of anti-S. Although alloimmunization was not associated with frequency of vaso-occlusive episodes, a higher percentage of alloimmunized patients had chronic pain, as defined by daily use of short acting narcotics (p=0.006), long acting narcotics (p=0.013), or both (p=0.03). Additionally, alloimmunized patients had poorer survival (HR=1.92, p=0.01) and were more likely to have avascular necrosis (p=0.024), end-organ damage (p=0.049) and red cell autoantibodies (p<0.001), even after controlling for the effects of age, gender, and hemoglobin diagnosis. Alloimmunization was not associated with other SCD related complications, such as acute chest syndrome or stroke. CONCLUSIONS Alloimmunization in SCD may be associated with chronic pain, risk of end-organ damage, and shorter survival. These novel findings suggest new directions for the investigation of immune response-mediated pathways common to alloimmunization and chronic pain. PMID:25444611

  1. Alloimmunization in Patients with Sickle Cell Disease in French Guiana

    Directory of Open Access Journals (Sweden)

    Narcisse Elenga

    2015-01-01

    Full Text Available This study in French Guiana assessed the frequency of alloimmunization to red cell antigens in sickle cell disease patients over 1995–2011 and identified the most common antibodies. A retrospective analysis of the transfusion history and medical records of 302 patients showed that 29/178 transfused patients had developed alloantibodies (16%. The most frequent alloantibodies were anti-LE1, anti-MNS1, anti-LE2, and anti-FY1 and were developed after transfusion of standard red cell units. The frequency of the clinically significant antibodies in this population of SCD patients was 11% (19/178. The antibodies found on those patients who had delayed hemolytic transfusion reaction were anti-K1, anti-FY1, and anti-MNS3. The strategies used to decrease alloimmunization in French Guiana are discussed.

  2. [Difficulties of the care of public antigen alloimmunization].

    Science.gov (United States)

    Ben Amor, I; Rekik, T; Louati, N; Lahiani, W; Rekik, H; Peyrard, T; Menif, H; Gargouri, J

    2016-05-01

    Alloimmunization against high-frequency erythrocyte antigens is a problematic situation in terms of laboratory diagnosis, transfusion and obstetrical management. We report the case of a pregnant woman alloimmunized against public Ag. We detail the difficulties of alloantibody (Ab) identification and transfusion management of the deliveries. A 29-year-old pregnant woman was hospitalized in gynecology and obstetrics departments at 36 weeks of gestation for assessment of hydrops fetalis. Antibody identification test revealed the presence of a pan-reactive antibody. Investigations realized in CNRGS (Paris) concluded in anti-GE2+anti-RH3+autoantibody. The red cell phenotype was GE: -2,3. A therapeutic interruption of the pregnancy was indicated. A program of autologous transfusion was organized with withdrawal of 2 units of blood. The 2nd pregnancy took place normally. Before delivery, an autologous blood reserve consisting of 2 red cell packs and 2 fresh frozen plasma was withdrawn and transfused after delivery. The management of anti-public alloimmunization poses several problems. The first one is of diagnostic nature with, on the one hand, the difficulty of Ab identification by the available red cell panels and, on the other hand, the possible presence of alloantibodies of transfusional or obstetric interest masked by anti-public Ab. The second is represented by transfusional care of these patients. In the absence of a national reserve of frozen rare blood, the autologous transfusion remains the only alternative. However, it can answer only a limited number of indications and only in case of moderate blood loss.

  3. Cost-effectiveness of antenatal screening for neonatal alloimmune thrombocytopenia

    DEFF Research Database (Denmark)

    Killie, M K; Kjeldsen-Kragh, J; Husebekk, A;

    2007-01-01

    -4 weeks before term. Severely thrombocytopenic newborn were transfused immediately with compatible platelets. MAIN OUTCOME MEASUREMENTS: Quality-adjusted life years (QALYs) and costs. RESULTS: Compared with no screening, a programme of screening and subsequent treatment would generate between 210 and 230...... in Norway encompassing a 2.78 million population. POPULATION: Pregnant women (n = 100,448) screened for human platelet antigen (HPA) 1a and anti-HPA 1a antibodies, and their babies. METHOD: Decision tree analysis. In three branches of the decision tree, pregnant women entered a programme while in one...

  4. Role of heat shock protein 70 in innate alloimmunity

    Directory of Open Access Journals (Sweden)

    Walter G. eLand

    2012-01-01

    Full Text Available This article briefly describes our own experience with the proven demonstration of heat shock protein 70 in reperfused renal allografts from brain-deaddonors and reflects about its potential role as a typical damage-associated molecular pattern (DAMP in the setting of innate alloimmunity. In fact, our group was able to demonstrate a dramatic up-regulation of heat shock protein 70 expression after postischemic reperfusion of renal allografts. Of note, up-regulation of this stress protein expression, although to a lesser extent, was already observed after cold storage of the organ indicating that this molecule is already induced in the stressed organism of a brain-dead donor. However, whether or not the dramatic up-regulation of heat shock protein 70 expression contributes to mounting an innate alloimmune response cannot be judged in view of these clinical findings.Nevertheless, heat shock protein 70, since generated in association with postischemic reperfusion-induced allograft injury, can be called a typical DAMP - as can everymolecule be termed a DAMP that is generated in associationwith any stressful tissue injury regardless of its final positive or negative regulatory function within the innate immune response elicited by it.In fact, as we discuss in this article, the context-dependent, even contradistinctive activities of heat shock protein 70 reflect the biological phenomenon that, throughout evolution, mammals have developed an elaborate network of positive and negative regulatory mechanisms, which provide balance between defensive and protective measures against unwarranted destruction of the host. In this sense, up-regulated expression of heat shock protein 70 in an injured allograft might reflect a pure protective response against the severe oxidative injury of a reperfused donor organ. On the other hand, up-regulated expression of this stress protein in an injured allograft might reflect a(futile attempt of the innate immune system to

  5. Epidemiologi, sygdomspræsentation og forløb af idiopatisk trombocytopenisk purpura hos børn i Danmark 1998-2000

    DEFF Research Database (Denmark)

    Kjaersgaard, Mimi; Edslev, Pernille Wendtland; Rosthøj, Steen

    2008-01-01

    INTRODUCTION: Idopathic Thrombocytopenic Purpura (ITP) is a condition with isolated thrombocytopenia and bleeding symptoms in skin and mucous membranes. It is easy to establish the diagnosis, but treatment is controversial, possibly due to different estimates of the risk for serious bleeding. We...... present the epidemiology and clinical course of ITP during the first 6 months after diagnosis in Danish children diagnosed from 1998-2000. MATERIALS AND METHODS: The Nordic Society of Paediatric Haematology and Oncology conducted a prospective registration study of children with newly diagnosed ITP from...... 1998-2000. The study included children ITP and platelet count (TBC) ITP related episodes was recorded at diagnosis and during the first six months. RESULTS: Of 109 included...

  6. Føtal og neonatal alloimmun trombocytopeni er en mulig fatal tilstand

    DEFF Research Database (Denmark)

    Taaning, Ellen Birkerod Morling; Kjeldsen-Kragh, Jens; Hedegaard, Morten;

    2011-01-01

    Fetal and neonatal alloimmune thrombocytopenia (FNAIT) may lead to intracranial haemorrhage (ICH) resulting in neurological damage or death. In FNAIT, transplacental maternal antibodies cause destruction of fetal platelets. Maternal immunisation occurs to fetal human platelet antigens (HPAs...

  7. Study of red blood cell alloimmunization in multitransfused thalassemic children of Jammu region

    OpenAIRE

    Ashu Dogra; Meena Sidhu; Raman Kapoor; Dinesh Kumar

    2015-01-01

    Introduction: Thalassemia is one of the most common genetic disorder of hemoglobin synthesis in Jammu region. Although RBC transfusion is life saving for these patients, it may be associated with some complications like RBC alloimmunization. Thus, the aim of this study was to determine the frequency of alloimmunization and the most common alloantibodies involved. Material and Methods: This was a descriptive study involving a total of 70 thalassemic patients in the age range of 2-17 years rece...

  8. The Nlrp3 Inflammasome Does Not Regulate Alloimmunization to Transfused Red Blood Cells in Mice

    Directory of Open Access Journals (Sweden)

    David R. Gibb

    2016-07-01

    Full Text Available Red blood cell (RBC transfusions are essential for patients with hematological disorders and bone marrow failure syndromes. Despite ABO matching, RBC transfusions can lead to production of alloantibodies against “minor” blood group antigens. Non-ABO alloimmunization is a leading cause of transfusion-associated mortality in the U.S. Despite its clinical importance, little is known about the immunological factors that promote alloimmunization. Prior studies indicate that inflammatory conditions place patients at higher risk for alloimmunization. Additionally, co-exposure to pro-inflammatory pathogen associated molecular patterns (PAMPs promotes alloimmunization in animal models, suggesting that RBC alloimmunization depends on innate immune cell activation. However, the specific innate immune stimuli and sensors that induce a T cell-dependent alloantibody response to transfused RBCs have not been identified. The NLRP3 inflammasome senses chemically diverse PAMPs and damage associated molecular patterns (DAMPs, including extracellular ATP and iron-containing heme. We hypothesized that activation of the NLRP3 inflammasome by endogenous DAMPs from RBCs promotes the alloimmune response to a sterile RBC transfusion. Using genetically modified mice lacking either NLRP3 or multiple downstream inflammasome response elements, we ruled out a role for the NLRP3 inflammasome or any Caspase-1 or -11 dependent inflammasome in regulating RBC alloantibody production to a model antigen.

  9. Low incidence of anti-D alloimmunization following D+ platelet transfusion: The Anti-D Alloimmunization after D-incompatible Platelet Transfusions (ADAPT) study

    OpenAIRE

    Cid, Joan; Lozano, Miguel; Ziman, Alyssa; West, Kamille A.; O'Brien, Kerry L.; Murphy, Michael F; Wendel, Silvano; Vázquez, Alejandro; Ortín, Xavier; Hervig, Tor A.; Delaney, Meghan; Flegel, Willy A.; Yazer, Mark H.

    2014-01-01

    The reported frequency of D alloimmunization in D- recipients after transfusion of D+ platelets varies. This study was designed to determine the frequency of D alloimmunization, previously reported to be an average of 5%±2%. A primary anti-D immune response was defined as the detection of anti-D ≥28 days following the first D+ platelet transfusion. Data were collected on 485 D- recipients of D+ platelets in 11 centres between 2010-2012. Their median age was 60 (range 2-100) years. Diagnoses i...

  10. On the association of the platelet-specific alloantigen, Pena, with glycoprotein IIIa. Evidence for heterogeneity of glycoprotein IIIa.

    OpenAIRE

    Furihata, K; Nugent, D. J.; Bissonette, A; Aster, R H; Kunicki, T J

    1987-01-01

    Neonatal alloimmune thrombocytopenic purpura associated with a new platelet-specific alloantigen Pena has been reported. We now provide direct evidence that the Pena determinant is associated with glycoprotein (GP) IIIa, but that it is distinct from epitopes that define the PlA system. By ELISA wherein monoclonal antibodies specific for GPIIb (Tab) and specific for GPIIIa (AP3) were used to capture and hold antigens from a platelet lysate prepared under conditions that generate free GPIIb and...

  11. Predictors of Red Cell Alloimmunization in Kurdish Multi Transfused Patients with Hemoglobinopathies in Iraq.

    Science.gov (United States)

    Al-Mousawi, Muqdad M N; Al-Allawi, Nasir A S; Alnaqshabandi, Rubad

    2015-01-01

    Hemoglobinopathies are significant health problems in Iraq, including its Northern Kurdistan region. One of the essential components of management of these disorders is regular lifelong blood transfusions. The latter is associated with several complications including red cell alloimmunization. No study has looked at the frequency of alloimmunization and its associations in the country. To address the latter issue, 401 multi transfused patients [311 with β-thalassemia (β-thal) syndrome and 90 with sickle cell disease], registered at a large thalassemia care center in Iraqi Kurdistan had their records reviewed, and their sera tested for atypical antibodies using screening and extended red cell panels. Red cell alloimmunization was detected in 18 patients (4.5%) with a total of 20 alloantibodies, while no autoantibodies were detected. The most frequent alloantibody was anti-E, followed by anti-D, anti-K, anti-C(w), anti-C, anti-c and anti-Le(a). Ethnicity was an important predictor of alloimmunization, while age at start of transfusion (>2 vs. ≤2 years) (p = 0.005), Rhesus D (RhD) negative status (p = 0.0017) and history of previous transfusion reactions (p = 0.007) showed a statistically significant higher rate of alloimmunization. However, patients' age, gender, number of units transfused, underlying diagnosis and splenectomy were not significantly associated with alloimmunization. Based on our observations, measures to reduce alloimmunization rates may include extended matching for Rhesus and Kell antigens and early initiation of blood transfusions. PMID:26327166

  12. Psychogenic purpura: a most distressing case.

    Science.gov (United States)

    Koblenzer, P J; Koblenzer, C S

    1990-01-01

    A case of psychogenic purpura is likely to be unforgettable for everyone concerned. The patient's bizarre and dramatic appearance can frighten both the patient and medical personnel unfamiliar with the condition, causing panic and intense demands for drastic, inappropriate remedies. Our second experience with this condition was such a memorable case. We stress how difficult it can be to withstand the pressures of colleagues, the patient, and his or her family, and how important it sometimes is to be resolute regarding the need for conservative management. PMID:2298043

  13. Henoch Schönlein purpura associated with pulmonary adenocarcinoma

    Directory of Open Access Journals (Sweden)

    Tetsuka Takafumi

    2011-06-01

    Full Text Available Abstract Introduction Henoch-Schönlein purpura is a common immunoglobulin A-mediated vasculitis syndrome in children. Henoch-Schönlein purpura can also affect adults and is probably related to malignancy. Case presentation We report the case of a 61-year-old Japanese man who presented for examination after an abnormal shadow was detected by chest radiography. He received a diagnosis of pulmonary adenocarcinoma, stage IV. Purpura on the legs, abdominal pain, diarrhea, hematuria and proteinuria developed at this time. Henoch-Schönlein purpura was diagnosed, base on the clinical symptoms and histological findings of biopsy specimens of the skin, which showed vasculitis with immunoglobulin A deposits. Our patient received chemotherapy with gemcitabine after successful steroid therapy for the Henoch-Schönlein purpura. Conclusion Although hematological malignancies are well-known causes of vasculitides, cases of Henoch-Schönlein purpura associated with lung adenocarcinoma are rare. Our patient was treated with corticosteroid therapy, which cleared the purpura and cytotoxic chemotherapy for the non-small cell lung cancer. However, he died from heart failure due to cardiac tamponade.

  14. Low frequency of anti-D alloimmunization following D+ platelet transfusion: the Anti-D Alloimmunization after D-incompatible Platelet Transfusions (ADAPT) study.

    Science.gov (United States)

    Cid, Joan; Lozano, Miguel; Ziman, Alyssa; West, Kamille A; O'Brien, Kerry L; Murphy, Michael F; Wendel, Silvano; Vázquez, Alejandro; Ortín, Xavier; Hervig, Tor A; Delaney, Meghan; Flegel, Willy A; Yazer, Mark H

    2015-02-01

    The reported frequency of D alloimmunization in D- recipients after transfusion of D+ platelets varies. This study was designed to determine the frequency of D alloimmunization, previously reported to be an average of 5 ± 2%. A primary anti-D immune response was defined as the detection of anti-D ≥ 28 d following the first D+ platelet transfusion. Data were collected on 485 D- recipients of D+ platelets in 11 centres between 2010 and 2012. Their median age was 60 (range 2-100) years. Diagnoses included: haematological (203/485, 42%), oncological (64/485, 13%) and other diseases (218/485, 45%). Only 7/485 (1·44%; 95% CI 0·58-2·97%) recipients had a primary anti-D response after a median serological follow-up of 77 d (range: 28-2111). There were no statistically significant differences between the primary anti-D formers and the other patients, in terms of gender, age, receipt of immunosuppressive therapy, proportion of patients with haematological/oncological diseases, transfusion of whole blood-derived or apheresis platelets or both, and total number of transfused platelet products. This is the largest study with the longest follow-up of D alloimmunization following D+ platelet transfusion. The low frequency of D alloimmunization should be considered when deciding whether to administer Rh Immune Globulin to D- males and D- females without childbearing potential after transfusion of D+ platelets. PMID:25283094

  15. A rare and successfully managed case of idiopathic thrombocytopenic purpura (ITP with previous caesarean with splenectomy with hepatitis C positive

    Directory of Open Access Journals (Sweden)

    Rajshree Dayanand Katke

    2014-08-01

    Full Text Available ITP occurs in approximately 2 of 1000 pregnant women. ITP may develop at any time during pregnancy, but is often initially recognized in the first trimester and is the most common cause of isolated thrombocytopenia in this time period. A 33 year old, married since 6 years, G2p1l1 with 33 weeks gestation referred to our tertiary centre. She was a known case of ITP with splenectomy done. She had a 5 year old male child, delivered by LSCS. She was diagnosed as having ITP at the age of 12 years. HCV antibody was weakly positive. ANA was positive. Emergency LSCS was done in view of scar tenderness. Post op she developed epistaxis, bleeding gums and per vaginum. Patient received multiple FFP, platelet transfusions and responded to treatment with methylprednisolone. The diagnosis and management of ITP in pregnancy is similar to that in the non-pregnant adult patient, but the risks to the developing fetus must be taken into account when choosing treatment and the maintenance of a safe platelet count, rather than prolonged remission, is the goal. Mode of delivery must be guided by obstetrical indications. [Int J Reprod Contracept Obstet Gynecol 2014; 3(4.000: 1119-1121

  16. Detection of alloimmunization to ensure safer transfusion practice

    Directory of Open Access Journals (Sweden)

    Rashmi Sood

    2013-01-01

    Full Text Available Background: Serological safety is an integral part of overall safety for blood banks. Emphasis is on the use of routinue Red Blood Cell (RBC antibody screen test, at set time intervals, to reduce risks related to alloantibodies. Also emphasis is on importance of issuing antigen negative blood to alloantibody positive patients. Effect of using leucodepleted blood on the rate of alloimmunization is highlighted. The concept of provision of phenotypically matched blood is suggested. Materials and Methods: Antibody screen test is important to select appropriate blood for transfusion. Repeat antibody screen testing, except if time interval between the earlier and subsequent transfusion was less than 72 hours, followed by antibody identification, if required, was performed in patients being treated with repeat multiple blood transfusions. Between February 2008 and June 2009, repeat samples of 306 multi-transfused patients were analyzed. Search for irregular antibodies and reading of results was conducted using RBC panels (three-cell panel of Column Agglutination Technology (CAT and two cell panel of the Solid Phase Red Cell Adherence Technology (SPRCAT. Specificities of antibodies were investigated using appropriate panels, 11 cell panel of CAT and 16 cell panel of SPRCA. These technologies, detecting agglutination in columns and reactions in solid phase, evaluate the attachment of irregular incomplete antibody to antigen in the first phase of immunological reaction more directly and hence improve the reading of agglutination. Three to four log leuco reduced red blood cells were transfused to patients in the study using blood collection bags with integral filters. Results: Alloimmunization rate of 4.24% was detected from 306 multiply transfused patients tested and followed up. The Transfusion therapy may become significantly complicated. Conclusion: Red cell antibody screening and identification and subsequent issue of antigen negative blood have a

  17. Augmentation of Recipient Adaptive Alloimmunity by Donor Passenger Lymphocytes within the Transplant

    Directory of Open Access Journals (Sweden)

    Ines G. Harper

    2016-05-01

    Full Text Available Chronic rejection of solid organ allografts remains the major cause of transplant failure. Donor-derived tissue-resident lymphocytes are transferred to the recipient during transplantation, but their impact on alloimmunity is unknown. Using mouse cardiac transplant models, we show that graft-versus-host recognition by passenger donor CD4 T cells markedly augments recipient cellular and humoral alloimmunity, resulting in more severe allograft vasculopathy and early graft failure. This augmentation is enhanced when donors were pre-sensitized to the recipient, is dependent upon avoidance of host NK cell recognition, and is partly due to provision of cognate help for allo-specific B cells from donor CD4 T cells recognizing B cell MHC class II in a peptide-degenerate manner. Passenger donor lymphocytes may therefore influence recipient alloimmune responses and represent a therapeutic target in solid organ transplantation.

  18. Genetics of Transfusion Recipient Alloimmunization: Can Clues from Susceptibility to Autoimmunity Pave the Way?

    OpenAIRE

    Tatari-Calderone, Zohreh; Luban, Naomi L.C.; Vukmanovic, Stanislav

    2014-01-01

    The search for genetic determinants of alloimmunization in sickle cell disease transfusion recipients was based on two premises: i) that polymorphisms responsible for stronger immune and/or inflammatory responses and hemoglobin βS mutation were co-selected by malaria; and ii) that stronger responder status contributes to development of lupus. We found a marker of alloimmunization in the gene encoding for Ro52 protein, also known as Sjögren syndrome antigen 1 (SSA1) and TRIM21. Surprisingly, t...

  19. Greater ethnic diversity correlates with lower HIV prevalence in Africa: justification for an alloimmunity vaccine

    Directory of Open Access Journals (Sweden)

    Zamani C

    2013-04-01

    Full Text Available Christopher Zamani, Jared D Elzey, James EK Hildreth Center for AIDS Health Disparities Research at Meharry Medical College, Nashville, Tennessee, USA Purpose: After decades of research, AIDS continues to be a major pandemic and to date, adaptive immunity vaccine designs have had little to no success. Data indicate the alloimmune response is a potent mitigator of human immunodeficiency virus (HIV infection, for which experiments of nature should be demonstrable to justify pursuit of an alloimmune vaccine strategy. We sought to determine if large-scale alloimmune diversity correlates with lower HIV infection rates. Methods: Using published data of African linguistic groups to determine sub-Saharan country ethnicity profiles as a proxy for human leukocyte antigen (HLA diversity, a correlation analysis was performed against respective sub-Saharan country HIV infection rates. Ethnicity data from 37 sub-Saharan nations in 2003 and from 38 nations in 2005 were used to calculate the Meyers-Macintosh ethnic diversity score for each nation as the independent variable. World Health Organization data on HIV infection rates for the same countries were used as the dependent variable. The main outcome measure was the correlation coefficient of ethnic diversity versus HIV infection rate. Results: A significant negative correlation was shown between ethnic diversity and HIV infection: for 2003 data, -0.4586 (two-tailed P-value of 0.0043; and, for 2005 data, -0.3866 (two-tailed P-value of 0.0165. Conclusion: In conjunction with substantial evidence that alloimmunity confers protection against HIV transmission and recent work identifying specific anti-HIV mechanisms, this analysis strongly justifies an HLA-based alloimmune vaccine strategy against HIV. Keywords: AIDS, adaptive immunity, human leukocyte antigen (HLA

  20. Alloimmune activation promotes anti-cancer cytotoxicity after rat liver transplantation.

    Directory of Open Access Journals (Sweden)

    Stéphanie Lacotte

    Full Text Available Liver transplantation for hepatocellular carcinoma (HCC results in a specific condition where the immune response is potentially directed against both allogeneic and cancer antigens. We have investigated the level of anti-cancer immunity during allogeneic immune response. Dark Agouti-to-Lewis and Lewis-to-Lewis rat liver transplantations were performed and the recipients anti-cancer immunity was analysed at the time of alloimmune activation. The occurrence of rejection in the allogeneic recipients was confirmed by a shorter survival (p<0.01, increased liver function tests (p<0.01, the presence of signs of rejection on histology, and a donor-specific ex vivo mixed lymphocyte reaction. At the time of alloimmune activation, blood mononuclear cells of the allogeneic group demonstrated increased anti-cancer cytotoxicity (p<0.005, which was related to an increased natural killer (NK cell frequency (p<0.05 and a higher monocyte/macrophage activation level (p<0.01. Similarly, liver NK cell anti-cancer cytotoxicity (p<0.005, and liver monocyte/macrophage activation levels (p<0.01 were also increased. The alloimmune-associated cytotoxicity was mediated through the NKG2D receptor, whose expression was increased in the rejected graft (p<0.05 and on NK cells and monocyte/macrophages. NKG2D ligands were expressed on rat HCC cells, and its inhibition prevented the alloimmune-associated cytotoxicity. Although waiting for in vivo validation, alloimmune-associated cytotoxicity after rat liver transplantation appears to be linked to increased frequencies and levels of activation of NK cells and monocyte/macrophages, and is at least in part mediated through the NKG2D receptor.

  1. [Severe digestive manifestations of rheumatoid purpura in adults].

    Science.gov (United States)

    Roche, B; Blazquez, M; Charlier, A; Bognel, J C

    1994-01-01

    The authors report three cases of Scholein-Henoch's purpura in adults which were characterized by atypical severity of the digestive signs which led to laparotomy. In one case, the digestive signs preceded the onset of the cutaneous purpura, which made the diagnosis difficult. The digestive impact was confirmed endoscopically (petechia, ulceration, ulcerated stenosis) in two of the three patients. Damage of the small intestine predominated, as in the literature, but only one patient required resection of the intestine. These observations highlight the importance of endoscopic exploration in cases of abdominal signs combined with Scholein-Henoch's purpura in adults. They also demonstrate the difficulty of evaluating the prognosis and treatment in severe, peritoneo-occlusive forms. PMID:8192421

  2. [Henoch-Schonlein purpura involving the penis: a case report].

    Science.gov (United States)

    Croche Santander, Borja; Campos, Elena; Sánchez, Adela; Marcos, Laura; Díaz, Isabel; Toro, Cristóbal

    2016-08-01

    Schonlein-Henoch purpura accounts for the majority of cases of systemic vasculitis in children. Classical presentation is characterized by palpable purpura, glomerulonephritis, arthralgias and abdominal pain. Although genitourinary manifestations, in form of testicular and scrotal involvement, have been widely described, penile involvement remains an extremely rare complication. We report a case of a 6-year-old boy who presented with purpuric rash on the glans, prepuce and penile shaft, with painful edema in the penile region. He also had a 3-days history of fever, palpable purpuric rash on the buttocks and lower extremities along with right wrist pain. He was admitted with the diagnosis of Schonlein-Henoch purpura with penile involvement. After 2 days on oral steroids therapy (prednisone) a marked improvement was observed. PMID:27399026

  3. Emerging science, emerging ethical issues: who should fund innate alloimmunity-suppressing drugs?

    Science.gov (United States)

    Land, W G; Gutmann, Th; Daar, A S

    2008-01-01

    An emerging body of evidence suggests that the innate immune system plays a critical role in allograft rejection. Any injury to the donor organ, e.g. the reperfusion injury, induces an inflammatory milieu in the allograft which appears to be the initial event for activation of the innate immune system. Injury-induced intragraft damage- associated molecular patterns (DAMPs) are recognized by donor-derived and recipient-derived, TLR4/2-bearing immature dendritic cells (iDCs). After recognition, these cells mature and initiate allorecognition/alloactivation in the lymphoid system of the recipient. Indeed, the key "innate" event, leading to activation of the adaptive alloimmune response, is the injury-induced, TLR4-triggered, and NFkappaB-mediated maturation of DCs ("innate alloimmunity"). Time-restricted treatment of innate immune events would include 1) treatment of the donor during organ removal, 2) in-situ/ex-vivo treatment of the donor organs alone, and 3) treatment of the recipient during allograft reperfusion and immediately postoperatively. Treatment modalities would include 1) minimization of the oxidative allograft injury with the use of antioxidants; 2) prevention of the TLR4-triggered maturation of DCs with the use of TLR4-antagonists; 3) inhibition of complement activation with the use of complement inhibiting agents. According to data from clinical and experimental studies it can be assumed that successful suppression of innate alloimmune events results in either subsequent significant reduction in, or even complete avoidance of the currently applied adaptive alloimmunity-suppressing drugs. However, in view of the time-restricted period of treatment, and the fear to potentially destroy its own business with currently applied alloimmunity-suppressing drugs, the pharmaceutical industry is still, but quite legitimately, reluctant to invest in the high cost of clinical development of those drugs for transplant patients because there are no marketing interests

  4. Delayed cord clamping in red blood cell alloimmunization: safe, effective, and free?

    Science.gov (United States)

    McAdams, Ryan M

    2016-04-01

    Hemolytic disease of the newborn (HDN), an alloimmune disorder due to maternal and fetal blood type incompatibility, is associated with fetal and neonatal complications related to red blood cell (RBC) hemolysis. After delivery, without placental clearance, neonatal hyperbilirubinemia may develop from ongoing maternal antibody-mediated RBC hemolysis. In cases refractory to intensive phototherapy treatment, exchange transfusions (ET) may be performed to prevent central nervous system damage by reducing circulating bilirubin levels and to replace antibody-coated red blood cells with antigen-negative RBCs. The risks and costs of treating HDN are significant, but appear to be decreased by delayed umbilical cord clamping at birth, a strategy that promotes placental transfusion to the newborn. Compared to immediate cord clamping (ICC), safe and beneficial short-term outcomes have been demonstrated in preterm and term neonates receiving delayed cord clamping (DCC), a practice that may potentially be effective in cases RBC alloimmunization. PMID:27186530

  5. Treatment of D alloimmunization in pregnancy with plasmapheresis and intravenous immune globulin: case report.

    Science.gov (United States)

    Fernández Alba, Juan J; León, Raquel; González-Macías, Carmen; Paz, Antonio; Prado, Fabiana; Moreno, Luis J; Torrejón, Rafael

    2014-08-01

    The prevalence of D alloimmunization occurs between 0.15% and 0.4%. The anti-D can cross the placenta and cause hemolysis and fetal anemia. At present, a Doppler study of the middle cerebral artery allows the monitoring of the degree of fetal anemia. The treatment in cases of moderate to severe anemia in fetuses of less than 34-35 weeks of gestation is intrauterine transfusion via cordocentesis. However, with high titers of anti-D, in the absence of fetal anemia it is possible to modulate the maternal immune response by plasmapheresis and intravenous immunoglobulin administration. We present a case report of an Rh(D) alloimmunized pregnancy treated with plasmapheresis followed by intravenous immunoglobulin administration. We performed a caesarean section at 31 weeks, 5 days of gestation. The hemoglobin at birth was 13.8 g/dl and hematocrit 40.8%. Intrauterine transfusion was not necessary. PMID:25312036

  6. Anti-E Alloimmunization: A Rare Cause of Severe Fetal Hemolytic Disease Resulting in Pregnancy Loss

    Directory of Open Access Journals (Sweden)

    An-Shine Chao

    2009-01-01

    Full Text Available We report a case of severe intrauterine hemolysis caused by sole anti-E alloimmunization. A 36-year-old multipara woman presented with hydrops fetalis at 27 weeks of gestation. She had a history of previous neonatal death. In this pregnancy, she was found to have very high titer of anti-E antibody. Ultrasonography detected marked skin edema, cardiomegaly, hepatosplenomegaly, pleural effusion, ascites, placentomegaly, and polyhydramnios. The Doppler peak systolic velocity in the middle cerebral artery was 0.8 m/s, indicating severe fetal anemia. Multiple intrauterine transfusions for the anemic fetus were administered. However, persistent severe fetal anemia and placentomegaly caused poor neonatal death and mirror syndrome in the mother. Uncommon red blood cell alloimmunization has to be watched for early in any population, especially in a woman with a history of unexplained perinatal loss.

  7. Dr. Zhao Jianxiong's Experience in Treating Allergic Purpura

    Institute of Scientific and Technical Information of China (English)

    2007-01-01

    @@ Dr. Zhao Jianxiong (赵健雄) has been engaged in traditional Chinese medicine for more than 40 years. He is good at treating severe and obstinate diseases of internal medicine and gynecology with his empirical prescriptions, and excels in the treatment of hematopathy. The following is a brief summary of his experience in the treatment of allergic purpura.

  8. Henoch-Schönlein purpura and meningococcal B vaccination.

    Science.gov (United States)

    Sexton, K; McNicholas, A; Galloway, Y; Radke, S; Kieft, C; Stehr-Green, P; Reid, S; Neutze, J; Drake, R

    2009-03-01

    The risk of Henoch-Schönlein purpura (HSP) following vaccination with a group B meningococcal vaccine was assessed through active hospital safety monitoring. There was no increase in the relative incidence of HSP within 30 days after vaccination nor recurrence in HSP cases who received one or more further vaccine doses (re-challenge). PMID:18650242

  9. Oral purpura as the first manifestation of primary systemic amyloidosis.

    Science.gov (United States)

    McCormick, Robert Stuart; Sloan, Philip; Farr, David; Carrozzo, Marco

    2016-07-01

    Oral blood blisters and purpura are rare features of primary systemic amyloidosis (amyloid light-chain (AL) amyloidosis). We report a case in which these unusual presentations led to a diagnosis of amyloidosis, which enabled effective treatment before organ failure. PMID:26708800

  10. Purpura fulminans associated with acute West Nile virus encephalitis.

    Science.gov (United States)

    Shah, Sheevam; Fite, Laura Paul; Lane, Natalie; Parekh, Palak

    2016-02-01

    Purpura fulminans is a progressive thrombotic disorder that presents with widespread purpura due to deficiency or dysfunction of protein C or protein S. Lesions present as well-demarcated erythematous macules that progress to irregular areas of hemorrhagic necrosis.West Nile virus is a member of the Flaviviridae family transmitted to humans through the bite of various mosquito species. It manifests as West Nile fever in 25% of those infected and less commonly as neuroinvasive disease. An African American man in his fortiespresented with altered mental status and was noted to have evidence of disseminated intravascular coagulation according to his lab data. He then developed dusky skin discoloration and systemic flaccid bullae with desquamation. Biopsy was consistent with purpura fulminans and the patient eventually developed symmetric peripheral gangrene, requiring amputations of all four extremities. Infectious work up revealed positive testing for IgM and IgG antibodies in serum and cerebrospinal fluid leading to the diagnosis of acute West Nile Virus encephalitis. We present this case to describe the rarely reported association of purpura fulminans with West Nile Virus infection.

  11. [Ulcerated duodenitis revealing Henoch-Schönlein purpura].

    Science.gov (United States)

    Marting, A; Defrance, P; Wain, E; Van Severen, M; Deflandre, J

    2015-01-01

    Inflammation and duodenal ulcers can meet many etiologies. We report the case of a young adult with an ulcerated duodenitis revealing Henoch-Schönlein purpura. The abdominal symptoms preceded the emergence of the classical cutaneous signs of the disease. PMID:26376566

  12. Pruritis and palpable purpura from leeches in the Australian Rainforest

    OpenAIRE

    Seiverling, Elizabeth V.; Amrit Khalsa; Hadjh T. Ahrns

    2014-01-01

    Highlights • Leech saliva contains hirudin (a thrombin inhibitor) and histamine. • Leech bites cause pruritis (itching) and purpura (visible hemorrhage into the skin). • Some Australian leeches can transmit trypanosomal infections. • Saturated salt solution, alcohol, or vinegar, may ease leech removal.

  13. Henoch-Schonlein Purpura Nephritis: Pathophysiology, Treatment, and Future Strategy

    NARCIS (Netherlands)

    J.C. Davin

    2011-01-01

    Henoch-Schonlein purpura nephritis is a rare kidney disease leading to chronic kidney disease in a non-negligible percentage of patients. Although retrospective studies suggest beneficial effects of some therapies, prospective randomized clinical trials proving treatment efficacy are still lacking.

  14. Alloimmunization and autoimmunization in transfusion dependent thalassemia major patients: Study on 319 patients

    Directory of Open Access Journals (Sweden)

    Hari Krishan Dhawan

    2014-01-01

    Full Text Available Background: The development of anti-red blood cell antibodies (both allo-and autoantibodies remains a major problem in thalassemia major patients. We studied the frequency of red blood cell (RBC alloimmunization and autoimmunization among thalassemia patients who received regular transfusions at our center and analyzed the factors, which may be responsible for development of these antibodies. Materials and Methods: The study was carried out on 319 multiply transfused patients with β-thalassemia major registered with thalassemia clinic at our institute. Clinical and transfusion records of all the patients were examined for age of patients, age at initiation of transfusion therapy, total number of blood units transfused, transfusion interval, status of splenectomy or other interventions. Alloantibody screening and identification was done using three cell and 11 cell panel (Diapanel, Bio-rad, Switzerland respectively. To detect autoantibodies, autocontrol was carried out using polyspecific coombs (IgG + C3d gel cards. Results: Eighteen patients out of total 319 patients (5.64% developed alloantibodies and 90 (28.2% developed autoantibodies. Nine out of 18 patients with alloantibodies also had autoantibodies. Age at first transfusion was significantly higher in alloimmunized than non-immunized patients (P = 0.042. Out of 23 alloantibodies, 52.17% belonged to Rh blood group system (Anti-E = 17%, Anti D = 13%, Anti-C = 13%, Anti-C w = 9%, 35% belonged to Kell blood group system, 9% of Kidd and 4% of Xg blood group system. Conclusion: Alloimmunization was detected in 5.64% of multitransfused thalassemia patients. Rh and Kell blood group system antibodies accounted for more than 80% of alloantibodies. This study re-emphasizes the need for RBC antigen typing before first transfusion and issue of antigen matched blood (at least for Rh and Kell antigen. Early institution of transfusion therapy after diagnosis is another means of decreasing

  15. Red cell alloimmunization among antenatal women attending a tertiary care hospital in south India

    OpenAIRE

    Jophy Varghese; Chacko, Mary P.; Molly Rajaiah; Dolly Daniel

    2013-01-01

    Background & objectives: Detection of maternal alloimmunization against red cell antigens is vital in the management of haemolytic disease of the foetus and newborn (HDFN). This study was conducted to measure the presence of allosensitization to blood group antibodies in the antenatal women attending a tertiary care hospital and to observe the proportion of minor blood group antibodies to assess the benefit of screening for the same. Methods: All antenatal women registered in the hospital...

  16. Red Cell Alloimmunization to Rhesus Antigen Among Pregnant Women Attending a Tertiary Care Hospital in Oman

    OpenAIRE

    Tamima Al-Dughaishi; Yusra Al Harrasi; Maymoona Al-Duhli; Ikhlass Al-Rubkhi; Nihal Al-Riyami; Al-Riyami, Arwa Z.; Pathare, Anil V.; Vaidyanathan Gowri

    2016-01-01

    Objectives: The detection of maternal alloimmunization against red cell antigens is vital in the management of hemolytic disease of the fetus and newborn. We sought to measure the presence of allosensitization to Rhesus D (RhD) antibodies in antenatal women attending a tertiary care hospital and assess the fetal outcome in sensitized women. Methods: We conducted a retrospective review of pregnant Omani women who registered at the Sultan Qaboos University Hospital between June 2011 and Ju...

  17. Alloimmunization is associated with older age of transfused red blood cells in sickle cell disease

    Science.gov (United States)

    Desai, Payal C.; Deal, Allison M.; Pfaff, Emily R.; Qaqish, Bahjat; Hebden, Leyna M.; Park, Yara A.; Ataga, Kenneth I.

    2016-01-01

    Red blood cell (RBC) alloimmunization is a significant clinical complication of sickle cell disease (SCD). It can lead to difficulty with cross-matching for future transfusions and may sometimes trigger life-threatening delayed hemolytic transfusion reactions. We conducted a retrospective study to explore the association of clinical complications and age of RBC with alloimmunization in patients with SCD followed at a single institution from 2005 to 2012. One hundred and sixty six patients with a total of 488 RBC transfusions were evaluated. Nineteen patients (11%) developed new alloantibodies following blood transfusions during the period of review. The median age of RBC units was 20 days (interquartile range: 14–27 days). RBC antibody formation was significantly associated with the age of RBC units (P = 0.002), with a hazard ratio of 3.5 (95% CI: 1.71–7.11) for a RBC unit that was 7 days old and 9.8 (95% CI: 2.66–35.97) for a unit that was 35 days old, 28 days after the blood transfusion. No association was observed between RBC alloimmunization and acute vaso-occlusive complications. Although increased echocardiography-derived tricuspid regurgitant jet velocity (TRV) was associated with the presence of RBC alloantibodies (P = 0.02), TRV was not significantly associated with alloimmunization when adjusted for patient age and number of transfused RBC units. Our study suggests that RBC antibody formation is significantly associated with older age of RBCs at the time of transfusion. Prospective studies in patients with SCD are required to confirm this finding. PMID:25963831

  18. Anti-E Alloimmunization: A Rare Cause of Severe Fetal Hemolytic Disease Resulting in Pregnancy Loss

    OpenAIRE

    Reyin Lien; Yao-Lung Chang; Szu Ying Ho; Angel Chao; An-Shine Chao

    2010-01-01

    We report a case of severe intrauterine hemolysis caused by sole anti-E alloimmunization. A 36-year-old multipara woman presented with hydrops fetalis at 27 weeks of gestation. She had a history of previous neonatal death. In this pregnancy, she was found to have very high titer of anti-E antibody. Ultrasonography detected marked skin edema, cardiomegaly, hepatosplenomegaly, pleural effusion, ascites, placentomegaly, and polyhydramnios. The Doppler peak systolic velocity in the middle cerebra...

  19. Associations of Rhesus and non-Rhesus maternal red blood cell alloimmunization with stillbirth and preterm birth

    Science.gov (United States)

    Fan, Jing; Lee, Brian K; Wikman, Agneta T; Johansson, Stefan; Reilly, Marie

    2014-01-01

    Background: Although the risks of adverse pregnancy outcomes associated with anti-D antibodies are well-recognized, much less is known concerning alloimmunization with other red blood cell antibodies detected during routine maternal screening. To date, most reports of adverse pregnancy outcomes associated with non-anti-D antibodies have been from small case studies. The aim of this study was to examine the associations of maternal alloimmunization with specific red blood cell antibodies and the risks of preterm birth and stillbirth in the Swedish population. Methods: All antibody screening, outcome and covariate data were obtained through linkages of Swedish national health and data registers. Follow-up in these population-based registers was available up to 31 December 2002. The final study sample consisted of 1 022 569 singleton births from 668 952 mothers during 1987–2002. Results: In total, 1.3% of the 1 022 569 study pregnancies were alloimmunized. In adjusted logistic regression models, compared with having no antibodies, alloimmunization with anti-D, anti-E, anti-C and anti-c was associated with increased risk of both stillbirth and preterm birth. In addition, anti-Kell was associated with increased risk of preterm birth and anti-Lea with increased risk of stillbirth. Compared with firstborn children, risk of preterm birth associated with alloimmunization was greater in subsequent births Conclusions: In the largest study to date, alloimmunization with Rhesus, K- and -Lea red blood cell antibodies increased the risk of preterm birth and/or stillbirth. The association of anti-Lea with stillbirth was an unexpected finding. Further study of the consequences of non-anti-D alloimmunization is warranted. PMID:24801308

  20. Successful treatment of Rh alloimmunization in a twin pregnancy: case report

    Directory of Open Access Journals (Sweden)

    Rahimi Sharbaf F

    2008-09-01

    Full Text Available "n Normal 0 false false false EN-US X-NONE AR-SA MicrosoftInternetExplorer4 /* Style Definitions */ table.MsoNormalTable {mso-style-name:"Table Normal"; mso-tstyle-rowband-size:0; mso-tstyle-colband-size:0; mso-style-noshow:yes; mso-style-priority:99; mso-style-qformat:yes; mso-style-parent:""; mso-padding-alt:0in 5.4pt 0in 5.4pt; mso-para-margin-top:0in; mso-para-margin-right:0in; mso-para-margin-bottom:10.0pt; mso-para-margin-left:0in; line-height:115%; mso-pagination:widow-orphan; font-size:11.0pt; font-family:"Calibri","sans-serif"; mso-ascii-font-family:Calibri; mso-ascii-theme-font:minor-latin; mso-fareast-font-family:"Times New Roman"; mso-fareast-theme-font:minor-fareast; mso-hansi-font-family:Calibri; mso-hansi-theme-font:minor-latin;} Background: The prevalence of Rh alloimmunization has decreased following the use of anti-D immunoglobulin. With serial amniocentesis, Doppler sonography of the middle cerebral artery and treatment of anemia with intrauterine blood transfusion, perinatal mortality has declined. However, Rh alloimmunization in twin pregnancies poses a diagnostic and therapeutic challenge."n"n Case report: We are reporting, for the first time in Iran, the successful treatment of severe Rh alloimmunization in a dichorionic- diamnionic twin pregnancy leading to the live births of both neonates. Before treatment, the fetal hemoglobin levels were 3.1g/dL and 3.9g/dL, with ascites in both fetuses. The fetuses were treated with several IUTs."n"n Results: After treatment, the neonates were delivered, weighing 2200 and 2300g, with good Apgar scores, at a gestational age of 34 weeks. "n"n Conclusion: 10% of population in Iran is Rh-negative, although Prophylaxis for Rh alloimmunization is universal, as other part of the world it cannot irrigated. For the best management of these cases, we need a well-equipped referral center."n"n Keywords: Twin, pregnancy, Rh alloimmunization, intrauterine blood transfusion, Doppler, middle cerebral

  1. [Randomized comparison of intravenous immunoglobulin and methylprednisolone pulse therapy in children with newly diagnosed idiopathic thrombocytic purpura. The Danish ITP Study Group].

    Science.gov (United States)

    Rosthøj, S; Nielsen, S M; Pedersen, F K

    1998-03-01

    Forty three children with newly diagnosed idiopathic thrombocytopenic purpura (ITP), platelet count (pl.c.) below 20 x 10(9)/l, and either clinically significant bleeding or failure to show a spontaneous platelet rise within three days of admission were randomly allocated to treatment with intravenous infusions of either immunoglobulin (IVIG) 1 g/kg or methylprednisolone (MPPT) 30 mg/kg on two consecutive days. Prompt induction of partial remission with pl.c. > 50 x 10(9)/l after 72 hours was seen in 21/23 given IVIG versus 10/20 given MPPT (exact p = 0.003); mean pl.c.s after 72 hours were 188 versus 77 x 10(9)/l (2p 150 x 10(9)/l was achieved in 16/23 versus 10/20 (p = 0.16). During six months follow-up, there were no significant differences regarding relapse rates or chronic course. Eleven children with relapse were crossed over to the alternative treatment arm: the estimated treatment effect in pl.c. after 72 hours was 134 x 10(9)/l in favour of IVIG. These results indicate that IVIG infusions may be preferable to high-dose corticosteroids as initial treatment for children with ITP. PMID:9522658

  2. Simultaneous occurrence of foetal and neonatal alloimmune thrombocytopenia and neonatal neutropenia due to maternal neutrophilic autoantibodies

    DEFF Research Database (Denmark)

    Morling Taaning, Ellen Birkerod; Jensen, Lise; Varming, Kim

    2012-01-01

    Foetal and neonatal alloimmune thrombocytopenia (FNAIT) and neonatal neutropenia caused by maternal autoantibodies against neutrophils are rare disorders. We describe a newborn with severe thrombocytopenia and intracerebral bleeding caused by maternal anti-HPA-3a alloantibodies and mild neutropenia...... caused by maternal autoantibodies against HNA-1b. This appears to be the first case of simultaneous occurrence of these two conditions. Conclusion:  This case report and review of the literature demonstrate that anti-HPA-3a antibodies can be overlooked by standard assays....

  3. Two cases of infectious purpura fulminans and septic shock caused by Capnocytophaga canimorsus transmitted from dogs

    DEFF Research Database (Denmark)

    Christiansen, Claus Behrend; Berg, Ronan Martin Griffen; Plovsing, Ronni R.;

    2012-01-01

    , haemolytic anaemia, metabolic acidosis, and renal failure, which may be common in C. canimorsus-associated purpura fulminans. The patients survived after treatment with broad-spectrum antibiotics and supportive intensive care. C. canimorsus should be considered as a possible cause of infectious purpura...

  4. Expression of AIF-1 and RANTES in Unexplained Spontaneous Abortion and Possible Association with Alloimmune Abortion

    Institute of Scientific and Technical Information of China (English)

    Yong-hong LI; Hai-lin WANG; Ya-juan ZHANG

    2007-01-01

    Objective To investigate the effects of allograft inflammatory factor-1(AIF-1)and (RANTES) in sera and deciduas on unexplained early spontaneous abortion.Methods AIF-1 and RANTES were examined in sera and deciduas/endometria of 43 unexplained early spontaneous abortion women (group A),40 healthy women with early pregnancy(group B)and 20 healthy women with no pregnancy (group C). Immunohistochemistry and enzyme linked immunosorbent assay (ELISA) were used in this study. Results AIF-1 protein was expressed both in deciduas of group A and in endometria of group C.In group A, H scores in the recurrent abortion deciduas specimens were significantly greater than those in the first abortion;in endometrium,expression of AIF-1 was greater in the secretory than in proliferative phase of group C.In group B,concentrations of RANTES in sera were higher in 7th-8th week of pregnancy than in 6th-7th and >8th week of pregnancy;expression of AIF-1 protein showed a negative correlation with RASNTES concentration;a significant increase of the RANTES levels in sera and tissue was observed in group B. Conclusion These results demonstrate, for the first time,that AIF-1 are expressed in deciduas of unexplained spontaneous abortion suggesting that AIF-1 involve in alloimmune abortion; RANTES might act as a novel blocking antibody;AIF-1 and RANTES might act as reliable markers for diagnosis of early alloimmune abortion.

  5. The variation of immature platelet fraction in patients with thrombocytopenic diseases%未成熟血小板分数在血小板减少性疾病中的变化

    Institute of Scientific and Technical Information of China (English)

    蒋伟燕; 江明华; 吴义忠; 章赵华; 陈小剑

    2013-01-01

    目的 了解血小板减少性疾病患者外周血未成熟血小板分数(IPF)、高荧光强度未成熟血小板分数(H-IPF)、未成熟血小板绝对值(IPF#)和血小板平均侧向荧光强度(PLT-X)的变化,探讨其在血小板减少性疾病中的临床意义.方法 选取血小板减少性疾病86例[特发性血小板减少性紫癜(ITP)50例、再生障碍性贫血(AA)15例、急性白血病(AL)21例]、骨髓增生性疾病(MPD)32例[慢性粒细胞白血病(CML)11例、原发性血小板增多症(ET)16例、真性红细胞增多症(PV)5例]和健康对照者50名.应用SYSMEX XE-5000全自动血液分析仪检测各疾病组及健康对照组外周血血小板(PLT)、IPF、H-IPF、IPF#和PLT-X.将ITP组按PLT计数结果分为≤30×109/L、(>30~0.05).ITP各组间IPF差异无统计学意义(P>0.05).结论检测血小板相关参数(IPF、H-IPF和PLT-X)可能有助于血小板减少性疾病的鉴别诊断.%Objective To investigate the variation of immature platelet fraction ( IPF ), high-fluorescence intensity of immature platelet fraction ( H-IPF ), absolute value of immature platelet ( IPF#) and mean side fluorescence intensity of platelet( PLT-X ) in patients with thrombocytopenic diseases and their clinical significance in the thrombocytopenic diseases. Methods The platelet ( PLT), IPF, H-IPF, IPF# and PLT-X of peripheral blood in 86 patients with thrombocytopenic diseases [ 50 cases of idiopathic thrombocytopenic purpura ( ITP), 15 cases of aplastic anemia ( AA ) and 21 cases of acute leukemia ( AL ) ], 32 patients with myeloprolif erative disorders ( MPD ) [ 11 cases of chronic myelogenous leukemia ( CML), 16 cases of essential thrombocythemia( ET ) and 5 cases of polycythemia vera ( PV ) ] and 50 healthy subjects were determined by automatic hematology SYSMEX XE-5000 analyzer. According to the results of PLT, the 50 cases of ITP were classified into ≤30 × 109/L, ( >30- 0. 05 ). There was no statistical significance for IPF in the

  6. Successful prevention of post-transfusion Rh alloimmunization by intravenous Rho (D) immune globulin (WinRho SD).

    Science.gov (United States)

    Anderson, B; Shad, A T; Gootenberg, J E; Sandler, S G

    1999-03-01

    Alloimmunization to the D blood group antigen following the transfusion of D-positive red blood cells to a D-negative recipient may be prevented in most persons by a prompt and adequate dose of Rho (D) immune globulin (RhIG). Until recently, the only RhIG approved by the US Food and Drug Administration (FDA) for this indication required intramuscular injection, an inconvenient and painful route for the relatively large volume that may be required. We describe the successful prevention of Rh alloimmunization following the unintentional transfusion of D-positive red blood cells to a D-negative infant by the intravenous infusion of WinRho SD, a new RhIG that is FDA-approved for prevention of post-transfusion Rh alloimmunization by intravenous administration. We believe that this more convenient and less painful approach should be the treatment of choice for preventing Rh alloimmunization following the transfusion of D-positive red cells to a D-negative recipient. PMID:10072121

  7. Extreme Elevation of Alkaline Phosphatase in a Pregnancy Complicated by Gestational Diabetes and Infant with Neonatal Alloimmune Thrombocytopenia.

    Science.gov (United States)

    Lozo, Svjetlana; Atabeygi, Amir; Healey, Michael

    2016-01-01

    There have been few case reports of isolated elevation of alkaline phosphatase beyond the normal physiologic amount with subsequent return to baseline after delivery. Here we present a similar case of extreme elevation of alkaline phosphatase in a pregnancy complicated by gestational diabetes and subsequently by neonatal alloimmune thrombocytopenia (NAIT). PMID:27610256

  8. Disease: H00225 [KEGG MEDICUS

    Lifescience Database Archive (English)

    Full Text Available ndrome Thrombotic thrombocytopenic purpura is caused by mutation in the ADAMTS13 gene and characterized by m...8) PMID:17666281 Tsai HM Thrombotic thrombocytopenic purpura: a thrombotic disorder cause

  9. Henoch-Schönlein Purpura in Northern Spain

    OpenAIRE

    Calvo-Río, Vanesa; Loricera, Javier; Mata, Cristina; Martín, Luis; Ortiz-Sanjuán, Francisco; Alvarez, Lino; González-Vela, M. Carmen; González-Lamuño, Domingo; Rueda-Gotor, Javier; Fernández-Llaca, Héctor; González-López, Marcos A.; Armesto, Susana; Peiró, Enriqueta; Arias, Manuel; Miguel A. González-Gay

    2014-01-01

    Abstract The severity of clinical features and the outcomes in previous series of patients reported with Henoch-Schönlein purpura (HSP) vary greatly, probably due to selection bias. To establish the actual clinical spectrum of HSP in all age groups using an unselected and wide series of patients diagnosed at a single center, we performed a retrospective review of 417 patients classified as having HSP according to the criteria proposed by Michel et al. Of 417 patients, 240 were male and 177 fe...

  10. Purpura fulminans in a patient with mixed connective tissue disease.

    LENUS (Irish Health Repository)

    Murad, Aizuri A

    2013-01-01

    A 43-year-old lady was admitted to the intensive care unit with sepsis. She had a history of mixed connective tissue disease, Raynaud\\'s syndrome and hypothyroidism. 2 days later, she developed a purpuric rash on her face and extremities with a livedoid background. Few days later, her distal fingers and toes became gangrenous which then had to be amputated. Laboratory investigations showed that she was coagulopathic and had multiple organ dysfunctions. Antiphospholipid antibodies were negative; however, protein C and antithrombin III levels were low. A skin biopsy showed fibrinoid necrosis in the vessel wall with microthrombi and red-cell extravasation. A diagnosis of purpura fulminans was made.

  11. 水痘合并急性血小板减少性紫癜1例%One Case of Chickenpox Complicating Acute Thrombocytopenic Purpura

    Institute of Scientific and Technical Information of China (English)

    杨巍; 朱莹; 李智伟

    2011-01-01

    @@ 1 病例介绍 患者男,7岁,学生.皮疹7 d,发热6 d,鼻衄2次,于2010-01-17入院.患者入院前7 d无诱因出现斑丘疹及水疱疹,次日皮疹增多,伴发热,体温最高38.2℃,于盛京医院感染科诊断为:水痘,化验血常规白细胞,血小板正常;口服蒲地兰抗病毒,甲紫外用等治疗;体温逐渐降至正常;入院前2d患者鼻衄1次,约10 ml,局部压迫后止血;

  12. HBV感染并发ITP病儿临床特征分析%A CLINICAL ANALYSIS BETWEEN HEPATITIS B VIRUS INFECTION AND IDIOPATHIC THROMBOCYTOPENIC PURPURA IN CHILDREN

    Institute of Scientific and Technical Information of China (English)

    宋爱琴; 董增义; 庞秀英; 卢愿; 李学荣; 孙立荣

    2006-01-01

    目的探讨乙型肝炎病毒(HBV)感染并发原发性血小板减少性紫癜(ITP)病儿临床特征、治疗反应和病程特点.方法将102例ITP病儿,分为两组,其中HBV-ITP组25例,非HBV-ITP组77例,病儿均采用大剂量丙种球蛋白、常规治疗量泼尼松、小剂量长春新碱等治疗,比较两组病儿的临床特征、治疗反应、病程特点等.结果 HBV-ITP组与非HBV-ITP组治疗前外周血血红蛋白水平、血小板数量及骨髓检查方面差异无显著意义(t=0.933、1.916,χ2=5.422, P>0.05);非HBV-ITP组外周血白细胞总数较HBV-ITP组高,差异有显著意义(t=5.050, P<0.01).采用大剂量丙种球蛋白、常规治疗量泼尼松、小剂量长春新碱治疗,HBV-ITP组疗效明显差于非HBV-ITP组(χ2=12.710、7.884, P<0.05,P=0.007).HBV-ITP病儿较非HBV-ITP病儿易于发展为慢性.结论 HBV-ITP病儿治疗反应差,病情易迁延、反复,容易向慢性转化.应进一步研究HBV感染对ITP发生发展的可能的作用机制.

  13. Analysis of 117 Patients with Diopathic Thrombocytopenic Purpura%117例特发性血小板减少性紫癜临床资料分析

    Institute of Scientific and Technical Information of China (English)

    王玲; 李华伟

    2011-01-01

    目的 初步探讨过去5年中泰安市特发性血小板减少性紫癜(ITP)发生的变化.方法回顾性分析了2004-2009年在泰安市中心医院住院的117例ITP患者的资料.结果①青壮年患者女性较男性患者多见(女:男=1.71:1);儿童患者男性多于女性(男:女=2.33:1)②重型(血小板小于20×109/L)ITP患者较轻型(血小板大于20×109/L)ITP患者多见(重型ITP:轻型ITP=3.03:1)③ITP患者以青壮年为主(64.9%),老年少见(9.4%)④儿童患者城市高于农村(P<0.05);青壮年患者农村高于城市(P<0.05).结论本研究进一步肯定了ITP发病情况的一些特征,同时也提出了一些新的特点

  14. Correlation of Notch1/Hes1 Genes Expression Levels in Egyptian Paediatric Patients with Newly Diagnosed and Persistent Primary Immune(Idiopathic) Thrombocytopenic Purpura.

    Science.gov (United States)

    Gawdat, Rania Mohsen; Hammam, Amira Ahmed; Ezzat, Dina Ahmed

    2016-09-01

    Notch signalling is involved in the development of several autoimmune diseases, one of such diseases is ITP. The aim of this study was to investigate and compare the expression levels of Notch1 receptor and its target Hes1 gene in Egyptian paediatric ITP patients. Real-time quantitative reverse transcriptase polymerase chain reaction was used to analyse the expression levels of Notch1 and Hes1 in 42 children with primary ITP (22 newly diagnosed and 20 persistent) cases. Twenty age and sex matched non-ITP controls were included. The expression levels of Notch1 were higher in newly diagnosed and persistent cases than controls with high statistical significant difference (P value ITP patients but Hes1 was markedly elevated than Notch1 in few cases. High expression levels of Notch1/Hes1 indicated the important role of Notch signalling in both newly diagnosed and persistent ITP. High expression levels of Hes1 than Notch1 may shed light on its value as a therapeutic target for future research in ITP. PMID:27429531

  15. Regulation of non-classical immune parameters in immune thrombocytopenic purpura mice by a spleen-invigorating, qi-replenishing and blood-containing formula

    Directory of Open Access Journals (Sweden)

    Tiantian Li

    2015-04-01

    Conclusions: The SQBF had a similar effect to prednisone with regards to enhancing peripheral blood platelet counts in ITP mice. Furthermore, it decreased β-EP levels and increased VIP and SIgA, and protected the thymus. This shows that, on base of the brain-gut axis functions, some non-classical immune vascular active factors or neurotransmitters are also involved in immune responses, and also have relationship with the onset of ITP and bleeding and/or hemostasis. It needs further study to determine whether a change in these active factors is related to immediate hemostasis.

  16. Simultaneous Manifestation of Chronic Myelomonocytic Leukemia and Multiple Myeloma during Treatment by Prednisolone and Eltrombopag for Immune-Mediated Thrombocytopenic Purpura.

    Science.gov (United States)

    Hagihara, Masao; Inoue, Morihiro; Kodama, Kenichiro; Uchida, Tomoyuki; Hua, Jian

    2016-01-01

    An 80-year-old man was admitted to our hospital because of severe thrombocytopenia. He was diagnosed with idiopathic thrombocytopenia, and prednisolone together with eltrombopag was started, leading to significant improvement of platelet counts. Four years later, there was a prominent increase of peripheral blood monocytes, which was accompanied by recurrence of thrombocytopenia. Bone marrow aspirates and serum electrophoresis revealed coexistence of chronic myelomonocytic leukemia (CMML) and multiple myeloma (MM). The patient received lenalidomide plus dexamethasone therapy but died due to exacerbation of the disorder. It was supposed that thrombocytopenia was secondarily caused by CMML and MM developed at a later period. PMID:27597907

  17. Do the acute platelet responses of patients with immune thrombocytopenic purpura (ITP) to IV anti-D and to IV gammaglobulin predict response to subsequent splenectomy?

    Science.gov (United States)

    Bussel, J B; Kaufmann, C P; Ware, R E; Woloski, B M

    2001-05-01

    The acute platelet response to Intravenous Gammaglobulin (IVIG) has been reported to predict response to subsequent splenectomy of patients with ITP. The current study was undertaken to determine if the platelet response to IV anti-D (Winrho-SDF) predicts response to subsequent splenectomy. The 61 HIV-uninfected children and adults in this study had taken part in the pre-licensing studies of IV anti-D and were all those who not only had evaluable platelet responses to IV anti-D but also had undergone splenectomy and had information available describing its 1-year outcome. Results of treatment with IVIG were available in 38 of these 61 patients. Neither response to the initial infusion of IV anti-D, nor response to the initial or last IVIG, predicted the response in either children or adults to subsequent splenectomy. However, response to the last anti-D infusion in adults was strongly correlated (P = 0.003) to response to subsequent splenectomy as was hemolysis >/=2.0 gm/dl after IV anti-D (P = 0.03). There was no overall relationship between response to IV anti-D or IVIG, and response to subsequent splenectomy. However, a good platelet response in adults to the last IV anti-D and a hemoglobin decrease >/=2.0 gm/dl both appeared to predict response to subsequent splenectomy. PMID:11279654

  18. RhIL-11 treatment normalized Th1/Th2 and T-bet/GATA-3 imbalance in in human immune thrombocytopenic purpura (ITP).

    Science.gov (United States)

    Lin, Ying; Zhou, Xieming; Guo, Wenjian; Li, Qianqian; Pan, Xiahui; Bao, Yunhua; He, Muqing; Zhu, Baoling; Lin, Xiaoji; Jin, Limin; Yao, Rongxin

    2016-09-01

    Immune thrombocytopenia (ITP) is an autoimmune hemorrhagic disorder characterized by reduction in platelet counts. T helper 1 (Th1) cells polarization with an increased shift of Th1/Th2 ratio has been reported in ITP. This shift is associated with transcription factor T-box expressed in T cells (T-bet) upregulation and GATA-binding protein 3 (GATA-3) downregulation, leading to an increased T-bet/GATA-3 ratio. Our previous in vitro study showed that recombinant human interleukin-11 (rhIL-11) could normalize Th1/Th2 imbalance in the peripheral blood mononuclear cells (PBMCs) isolated from adult ITP patients, which co-occurred with T-bet/GATA-3 ratio restoration. In this report, we investigated whether rhIL-11 had therapeutic effect in clinical ITP patients and whether rhIL-11 treatment could normalize Th1/Th2 and T-bet/GATA-3 levels in vivo. We found rhIL-11 treatment had a response rate of 67.7% and significantly decreased Th1 and T-bet levels but increased Th2 and GATA-3 levels in ITP patients who showed good response, normalizing Th1/Th2 and T-bet/GATA-3 ratios similar to that in healthy controls. Thus our study suggested rhIL-11 was effective with tolerable adverse effects in ITP. The treatment strategy warrants further clinical investigation. PMID:27235596

  19. Facial purpura in an elderly patient after upper gastrointestinal endoscopy

    Institute of Scientific and Technical Information of China (English)

    Ilhami Yüksel; (O)zlem Ekiz; Fuat Ekiz; (O)mer Basar; Oeman Yüksel

    2012-01-01

    To the editor:Upper gastrointestinal endoscopy (UGE) is a safe procedure and it is widely used without any complication in general.Complications are due to endoscopic procedure,invasive applications,local anesthetic medication or the sedative drugs like midazolam.Complications of the procedure are hemorrhage,peroration of the stomach or gut,respiratory arrest,reaction to the sedative drugs used like midazolam or pethidine,aspiration pneumonia,damaging of crowned teeth or dental bridgework,infection, cardiac arrest and finally under very unusual circmstances death.Mild complications are sore throat,numb throat,bloated feeling and feeling sleepy.1,2 Oral rash was reported to be a very rare complication (0.3%) of UGE in pediatric population.2 Some scholars reported a facial purpura after endoscopic procedure,previously3-5 Cutaneous complications after UGE may occur,however this condition is usually ignored by physicians.Herein,we present a case of facial purpura in an elderly patient as a mild complication of UGE.

  20. Recent progress in understanding the pathogenesis of fetal and neonatal alloimmune thrombocytopenia.

    Science.gov (United States)

    Curtis, Brian R

    2015-12-01

    Fetal and neonatal alloimmune thrombocytopenia (FNAIT) occurs in c. 1 in 1000 births and is caused by maternal antibodies against human platelet alloantigens that bind incompatible fetal platelets and promote their clearance from the circulation. Affected infants can experience bleeding, bruising and, in severe cases, intracranial haemorrhage and even death. As maternal screening is not routinely performed, and first pregnancies can be affected, most cases are diagnosed at delivery of a first affected pregnancy. Unlike its erythrocyte counterpart, Haemolytic Disease of the Fetus and Newborn, there is no prophylactic treatment for FNAIT. This report will review recent advances made in understanding the pathogenesis of FNAIT: the platelet alloantigens involved, maternal exposure and sensitization to fetal platelet antigens, properties of platelet Immunoglobulin G antibodies, maternal-fetal antibody transport mechanisms and efforts to develop an effective FNAIT prophylaxis.

  1. Mouse model of alloimmune-induced vascular rejection and transplant arteriosclerosis.

    Science.gov (United States)

    Enns, Winnie; von Rossum, Anna; Choy, Jonathan

    2015-05-17

    Vascular rejection that leads to transplant arteriosclerosis (TA) is the leading representation of chronic heart transplant failure. In TA, the immune system of the recipient causes damage of the arterial wall and dysfunction of endothelial cells and smooth muscle cells. This triggers a pathological repair response that is characterized by intimal thickening and luminal occlusion. Understanding the mechanisms by which the immune system causes vasculature rejection and TA may inform the development of novel ways to manage graft failure. Here, we describe a mouse aortic interposition model that can be used to study the pathogenic mechanisms of vascular rejection and TA. The model involves grafting of an aortic segment from a donor animal into an allogeneic recipient. Rejection of the artery segment involves alloimmune reactions and results in arterial changes that resemble vascular rejection. The basic technical approach we describe can be used with different mouse strains and targeted interventions to answer specific questions related to vascular rejection and TA.

  2. /sup 111/In-oxine platelet survivals in thrombocytopenic infants

    Energy Technology Data Exchange (ETDEWEB)

    Castle, V.; Coates, G.; Kelton, J.G.; Andrew, M.

    1987-09-01

    Thrombocytopenia is a common occurrence (20%) in sick neonates, but the causes have not been well studied. In this report we demonstrate that thrombocytopenia in the neonate is characterized by increased platelet destruction as shown by shortened homologous /sup 111/In-oxine-labeled platelet life spans. Thirty-one prospectively studied thrombocytopenic neonates were investigated by measuring the /sup 111/In-labeled platelet life span, platelet-associated IgG (PAIgG), and coagulation screening tests. In every infant, the thrombocytopenia was shown to have a destructive component since the mean platelet life span was significantly shortened to 65 +/- 6 (mean +/- SEM) hours with a range of one to 128 hours compared with adult values (212 +/- 8; range, 140 to 260; gamma function analysis). The platelet survival was directly related to the lowest platelet count and inversely related to both the highest mean platelet volume and duration of the thrombocytopenia. In 22 infants the percent recovery of the radiolabeled platelets was less than 50%, which suggested that increased sequestration also contributed to the thrombocytopenia. Infants with laboratory evidence of disseminated intravascular coagulation (n = 8) or immune platelet destruction evidenced by elevated levels of PAIgG (n = 13) had even shorter platelet survivals and a more severe thrombocytopenia compared with the ten infants in whom an underlying cause for the thrombocytopenia was not apparent. Full-body scintigraphic images obtained in 11 infants showed an increased uptake in the spleen and liver, with a spleen-to-liver ratio of 3:1. This study indicates that thrombocytopenia in sick neonates is primarily destructive, with a subgroup having evidence of increased platelet sequestration.

  3. Platelet kinetics and scintigraphic imaging in thrombocytopenic malaria patients.

    Science.gov (United States)

    Karanikas, Georgios; Zedwitz-Liebenstein, Konstantin; Eidherr, Harald; Schuetz, Matthias; Sauerman, Robert; Dudczak, Robert; Winkler, Stefan; Pabinger, Ingrid; Kletter, Kurt

    2004-03-01

    Thrombocytopenia is a common occurrence in acute malaria. It is attributed, among other factors, to excessive splenic platelet pooling and a shortened platelet lifespan. The aim of our study was to evaluate the platelet kinetics and sequestration site by isotopic studies in uncomplicated malaria-induced thrombocytopenia. Seven thrombocytopenic malaria patients (74,000+/-36,000 platelets/ micro l) were included in the study. Autologous (111)In-labeled platelet scintigraphy was performed up to 96 hours (h) post injection (p.i.) to evaluate the platelet sequestration site. Late sequestration for the spleen (S) and the liver (L) was analyzed according to the following activity ratios: S (spleen count on the last day of the platelet lifespan / spleen count at 30 min) and L (liver count on the last day of the platelet lifespan / liver count at 30 min). Additionally, platelet survival studies were performed. A normal late sequestration (S: 0.95+/-0.06 and L: 1.04+/-0.08; normal values, S and L: 1+/-0.2.) was observed in all of our patients. The platelet lifespan was reduced (1 to 4 days; normal range, 7-9 days), recovery was normal (mean, 63+/-6%; normal range, 55-75%), and the turnover rate was enhanced (mean, 95,000+/-80,000/ micro l/day; normal value, 35,000+/-4,500/ micro l/ day). According to the results of scintigraphy, the sequestration site by uncomplicated malaria-induced thrombocytopenia appears to be non-splenic and/or hepatic, yet diffuse. PMID:14983232

  4. Standard UV-B irradiation of platelets concentrates to prevent from PT-GVHD for alloimmunization

    Energy Technology Data Exchange (ETDEWEB)

    Takahashi, Takayoshi [Toranomon Hospital, Tokyo (Japan); Nagahashi, Hisakata; Takenouchi, Kogi; Tayama, Tatsuya; Tadokoro, Kenji; Juji, Takeo

    1994-11-01

    We tried to make an appropriate standard condition for UV-B irradiation of platelets concentrates (PC), which is useful for prophylaxis against post-transfusion graft versus host disease (PT-GVHD) as well as prevention against alloimmunization. Agitation of PC bags during UV-B irradiation is necessary to irradiate evenly cells in the bag, because a lot of UV-B ray should be absorbed by bag membrane and plasma. Amounts of UV-B that each lymphocyte or platelet would actually receive on an average (UVavg) was calculated by the equation as below. UV{sup *}avg=K{sub 1} x (K{sub 2}{sup L} - 1) x UV/(log{sub e}K{sub 2} x L), K{sub 1} and K{sub 2} are permeability index of bag membrane and that in plasma, respectively; while L and UV stands for depth of PC bag and emitting dose of UV-B, respectively. We irradiated PC bags with UV-B in a dose of 541-13,525 J/m{sup 2} of UV{sup *}avg, and examined lymphocytes in the bags about the responder and stimulator activities in mixed lymphocytes culture (MLR), as well as platelet function in the bags. Irradiation more than 5,000 J/m{sup 2} of UV{sup *}avg is needed to suppress responder and stimulator activities, and platelet function is maintained up to 13,525 J/m{sup 2} of UV{sup *}avg. In conclusion, UV-irradiation in a range of 6,000-13,000 J/m{sup 2} of UV{sup *}avg is considered appropriate to prevent from RT-GVHD or alloimmunization. (author).

  5. An overview of platelet indices and methods for evaluating platelet function in thrombocytopenic patients

    DEFF Research Database (Denmark)

    Vinholt, Pernille Just; Hvas, Anne-Mette; Nybo, Mads

    2014-01-01

    in thrombocytopenia. Flow cytometry, platelet aggregometry and platelet secretion tests are used to diagnose specific platelet function defects. The flow cytometric activation marker P-selectin and surface coverage by the Cone and Plate[let] analyser™ predict bleeding in selected thrombocytopenic populations...

  6. Spectrum of purpura fulminans: Report of three classical prototypes and review of management strategies

    Directory of Open Access Journals (Sweden)

    Ankur Talwar

    2012-01-01

    Full Text Available Purpura fulminans is a rare syndrome of intravascular thrombosis and hemorrhagic infarction of the skin that is rapidly progressive and is accompanied by vascular collapse and disseminated intravascular coagulation. It usually occurs in children, but this syndrome has also been noted in adults. The three forms of this disease are classified by the triggering mechanisms. We describe three classical cases of purpura fulminans of the three classical prototypes treated at our center and their varied clinical outcomes. We also describe a case of acute infectious purpura fulminans secondary to systemic leptospirosis which to our best knowledge is the first reported case in world literature. The various treatment options for purpura fulminans have also been reviewed.

  7. Thirty-five Infantile Purpura Nephritis Patients Treated with Integrated Traditional Chinese and Western Medicine

    Institute of Scientific and Technical Information of China (English)

    ZHENG; Tian-wen

    2001-01-01

    Thirty-five patients of infantile purpura nephritis (IPN) were treated with integrated traditional Chinese and western medicine (TCM-WM) from January 1994 to December 1998, with good efficacy obtained, and following is the report.

  8. Management of Pregnancy-Associated Thrombotic Thrombocytopenia Purpura

    Directory of Open Access Journals (Sweden)

    Ashley Fyfe-Brown

    2013-05-01

    Full Text Available Thrombotic thrombocytopenia purpura (TTP is an infrequent but serious disease. Pregnancy is a known risk factor for presentation or relapse of TTP. Difficulties in differentiating TTP from preeclampsia/HELLP (hemolysis, elevated liver enzymes and low platelets syndrome, and current treatment recommendations are discussed in this case report. A woman with previously treated and stable TTP had a relapse at 36 weeks' gestation. Careful surveillance led to an early diagnosis. Severe disease in the peripartum period was treated successfully with cryosupernatant plasma-based plasmapheresis and platelet transfusion, with good maternal and neonatal outcomes. Cryosupernatant plasma is a viable alternative to fresh frozen plasma for plasmapheresis for TTP and may offer some therapeutic and logistical advantages. Platelet transfusion can be undertaken safely if needed to prevent or treat significant hemorrhage.

  9. Henoch-Schonlein purpura: ultrasonography of scrotal and penile involvement

    Directory of Open Access Journals (Sweden)

    Youngsik Lim

    2015-04-01

    Full Text Available Testicular or scrotal involvement has been reported in children with Henoch-Schonlein purpura (HSP, but there are very few reports on penile involvement. We report the initial and follow-up ultrasonographic findings of scrotal and penile involvement of HSP in a 5-year-old boy. On ultrasonography, scrotal soft tissue thickening and epididymal swelling with increased vascularity were noted, and on the penis, a focal mass-like lesion appeared on the dorsal surface of the distal penis, having a hypoechoic mass-like appearance without visible vascular flow on a Doppler study. After 2 days of treatment, follow-up ultrasonography showed normal scrotum and penis with a resolved soft tissue mass-like lesion. Therefore, we think that HSP ultrasonographic findings involving the scrotum and penis might help to diagnose scrotal and penile involvement in a case of HSP and to avoid unnecessary medication and/or surgical procedures.

  10. Relapses in patients with Henoch–Schönlein purpura

    Science.gov (United States)

    Calvo-Río, Vanesa; Hernández, José Luis; Ortiz-Sanjuán, Francisco; Loricera, Javier; Palmou-Fontana, Natalia; González-Vela, Maria C.; González-Lamuño, Domingo; González-López, Marcos A.; Armesto, Susana; Blanco, Ricardo; González-Gay, Miguel A.

    2016-01-01

    Abstract To further investigate into the relapses of Henoch–Schönlein purpura (HSP), we analyzed the frequency, clinical features, and predictors of relapses in series of 417 unselected patients from a single center. After a median follow-up of 12 (interquartile range [IQR]: 2–38) years, almost one-third of the 417 patients (n = 133; 32%; 85 men/48 women) had experienced at least 1 relapse. At the time of disease diagnosis, patients who later experienced relapses had less commonly infections than those who never suffered flares (30.8% vs 41.9%; P = 0.03). In contrast, patients who experienced relapses had a longer duration of the first episode of palpable purpura than those without relapses (palpable purpura lasting >7 days; 80.0% vs 68.1%; P = 0.04). Abdominal pain (72.3% vs 62.3%; P = 0.03) and joint manifestations (27.8% vs 15.5%; P = 0.005) were also more common in patients who later developed relapses. In contrast, patients who never suffered relapses had a slightly higher frequency of fever at the time of disease diagnosis (9.3% vs 3.8%; P = 0.06). At the time of disease diagnosis, corticosteroids were more frequently given to patients who later had relapses of the disease (44% vs 32% in nonrelapsing patients; P = 0.03). Relapses generally occurred soon after the first episode of vasculitis. The median time from the diagnosis of HSP to the first relapse was 1 (IQR: 1–2) month. The median number of relapses was 1 (IQR 1–3). The main clinical features at the time of the relapse were cutaneous (88.7%), gastrointestinal (27.1%), renal (24.8%), and joint (16.5%) manifestations. After a mean ± standard deviation follow-up of 18.9 ± 9.8 years, complete recovery was observed in 110 (82.7%) of the 133 patients who had relapses. Renal sequelae (persistent renal involvement) was found in 11 (8.3%) of the patients with relapses. The best predictive factors for relapse were joint and gastrointestinal manifestations at HSP diagnosis (odds ratio [OR]: 2

  11. An unusual case of cryoglobulinemic purpura in elderly patient

    Directory of Open Access Journals (Sweden)

    Mauro Turrin

    2015-03-01

    Full Text Available Cryoglobulinemia is associated with infections, in particular chronic hepatitis C, autoimmune diseases, and lymphoproliferative disorders. Its frequency seems to be higher than is commonly considered, even in elderly patients.We describe the case of an elderly patient with diabetes, nephroangiosclerosis and purpura who presented marked and persistent increase in rheumatoid factor, hypocomplementemia and cryoglobulinemia with nephrotic disagreement unrelated to HCV. A thirteen‑month follow‑up showed neither immunorheumatologic nor neoplastic disorders. In literature, associations with hepatic pseudocyst is not described and a lack of association with HCV is very rare. Therefore, the hypothesis of “essential” mixed cryoglobulinemia (EMC associated with nephrotic syndrome was formulated. Renal disease associated with EMC (unrelated to HCV is characterized by the high prevalence of primary Sjögren syndrome and overt B‑cell non‑Hodgkin’s lymphoma for which repetitive clinical evaluation is necessary.http://dx.doi.org/10.7175/cmi.v9i1.1165

  12. Identification of Differentially Expressed Serum Proteins in Infectious Purpura Fulminans

    Directory of Open Access Journals (Sweden)

    Ting He

    2014-01-01

    Full Text Available Purpura fulminans (PF is a life-threatening hemorrhagic condition. Because of the rarity and randomness of the disease, no improvement in treatment has been made for a long time. In this study, we assessed the serum proteome response to PF by comparing serum proteins between healthy controls and PF patient. Liquid chromatography with tandem mass spectrometry (LC-MS/MS approach was used after depleting 6 abundant proteins of serum. In total, 262 proteins were confidently identified with 2 unique peptides, and 38 proteins were identified significantly up- (≥2 or downregulated (≤0.5 based on spectral counting ratios (SpCPF/N. In the 38 proteins with significant abundance changes, 11 proteins were previously known to be associated with burn or sepsis response, but 27 potentially novel proteins may be specifically associated with PF process. Two differentially expressed proteins, alpha-1-antitrypsin (SERPINA1 and alpha-2 antiplasmin (SERPINF2, were validated by Western blot. This is the first study where PF patient and healthy controls are compared in a proteomic study to elucidate proteins involved in the response to PF. This study provides an initial basis for future studies of PF, and the differentially expressed proteins might provide new therapeutic targets to decrease the mortality of PF.

  13. Clinical and blood bank factors in the management of platelet refractoriness and alloimmunization.

    Science.gov (United States)

    Friedberg, R C; Donnelly, S F; Boyd, J C; Gray, L S; Mintz, P D

    1993-06-15

    Numerous independent and interdependent factors are involved in the posttransfusion platelet response. Factors such as ABO match and platelet age are related to circumstances potentially under the control of the blood bank physician and therefore may permit circumvention by an active transfusion service. On the other hand, factors such as fever or sepsis may be unavoidable, being related more to the individual patient or clinical condition. To evaluate which factors could be circumvented, we prospectively followed the 1-hour corrected count increments (CCIs) for 962 single-donor apheresis platelet transfusions to 71 refractory hematologic oncology inpatients, with concomitant recording of implicated factors. Stepwise regression analysis allowed for determination of which concurrent and confounding clinical-, patient-, and blood bank-related factors significantly affected the CCIs. Although many implicated factors proved to be independently associated with an increased or decreased CCI, we found that no single variable consistently explained the CCI variation across the patient population. Each patient appeared sensitive to one or a few particular factors, but because of marked intraindividual variation, it was not possible to identify a priori which factors were important for a given patient. The single exception was a solid-phase red blood cell adherence assay used to cross-match platelets, but only for alloimmunized patients. We also evaluated the utility of requesting HLA-matched platelets from the local suppliers and maintained a clear distinction between platelets simply ordered as HLA matched and actually HLA-identical platelets. Accounting for the confounding clinical-, patient-, and blood bank-related factors, the cross-match assay was a better predictor of an adequate CCI than ordering platelets as HLA matched.

  14. Gastrointestinal manifestations of Henoch-Schonlein purpura: A report of two cases

    Institute of Scientific and Technical Information of China (English)

    Parameswaran; Prathiba; Rajalakshmi; Kalyanasundaram; Srinivasan

    2015-01-01

    Henoch-Schonlein purpura(HSP) is a small vessel vasculitis mediated by type Ⅲ hypersensitivity with deposition of Ig A immune complex in the walls of vessels. It is a multi-system disorder characterizedby palpable purpura, arthritis, glomerulonephritis and gastrointestinal manifestations and commonly occurs in children and young adults. The patients with gastrointestinal involvement usually present with colicky abdominal pain, vomiting and melena. The imaging findings include multifocal bowel thickening with mucosal hyperenhancement, presence of skip areas, mesenteric vascular engorgement, with involvement of unusual sites like stomach, duodenum and rectum. These imaging findings in a child or young adult with appropriate clinical findings could suggest HSP.

  15. Provision of HPA-1a (PlA1)-negative platelets for neonatal alloimmune thrombocytopenia: screening, testing, and transfusion protocol.

    Science.gov (United States)

    Munizza, M; Nance, S; Keashen-Schnell, M A; Sherwood, W; Murphy, S

    1999-01-01

    HPA-1a-negative platelet products are not routinely available for newborns with alloimmune thrombocytopenia. In this article we describe a program established to identify normal pheresis donors who are HPA-1a-negative and to organize their future donations so that our regional blood center would always have an HPA-1a-negative platelet product available. The solid phase red cell adherence assay was used for initial screening of platelet pheresis products. HPA-1a-negative donors were confirmed with the platelet suspension immunofluorescence test using three anti-HPA-1a sera. Screening of 2600 plateletpheresis donor samples identified 40 HPA-1a-negative donors. Of these, 36 are active and are coded for recognition on the daily pheresis inventory sheet. Theoretically, assuming four donations per year and donors' cooperation with scheduling, these 36 donors would enable us to have at least one HPA-1a-negative product available every day. In addition, a decision tree for patient management using platelet serology and availability of HPA-1a-negative products was developed. The GTI-PAK trade mark 12 is the major technique used for serologic screening of mothers of patients thought to have neonatal alloimmune thrombocytopenia. By screening pheresis donors and developing a clinical decision tree, HPA-1a-negative products, a rare resource, can be fully utilized.

  16. The absolute recommendation of chamber Neubauer method for platelets counting instead of indirect methods in severe thrombocytopenic patients

    Directory of Open Access Journals (Sweden)

    Oliveira Raimundo Antônio Gomes

    2003-01-01

    Full Text Available Accurate and precise platelet counting is crucial for recommending platelets transfusion for thrombocytopenic patients, principally when platelet counts are bellow 30,000/µl. As most laboratories still use the indirect methods for confirming low automated platelet counts, this work compared two indirect methods used in practice (Fonio and Nosanchunk et al. with the International Committee for Standardization in Hematology recommended direct method (Brecher and Cronkite. The obtained data show that the indirect methods present low precision and accuracy, and that the direct method should always be employed in severe thrombocytopenic samples thanks to its high precision.

  17. Relation between cytokines and routine laboratory data in children with septic shock and purpura

    NARCIS (Netherlands)

    J.A. Hazelzet (Jan); E. Voort (Edwin); J. Lindemans (Jan); P.G.J. ter Heerdt (P. G J); H.J. Neijens (Herman)

    1994-01-01

    textabstractObjective To establish the relation between routine laboratory data (lactate, fibrinogen, CRP) and cytokines (TNF,IL-1 and-6) and to estimate their prognostic value in pediatric patients with severe infectious purpura on admission. Design Prospective study. Setting Pediatric intensive

  18. Schönlein-Henoch purpura during pregnancy with successful outcome for mother and newborn

    Directory of Open Access Journals (Sweden)

    Gschnait Friedrich

    2002-01-01

    Full Text Available Abstrac Background Schönlein-Henoch purpura is a systemic vasculitis that affects vessels of a small caliber and rarely reported in the literature. Case presentation We report on a 35-year-old woman who developed palpable purpura with necrotizing cutaneous lesions on the lower limbs at 27 weeks of gestation. She also complained of epigastric pain and arthralgias. Histologic examination of a skin biopsy showed leukocytoclastic vasculitis with intravascular fibrin thrombi. The direct immunofluorescence analysis evidenced vascular deposits of IgA and C3 in the upper and mid-dermis. These findings were consistent with Schönlein-Henoch purpura. There was no evidence of renal involvement or placental dysfunction. The patient was treated with low-dose oral corticosteroids and a healthy infant was delivered by cesarean section. Examination of the placenta and the navel string disclosed no signs of vasculitis or infarction. Conclusion Schönlein-Henoch purpura is rarely reported in pregnancy. Treatment with orally administred corticosteroids may lead to a beneficial outcome for mother and newborn.

  19. Presentations and outcome analysis of 205 adult patients with Henoch-Schnlein purpura nephritis

    Institute of Scientific and Technical Information of China (English)

    宣萍

    2014-01-01

    Objective To analyze the clinical,pathological data and outcomes of the adult patients with HenochSchnlein purpura nephritis(HSPN).Methods The data of 205 HSPN patients who were diagnosed in Kidney Disease Center of the First Affiliated Hospital of Zhejiang University betwee Jan 2004 and May 2013 were collected and analyzed in different groups.Results The average

  20. Banking of pluripotent adult stem cells as an unlimited source for red blood cell production: potential applications for alloimmunized patients and rare blood challenges.

    Science.gov (United States)

    Peyrard, Thierry; Bardiaux, Laurent; Krause, Claire; Kobari, Ladan; Lapillonne, Hélène; Andreu, Georges; Douay, Luc

    2011-07-01

    The transfusion of red blood cells (RBCs) is now considered a well-settled and essential therapy. However, some difficulties and constraints still occur, such as long-term blood product shortage, blood donor population aging, known and yet unknown transfusion-transmitted infectious agents, growing cost of the transfusion supply chain management, and the inescapable blood group polymorphism barrier. Red blood cells can be now cultured in vitro from human hematopoietic, human embryonic, or human-induced pluripotent stem cells (hiPSCs). The highly promising hiPSC technology represents a potentially unlimited source of RBCs and opens the door to the revolutionary development of a new generation of allogeneic transfusion products. Assuming that in vitro large-scale cultured RBC production efficiently operates in the near future, we draw here some futuristic but realistic scenarios regarding potential applications for alloimmunized patients and those with a rare blood group. We retrospectively studied a cohort of 16,486 consecutive alloimmunized patients (10-year period), showing 1 to 7 alloantibodies with 361 different antibody combinations. We showed that only 3 hiPSC clones would be sufficient to match more than 99% of the 16,486 patients in need of RBC transfusions. The study of the French National Registry of People with a Rare Blood Phenotype/Genotype (10-year period) shows that 15 hiPSC clones would cover 100% of the needs in patients of white ancestry. In addition, one single hiPSC clone would meet 73% of the needs in alloimmunized patients with sickle cell disease for whom rare cryopreserved RBC units were required. As a result, we consider that a very limited number of RBC clones would be able to not only provide for the need for most alloimmunized patients and those with a rare blood group but also efficiently allow for a policy for alloimmunization prevention in multiply transfused patients. PMID:21377319

  1. Alloimmunization screening after transfusion of red blood cells in a prospective study Pesquisa de aloimunização após transfusão de concentrados de hemácias em um estudo prospectivo

    OpenAIRE

    Vitor Mendonça Alves; Paulo Roberto Juliano Martins; Sheila Soares; Gislene Araújo; Luciana Cayres Schmidt; Sidneia Sanches de Menezes Costa; Dante Mário Langhi; Helio Moraes-Souza

    2012-01-01

    BACKGROUND: Several irregular red blood cell alloantibodies, produced by alloimmunization of antigens in transfusions or pregnancies, have clinical importance because they cause hemolysis in the fetus and newborn and in transfused patients. OBJECTIVE: a prospective analysis of patients treated by the surgical and clinical emergency services of Hospital de Clínicas of the Universidade Federal do Triângulo Mineiro (HC/UFTM), Brazil was performed to correlate alloimmunization to clinical and epi...

  2. Anti-Human Platelet Antigen-1a Immunoglobulin G Preparation Intended to Prevent Fetal and Neonatal Alloimmune Thrombocytopenia

    Science.gov (United States)

    Weng, Ying-Jan; Husebekk, Anne; Skogen, Björn; Kjaer, Mette; Lin, Liang-Tzung; Burnouf, Thierry

    2016-01-01

    Fetal and neonatal alloimmune thrombocytopenia (FNAIT) is a severe disease that is caused by maternal alloantibodies generated during pregnancy or at delivery as a result of incompatibility between maternal and fetal human platelet antigens (HPAs) inherited from the father. Antibody-mediated immune suppression using anti-HPA-1a immunoglobulins is thought to be able to prevent FNAIT caused by HPA-1a. A fractionation process to prepare anti-HPA-1a immunoglobulin (Ig) G (IgG) from human plasma was therefore developed. Anti-HPA-1a plasma was obtained from volunteer mothers who underwent alloimmunization against HPA-1a during a previous pregnancy. Plasma was cryoprecipitated and the supernatant treated with caprylic acid and solvent/detergent (S/D), purified by chromatography, nanofiltered, concentrated, and sterile-filtered. The anti-HPA-1a immunoglobulin fraction was characterized for purity and safety. PAK12 and quantitative monoclonal antibody immobilization of platelet antigen (MAIPA) assays were used to detect anti-HPA-1a IgG. Hepatitis C virus (HCV) removal during nanofiltration was assessed by spiking experiments, using cell culture-derived reporter HCV and luciferase analysis. The caprylic acid treatment precipitated non-Ig proteins yielding a 90% pure Ig supernatant. S-HyperCel chromatography of the S/D-treated supernatant followed by HyperCel STAR AX provided high IgG recovery (>80%) and purity (>99.5%), and efficient IgA and IgM removal. Concentrations of complement factors C3 and C4 were HPA-1a throughout the process. Clinical-grade HPA-1a IgG can be prepared using a process compliant with current quality requirements opening perspectives for the prevention of FNAIT. PMID:27627660

  3. Post-transfusion purpura treated with plasma exchange by haemonetics cell separator. A case report

    DEFF Research Database (Denmark)

    Laursen, B; Morling, N; Rosenkvist, J;

    1978-01-01

    A case of post-transfusion purpura in a 61-year-old, multiparous female with a platelet alloantibody (anti-Zwa) in her serum is reported. The patient was successfully treated with plasma exchange by means of a Haemonetics 30 cell separator and corticosteroids. Compared with other therapeutic...... measures, plasma exchange seems to shorten the duration of thrombocytopenia. Major surgery was possible in our patient within ten days of development of the syndrome....

  4. Acute infantile hemorrhagic edema mimicking henoch-schonlein purpura: a case report

    OpenAIRE

    Kars, Veysel; Yilmaz, Ahmet; Celepkolu, Tahsin; Aslanhan, Hamza; ASLAN, Necmi; Demir, Vasfiye

    2015-01-01

    Acute infantile hemorrhagic edema is an acute cutaneous leucocytoclastic vasculitis that can be seen in infancy and characterized by fever, palpable purpura, and edema. Although it presents with severe symptoms, the clinical course is benign and the disease resolves in a short time. In this report, we present a 17-month-old infant who was admitted with cutaneous purpuric rash and edema of the extremities and subsequently diagnosed as acute infantile hemorrhagic edema. 

  5. Oxford classification of IgA nephropathy is applicable to predict long-term outcomes of Henoch-Schönlein purpura nephritis.

    Directory of Open Access Journals (Sweden)

    Hamid Nasri

    2014-12-01

    Full Text Available Henoch-Schönlein purpura nephritis and IgA nephropathy are currently considered to be different clinical presentations of the same disease. There is need for a reliable proven, morphologic classification that can help clinicians more accurately formulate treatment strategies for patients with Henoch-Schönlein purpura nephritis. Considering that Henoch-Schönlein purpura nephritis and IgA nephropathy have common characteristics of pathogenesis and histopathologic findings, we postulate that, the Oxford classification could also help predict long-term outcomes in Henoch-Schönlein purpura nephritis. Hence, we suggest to applicate the Oxford classification for patients with Henoch-Schönlein purpura nephritis.

  6. Anti-Human Platelet Antigen-1a Immunoglobulin G Preparation Intended to Prevent Fetal and Neonatal Alloimmune Thrombocytopenia.

    Science.gov (United States)

    Weng, Ying-Jan; Husebekk, Anne; Skogen, Björn; Kjaer, Mette; Lin, Liang-Tzung; Burnouf, Thierry

    2016-01-01

    Fetal and neonatal alloimmune thrombocytopenia (FNAIT) is a severe disease that is caused by maternal alloantibodies generated during pregnancy or at delivery as a result of incompatibility between maternal and fetal human platelet antigens (HPAs) inherited from the father. Antibody-mediated immune suppression using anti-HPA-1a immunoglobulins is thought to be able to prevent FNAIT caused by HPA-1a. A fractionation process to prepare anti-HPA-1a immunoglobulin (Ig) G (IgG) from human plasma was therefore developed. Anti-HPA-1a plasma was obtained from volunteer mothers who underwent alloimmunization against HPA-1a during a previous pregnancy. Plasma was cryoprecipitated and the supernatant treated with caprylic acid and solvent/detergent (S/D), purified by chromatography, nanofiltered, concentrated, and sterile-filtered. The anti-HPA-1a immunoglobulin fraction was characterized for purity and safety. PAK12 and quantitative monoclonal antibody immobilization of platelet antigen (MAIPA) assays were used to detect anti-HPA-1a IgG. Hepatitis C virus (HCV) removal during nanofiltration was assessed by spiking experiments, using cell culture-derived reporter HCV and luciferase analysis. The caprylic acid treatment precipitated non-Ig proteins yielding a 90% pure Ig supernatant. S-HyperCel chromatography of the S/D-treated supernatant followed by HyperCel STAR AX provided high IgG recovery (>80%) and purity (>99.5%), and efficient IgA and IgM removal. Concentrations of complement factors C3 and C4 were < 0.5 and < 0.4 mg/dL, respectively. The final IgG could be nanofiltered on Planova 20N under conditions removing more than 3 log HCV infectivity to baseline mock infection level, and concentrated to ca. 30 g/L. Proteolytic activity and thrombin generation were low in the final fraction. The Pak12 and MAIPA assays showed good recovery of anti-HPA-1a throughout the process. Clinical-grade HPA-1a IgG can be prepared using a process compliant with current quality requirements

  7. Sirolimus for Autoimmune Disease of Blood Cells

    Science.gov (United States)

    2016-04-22

    Autoimmune Pancytopenia; Autoimmune Lymphoproliferative Syndrome (ALPS); Evans Syndrome; Idiopathic Thrombocytopenic Purpura; Anemia, Hemolytic, Autoimmune; Autoimmune Neutropenia; Lupus Erythematosus, Systemic; Inflammatory Bowel Disease; Rheumatoid Arthritis

  8. Large-scale DNA typing for human platelet alloantigens by PCR-PHFA (preferential homoduplex formation assay).

    Science.gov (United States)

    Fujiwara, K; Isa, K; Oka, T; Maekawajiri, S; Yamane, A; Akaza, T; Tadokoro, K; Juji, T; Shibata, Y; Tokunaga, K

    1996-10-01

    Alloimmunization against human platelet alloantigens (HPA) is known to be involved in disorders such as neonatal alloimmune thrombocytopenic purpura, posttransfusion purpura, and refractoriness to platelet transfusion therapy. HPA typing is essential in diagnosis and management of patients. Therefore a reliable and speedy method is necessary for HPA typing. We have successfully applied a new DNA typing method, PCR-preferential homoduplex formation assay (PHFA) method, to typing for the HPA-1, -2, -3, -4, -5 and -6 systems. This method is based on DNA strand competition during hybridization under a precisely controlled temperature gradient between a double-labelled amplicon (standard DNA), prepared from biotin- and DNP-labelled primers, and an unlabelled amplicon (sample DNA). The results obtained by PCR-PHFA typing were in good agreement with the allotypes determined by serological typing and by other DNA typing methods. The PCR-PHFA method can be easily automated, is suitable for typing both small and large numbers of samples, and thus is applicable to routine HPA typing.

  9. Role of Regulatory T Cells (Treg and the Treg Effector Molecule Fibrinogen-like Protein 2 in Alloimmunity and Autoimmunity

    Directory of Open Access Journals (Sweden)

    Andrzej Chruscinski

    2015-07-01

    Full Text Available CD4+CD25+Foxp3+ regulatory T cells (Treg are critical to the maintenance of immune tolerance. Treg are known to utilize a number of molecular pathways to control immune responses and maintain immune homeostasis. Fibrinogen-like protein 2 (FGL2 has been identified by a number of investigators as an important immunosuppressive effector of Treg, which exerts its immunoregulatory activity by binding to inhibitory FcγRIIB receptors expressed on antigen-presenting cells including dendritic cells, endothelial cells, and B cells. More recently, it has been suggested that FGL2 accounts for the immunosuppressive activity of a highly suppressive subset of Treg that express T cell immunoreceptor with Ig and ITIM domains (TIGIT. Here we discuss the important role of Treg and FGL2 in preventing alloimmune and autoimmune disease. The FGL2–FcγRIIB pathway is also known to be utilized by viruses and tumor cells to evade immune surveillance. Moving forward, therapies based on modulation of the FGL2–FcγRIIB pathway hold promise for the treatment of a wide variety of conditions ranging from autoimmunity to cancer.

  10. Henoch-Schönlein purpura without systemic involvement beginning with acute scrotum and mimicking torsion of testis

    Directory of Open Access Journals (Sweden)

    Levent Verim

    2013-04-01

    Full Text Available Henoch-Schönlein purpura (HSP is the most common systemic vasculitis in children. Typical presentations of HSP are palpable purpura of the small vessels in the hips and lower limbs, abdominal pain, arthritis, and hematuria. Scrotal involvement manifested by the presence of scrotal pain and swelling during the course of the disease is rarely seen. HSP without systemic involvement with acute scrotum mimicking testicular torsion is even rare in the medical literature. In most cases, patients with this disease achieve complete recovery. Herein, we report an interesting HSP case with skin symptoms but without systemic involvement and then progression to acute scrotum resembling torsion of testis.

  11. Effects of increased von Willebrand factor levels on primary hemostasis in thrombocytopenic patients with liver cirrhosis.

    Directory of Open Access Journals (Sweden)

    Andreas Wannhoff

    Full Text Available In patients with liver cirrhosis procoagulant and anticoagulant changes occur simultaneously. During primary hemostasis, platelets adhere to subendothelial structures, via von Willebrand factor (vWF. We aimed to investigate the influence of vWF on primary hemostasis in patients with liver cirrhosis. Therefore we assessed in-vitro bleeding time as marker of primary hemostasis in cirrhotic patients, measuring the Platelet Function Analyzer (PFA-100 closure times with collagen and epinephrine (Col-Epi, upper limit of normal ≤ 165 s or collagen and ADP (Col-ADP, upper limit of normal ≤ 118 s. If Col-Epi and Col-ADP were prolonged, the PFA-100 was considered to be pathological. Effects of vWF on primary hemostasis in thrombocytopenic patients were analyzed and plasma vWF levels were modified by adding recombinant vWF or anti-vWF antibody. Of the 72 included cirrhotic patients, 32 (44.4% showed a pathological result for the PFA-100. They had mean closure times (± SD of 180 ± 62 s with Col-Epi and 160 ± 70 s with Col-ADP. Multivariate analysis revealed that hematocrit (P = 0.027 and vWF-antigen levels (P = 0.010 are the predictors of a pathological PFA-100 test in cirrhotic patients. In 21.4% of cirrhotic patients with platelet count ≥ 150/nL and hematocrit ≥ 27.0%, pathological PFA-100 results were found. In thrombocytopenic (< 150/nL patients with cirrhosis, normal PFA-100 results were associated with higher vWF-antigen levels (462.3 ± 235.9% vs. 338.7 ± 151.6%, P = 0.021. These results were confirmed by multivariate analysis in these patients as well as by adding recombinant vWF or polyclonal anti-vWF antibody that significantly shortened or prolonged closure times, respectively. In conclusion, primary hemostasis is impaired in cirrhotic patients. The effect of reduced platelet count in cirrhotic patients can at least be partly compensated by increased vWF levels. Recombinant vWF could be an alternative to platelet transfusions in the

  12. Effect of Composite Salvia Injection on Platelet Parameters in Children with Anaphylactoid Purpura

    Institute of Scientific and Technical Information of China (English)

    谢雪兰; 寇素茹; 许月红; 李朝英

    2009-01-01

    Objective:To explore the effect of composite salvia injection(CSI) on platelet parameters in children with anaphylactoid purpura(AP) and its clinical significance.Methods:One hundred and fifty children with AP were assigned to two groups,80 in Group A and 70 in Group B.They were treated,respectively,with conventional therapy only or conventional therapy combined with CSI.Their platelet parameters,including blood platelet count(BPC),mean platelet volume(MPV),platelet distribution width(PDW) and plateletcr...

  13. Bir henoch-schönlein purpurası komplikasyonu : stoma

    OpenAIRE

    Yıldız, Tülin

    2011-01-01

    Henoch Schönlein Purpurası (HSP) deri, böbrek, eklem ve gastrointestinal sistemi etkileyen ve immun kompleks aracılıgı ile olusan bir lökositoklastik veya hipersensitivite vaskülitidir. Esas olarak çocukluk çagı hastalıgıdır. Prognoz genellikle iyidir; ancak siddetli gastrointestinal ve renal bulguları olan hastaların dikkatle tedavi edilmesi ve izlenmesi gerekir. Bazı durumlar ise, hastalık sonucu stoma açılmasını gerektirebilir. Olgumuz, HSP tanısı ile servise kabul edildikten k...

  14. Koebner's Phenomenon in Childhood Henoch-Schönlein Purpura: A Report of Two Cases.

    Science.gov (United States)

    Sharma, Shreya; Chandrasekaran, Venkatesh; Krishnamurthy, Sriram; Mekala, Suresh; Mahadevan, Subramanian

    2016-07-01

    Koebner's phenomenon occurs rarely in connection with Henoch-Schönlein purpura (HSP). We report two children with HSP who developed Koebner's phenomenon on the second day after the onset of rash. The first was an 11-year-old girl with rheumatic heart disease who presented with abdominal pain for 1 month and subsequently developed rash and nephritis. The second patient was a 7-year-old girl who presented with rash and polyarthritis. To the best of our knowledge, Koebner's phenomenon in childhood HSP has not been reported. PMID:27282588

  15. Rapid improvement of Henoch-Schonlein purpura associated with the treatment of Helicobacter pylori infection

    Directory of Open Access Journals (Sweden)

    Turgay Ulas

    2012-01-01

    Full Text Available Helicobacter pylori (H. pylori are one of the most common bacterial infections, seen in humans, worldwide and their possible relationships to different diseases are a focus of attention nowadays. H. pylori may cause some extra intestinal manifestations some of which are dermatological conditions, including Henoch-Schönlein purpura (HSP, chronic urticaria and atopic dermatitis. We describe a 49-year-old man who presented with HSP triggered by gastric H. pylori infection. Treatment of H. Pylori infection was accompanied by prompt resolution of the gastrointestinal manifestations and purpuric rashes. These findings suggest a causative role for H. pylori in the occurrence of HSP.

  16. Mesenteric vasculitis in adults with Henoch-Schonlein purpura: a not-so-benign condition.

    LENUS (Irish Health Repository)

    Sibartie, V

    2009-02-07

    INTRODUCTION: The gastrointestinal manifestations of Henoch-Schonlein purpura (HSP) are well characterised, but their recognition can be difficult when they occur in isolation. Furthermore, HSP can run a more serious course in adults, compared to children, in whom the disease usually occurs. MATERIALS AND METHODS: We describe two cases that illustrate the challenges of HSP with mesenteric vasculitis and the outcome in adults. CONCLUSION: Although self-limiting in most patients, the outcome of HSP in adults can be far from benign and even fatal.

  17. Fatores de risco para aloimunização em pacientes com anemia falciforme Risk factors for alloimmunization in patients with sickle cell anemia

    Directory of Open Access Journals (Sweden)

    Patrícía Costa Alves Pinto

    2011-12-01

    Full Text Available OBJETIVO: Determinar a imunofenotipagem eritrocitária em doadores de sangue e em pacientes com anemia falciforme (SS atendidos no Hemocentro de Alagoas e descrever a frequência e os fatores associados à aloimunização eritrocitária. MÉTODOS: Estudo transversal com 102 pacientes SS e 100 doadores de sangue. Realizou-se a fenotipagem eritrocitária, teste de Coombs Direto e Indireto e detecção de anticorpos irregulares por painel de hemácias fenotipadas. Os dados foram comparados por meio do teste de Mann-Whitney, qui-quadrado ou teste exato de Fisher. Para análise dos fatores associados à aloimunização utilizou-se a regressão logística univariada e múltipla. RESULTADOS: Os antígenos mais frequentes entre os pacientes e os doadores foram c, e, M, s, JK(a. Observaram-se diferenças significativas entre as frequências dos fenótipos dos pacientes e dos doadores em relação aos antígenos s, FY(a e JK(b. Dos 79 pacientes transfundidos, 10 (12,7% apresentaram Coombs Indireto positivo. Detectaram-se 13 aloanticorpos, sete do sistema Rh, dois do Kell e quatro não identificados. Os fatores associados à aloimunização foram o intervalo de tempo entre a última transfusão e a data do teste e ter recebido mais de dez transfusões de hemácias. Receber mais de dez transfusões representou uma chance 16,39 (IC 95%: 2,23-120,59 vezes maior de ser aloimunizado, em comparação aos que receberam menos que dez. CONCLUSÃO: A prevalência de aloimunização nos pacientes SS foi 12,7%, sendo 70% dos anticorpos encontrados pertencentes a grupos sanguíneos Rh e Kell. Este estudo mostra a importância da fenotipagem eritrocitária em doadores e receptores para diminuir o risco de aloimunização.OBJECTIVE: To determine erythrocyte phenotyping in blood donors and patients with sickle cell anemia (SS treated at Hemocentro of Alagoas and describe the frequency and factors associated with erythrocyte alloimmunization. METHODS: Cross-sectional study

  18. Red cell alloimmunization and infectious marker status (human immunodeficiency virus, hepatitis B virus and hepatitis C virus in multiply transfused thalassemia patients of North India

    Directory of Open Access Journals (Sweden)

    Raj Nath Makroo

    2013-01-01

    Full Text Available Background: Patients with thalassemia major are largely transfusion dependent and are thus exposed to a variety of risks such as transmission of infectious diseases, iron overload and alloimmunization. This study was performed to determine the prevalence of human immune deficiency virus (HIV, hepatitis B virus (HBV, hepatitis C virus (HCV and red cell antibodies among multiple-transfused thalassemic patients in and around the national capital region. Materials and Methods: The Department of Transfusion Medicine, Indraprastha Apollo Hospitals, conducted this study in collaboration with the National Thalassemia Welfare Society over a period of 1 year starting February2011. Blood samples from the patients were tested for blood group, red cell alloantibody/ies, anti-HIV, anti-HCV and hepatitis B surface antigen (HBsAg by ELISA and for the respective viral ribonucleic acid (RNA or deoxyribonucleic acid (DNA by nucleic acid testing (NAT. Results: A total of 462 thalassemics which consists of 290 males and 172 females were tested. The overall alloimmunization rate was 4.1% and anti-Kell was the most common antibody identified. Thirteen cases (2.8% were positive for HBsAg by ELISA, 107 (23.1% were reactive for anti HCV and 11 (2.38% for anti HIV antibodies. Further screening and discriminatory assays by NAT confirmed the presence of HBV DNA in 11 cases, HIV RNA in 7 cases and HCV RNA in 48 cases. Conclusion: In spite of advances in Immunohematology and infectious marker testing in recent years, the rates of alloimmunization and infectious marker positivity remains high among multiply transfused patients like thalassemics. Provision of safe and adequate blood supply to these patients is a key to improving their quality-of-life and longevity.

  19. HENOCH SCHONLEIN PURPURA ASSOCIATED WITH ACUTE POSTSTREPTOCOCCAL GLOMERULONEPHRITIS: A CASE REPORT

    Directory of Open Access Journals (Sweden)

    Deborah Melati

    2015-05-01

    Full Text Available Acute post-streptococcal glomerulonephritis (APSGN is one of the most common renal disease resulting from a prior infection with group A â-hemolytic streptococcus (GAS. Henoch Schonlein Purpura (HSP is a systemic disease with frequent renal involvement, its etiology is still unknown but several infections have been described as trigger includingGAS infection. A 4 year 10 month old Balinese boy presented with full blown acute nephritic syndrome, an elevation in serum creatinine and four fold increase of anti streptolysin-O, also low serum levels of complement C3 with normal C4 confirmed the diagnosis of APSGN. During hospitalization he developed palpable purpura, gastrointestinal symptoms as well as leucytoclastic vasculitis in skin biopsy conclude HSP diagnosis.He was treated with anti-hypertensions and metylprednisolone intravenous. The prognosis of the patient was excellent, he showed normal physical examination with normal complete blood count and urinalysis after 3 months follow up. We conclude that both APSGN and HSP could appear concurrently after GAS infection. [MEDICINA 2014;45:102-7]  

  20. Henoch-Schönlein without Purpura: A Case Report and Review Literature.

    Science.gov (United States)

    Jarasvaraparn, Chaowapong; Lertudomphonwanit, Chatmanee; Pirojsakul, Kwanchai; Worawichawong, Suchin; Angkathunyakul, Napat; Treepongkaruna, Suporn

    2016-04-01

    Henoch-Schönlein purpura (HSP) is a multi-organ vasculitis involving skin, joints, gastrointestinal tract, and kidneys. The present study reported a 5-year-old boy presenting with intense abdominal pain, bloody diarrhea, and protein-losing enteropathy. Investigations for infectious enteritis were negative. Esophagogastroduodenoscopy showed swelling and erythematous mucosa with hemorrhagic spots at duodenal bulb to the third part of duodenum. Histopathology of endoscopic biopsies revealed non-specific duodenitis. HSP was suspected, based on duodenitis and the presence of inflammatory markers without identifiable causes. Corticosteroid was started resulting in marked improvement of his clinical symptoms. Two weeks later, he developed nephrotic-range proteinuria, thus kidney biopsy was performed. Renal histology was consistent with IgA nephropathy, supporting the diagnosis of HSP This report emphasizes that patients with HSP may not always show visible purpura, and the diagnosis requires a high index of suspicion. GI endoscopy and renal biopsy may be helpful for the diagnosis in selected patients presenting with atypical presentations. PMID:27396230

  1. Regulation of T cell differentiation and alloimmunity by the cyclin-dependent kinase inhibitor p18ink4c.

    Directory of Open Access Journals (Sweden)

    Emily A Rowell

    Full Text Available Cellular proliferation in response to mitogenic stimuli is negatively regulated by the Cip/Kip and the Ink4 families of cyclin-dependent kinase (CDK inhibitors. Several of these proteins are elevated in anergic T cells, suggesting a potential role in the induction or maintenance of tolerance. Our previous studies showed that p27kip1 is required for the induction of T cell anergy and transplantation tolerance by costimulatory blockade, but a role for Ink4 proteins in these processes has not been established. Here we show that CD4+ T cells from mice genetically deficient for p18ink4c divide more rapidly than wild-type cells in response to antigenic, costimulatory and growth factor signals. However, this gain of proliferative function was accompanied by a moderate increase in the rate of cell death, and was accompanied by an overall defect in the generation of alloreactive IFNγ-producing effector cells. Consistent with this, p18ink4c-deficient T cells were unable to induce graft-vs-host disease in vivo, and p18ink4c deficiency cooperated with costimulatory blockade to significantly increase the survival of fully mismatched allografts in a cardiac transplantation model. While both p18ink4c and p27kip1 act to restrict T cell proliferation, p18ink4c exerts an opposite effect from p27kip1 on alloimmunity and organ transplant rejection, most likely by sustaining T cell survival and the development of effector function. Our studies point to additional important links between the cell cycle machinery and the processes of T cell differentiation, survival and tolerance.

  2. Treatment of childhood idiopathic thrombocytopenic purpura with high-dose immunoglobulin%大剂量免疫球蛋白治疗儿童特发性血小板减少性紫癜

    Institute of Scientific and Technical Information of China (English)

    郭慧彬; 石苇; 谢晓恬

    2006-01-01

    目的:观察静脉输注大量免疫球蛋白(HDIG)治疗儿童特发性血小板减少性紫癜(TIP)的疗效.方法:联合应用HDIG和皮质激素治疗儿童ITP,观察疗效,并采用统计学方法与对照组作疗效对比.结果:治疗观察组外周血小板上升明显较疗效对照组快,血小板上升达峰值时间也短于对照组(p均<0.05);两组显效率分别为93.8%和63.6%,经统计分析疗效有显著差异(X2=4.18,p<0.05).结论 HDIG联合皮质激素为治疗儿童ITP的有效疗法,具有外周血小板恢复时间快,显效率高,不易复发和较高的安全性等优点,值得临床推广.

  3. The bone marrow megakaryocyte change of acute and chronic idiopathic thrombocytopenic purpura%急慢性特发性血小板减少性紫癜骨髓巨核细胞改变

    Institute of Scientific and Technical Information of China (English)

    周建中

    2007-01-01

    目的 比较急慢性特发性血小板减少性紫癜(ITP)骨髓巨核细胞改变特点.方法 包括30例急性ITP及38例慢性ITP初诊患者,其骨髓片经瑞氏染色,显微镜下计数巨核细胞.结果 急慢性ITP的原巨核细胞、幼巨核细胞、颗粒型巨核细胞及裸核巨核细胞数量增多,尤其是急性ITP.急慢性ITP的产生,血小板型巨核细胞均减少,急性ITP更为显著.慢性ITP易见血小板型幼巨核细胞.结论 急慢性ITP巨核细胞增多,且显著成熟障碍,尤其是急性ITP.

  4. Changes of T cell subgroups and κ,λ light chain of blood in children with idiopathic thrombocytopenic purpura%ITP患儿血T细胞亚群和轻链的改变

    Institute of Scientific and Technical Information of China (English)

    胡斌飞; 李白恩; 吴杏丹

    2001-01-01

    @@ 特发性血小板减少性紫癜(ITP)是一种自身免疫性疾病.为了进一步探讨ITP与免疫功能变化的关系,我们检测了40例ITP患儿的外周血T细胞亚群和血免疫球蛋白轻链在病程中的变化,现报告如下.

  5. Clinical Significance of Platelet Indices in Immunologic Thrombocytopenic Purpura%免疫性血小板减少性紫癜血小板参数的临床意义

    Institute of Scientific and Technical Information of China (English)

    黄月琴

    2010-01-01

    目的:探讨血小板参数对免疫性血小板减少性紫癜(ITP)的临床意义.方法:对ITP组、非ITP组、健康对照组采用血细胞分析仪检测血小板相关参数,即血小板计数(PLT)、血小板平均体积(MPV)、血小板压积(PCT)、血小板平均分布宽度(PDW)及ITP治疗后血小板参数的变化.结果:与非ITP组及健康对照组相比,ITP组2(PLT>10×109/L)MPV、PDW明显增加(P<0.01), ITP组1(PLT<10×109/L)MPV、PDW明显减低(P<0.01),在ITP的治疗过程中,MPV比PLT更早出现明显升高.结论:血小板参数对血小板减少症有鉴别意义,动态观察血小板参数对疗效观察有重要的临床意义.

  6. Relationship between human parovirous B19 infection and idiopathic thrombocytopenic purpura%特发性血小板减少性紫癜与人细小病毒B19的关系

    Institute of Scientific and Technical Information of China (English)

    严智先; 蔡定邦; 陈享

    2003-01-01

    目的探讨特发性血小板减少性紫癜(ITP)与人细小病毒B19感染性的关系.方法采用巢式聚合酶链反应技术分别对63例ITP患儿的血清、38例ITP患儿的骨髓液检测人细小病毒B19-DNA.并与对照组进行对比.结果①63例ITP静脉血中人细小病毒B19-DNA阳性12例,阳性率为19.1%,186例健康儿童11例阳性,阳性率为5.91%.血清的ITP组与对照组差异有显著意义(χ2=9.68,P<0.01).②38例ITP患儿的骨髓液中人细小病毒B19-DNA 16例阳性,阳性率为42.1%,对照组30例阳性3例,阳性率为10%,骨髓ITP组与对照组差异有显著意义(χ2=8.58,P<0.01).③血清的ITP组12例阳性病例中,11例是急性ITP,阳性率为23.4%;1例是慢性ITP,阳性率为5.9%,差异有显著意义(χ2=3.09,P<0.05).骨髓的ITP组急性29例,阳性15例,阳性率为51.7%;慢性9例,阳性1例,阳性率为11.1%.ITP组与对照组差异有显著意义(χ2=4.65,P<0.05).男女之间无差异.结论①ITP人群有较高的B19感染率,一部分ITP病儿与人细小病毒B19感染密切相关.②临床上一部分ITP患儿的发病可能为人细小病毒B19感染所导致.③B19感染相关性ITP以急性ITP为主.

  7. Progress in the treatment for glucocorticoid drug-fast idiopathic thrombocytopenic purpura%糖皮质激素耐药特发性血小板减少性紫癜治疗进展

    Institute of Scientific and Technical Information of China (English)

    侯明

    2008-01-01

    特发性血小板减少性紫癜(ITP)是一种自身免疫性疾病,糖皮质激素常作为ITP治疗首选药物,有效率70%~80%,仍有部分耐药ITP。下面将糖皮质激素耐药ITP治疗进展做一综述。

  8. Nursing Care for One Patient Suffering from Thrombotic Thrombocytopenic Purpura and Hemolytic Uremic Syndrome After Caesarean Birth%1例剖宫产术后并发TTP-HUS患者的护理

    Institute of Scientific and Technical Information of China (English)

    刘波; 张鹏; 刘丽云

    2009-01-01

    对1例剖宫产术后并发血栓性血小板减少性紫癜-溶血性尿毒综合征(TTP-HUS)患者予激素、血浆置换、抗感染、对症支持等治疗,患者好转出院.提出严密观察病情变化,加强心理、基础、饮食、药物不良反应、血浆置换的护理及健康教育,可促进患者康复.

  9. 小儿特发性血小板减少性紫癜外周血T、B淋巴细胞观察%The observation of T and B lymphocyte in peripheral of infantile idiopathic thrombocytopenic purpura

    Institute of Scientific and Technical Information of China (English)

    白文杰; 翟凤兰

    2000-01-01

    Objective: To detect the ratio changes of subgroup T lymphocyte and B lymphocyte in infants with ITP. Methods:Manufacturing single nucleus cell with lymphocyte, separate liquid, adding antibody after thermal cultivation and calculatingthe percentage of positive cells using inmuno-fluorescence method. Results: There are no statistie differences between the ITPpatients' T3、T4 and the healthy contral' s there are statistie differences between the ITP patients' T8、 B groups and the healthycontral' s. Conclusion: The patients of infantile ITP may have humoral and cellular immunity factional disorder.%目的:检测ITP患儿T淋巴细胞亚群及B细胞的比值变化。方法:采用荧光免疫法用淋巴细胞分离液配制单个细胞,温育后加入抗体,计算阳性百分率。结果:患儿组T3、T4与健康组差异不显著,T8及B细胞患儿组与健康组差异有显著性。结论:ITP患儿可出现细胞免疫及体液免疫功能紊乱。

  10. 选择素在脾切除治疗特发性血小板减少性紫癜中的意义%Sificance of selectin in treatment of idiopathic thrombocytopenic purpura with splenectomy

    Institute of Scientific and Technical Information of China (English)

    秦锡虎; 陈易人; 华铮; 黄伯华; 邱国强; 曹苇

    2001-01-01

    目的研究特发性血小板减少性紫癜(ITP)时脾脏血管内皮细胞的活化程度,了解脾切除后选择素(selectin)水平的变化,探讨脾切除治疗的ITP的指征.方法A组为12例激素疗效不佳,术前sE-selectin、sP-selectin/Plt水平明显高于正常的ITP病例;B组为8例肝炎后性肝硬化继发脾机能亢进病人.二组病人均行脾切除,术前、术后检测血小板(Plt)、选择素(P-selectin、L-selectin)、可溶性选择素(sE-selectin、sL-selectin、sP-selectin).结果A组术后获随访11例,10例反应极好,1例反应良好.术前sE-selectin水平A、B两组均高于正常,术后以近似幅度逐步下降;A组术前P-selectin明显高于B组(16.8%vs5.2%,P<0.05),术后快速下降;A组术前sP-selectin/Plt远高于B组(7.55vs2.03fg/plt,P=0.02),术后1个月两组均下降至正常水平;A组sE-selectin、P-selectin、sP-selectin/Plt在术后6个月仍维持低水平.A、B两组术前和术后L-selectin、sL-selectin水平无明显差异.结论E-selectin、P-selectin参与了ITP血小板破坏的病理过程.sE-selectin、sP-selectin/Plt水平可作为术前预测ITP脾切除疗效的参考指标,高者可能手术效果较好.

  11. 选择素在特发性血小板减少性紫癜中的表达%The selectin expression in patients with idiopathic thrombocytopenic purpura

    Institute of Scientific and Technical Information of China (English)

    钱珂; 秦锡虎; 邱国强

    2006-01-01

    目的研究选择素的表达在特发性血小板减少性紫癜(ITP)发生中的作用.方法激素治疗ITP23例中,13例疗效不良者为A组,10例疗效良好者为B组;另8例肝硬化脾亢者为C组,15名健康献血者为D组.抽全血用ELISA法检测可溶性E-选择素(E-selectin)、可溶性L-选择素(sL-selectin)、可溶性P-选择素(sP-selectin);通过流式细胞仪检测P-选择素(P-selectin)及L-选择素(L-selectin).结果血小板计数,A组为34.2×109/L,B组35.6×109/L,C组56.3×109/L,D组166×109/L.A组sE-selectin水平为53.1ng/ml,明显高于D组(28.3ng/ml,P<0.05);B组为21.2ng/ml;C组61.8ng/ml,也高于D组(P<0.05).A组sP-selectin水平上升(15.3ng/ml),高于B组(3.2ng/ml,P<0.01)和D组(10.9ng/ml);A组sP-selectin/Plt显著高于D组(6.9 vs 0.6fg/platelet、P<0.01);B、C组P-selectin低于D组,但sP-selectin/Plt却高于D组.A、B、C 3组sL-selectin、L-selectin水平与D组无明显差异.结论 P-selectin、E-selectin参与了ITP时血小板破坏的病理过程,sE-selectin、sP-selectin/Plt明显上升的ITP患者激素治疗效果可能较差,白细胞的活化与否和ITP无关.

  12. 妊娠并血小板减少性紫癜、母婴共患梅毒1例分析%Report on a gravida and her fetus complicated with syphilis and thrombocytopenic purpura

    Institute of Scientific and Technical Information of China (English)

    李克英; 李煜

    2002-01-01

    @@ 患者女性,32岁,于2001年11月4日入院,初步诊断妊娠34wk2/0 LOA妊娠并血小板减少性紫癜.2wk前曾到诊所打针,否认不洁性生活史.近2wk因双下肢碰撞后起瘀斑及肉眼血尿就诊.入院后查体:T36.8℃,P88/min,R20/min,BP13.0/9.0kpa(98/68 mmHg)全身浅表淋巴结无肿大,心肺正常,腹膨隆、无压痛,肝脾肋缘下未触及,双下肢水肿(+).产科检查:宫高30cm,腹围84cm,胎心140次/min,胎方位LOA,胎心监护正常;妇科检查:外阴、阴道无溃疡,无硬结.肛查:宫口未开.

  13. The Development of Severe Neonatal Alloimmune Thrombocytopenia due to Anti-HPA-1a Antibodies Is Correlated to Maternal ABO Genotypes

    Directory of Open Access Journals (Sweden)

    Maria Therese Ahlen

    2012-01-01

    Full Text Available Background. Maternal alloantibodies against HPA-1a can cross placenta, opsonize foetal platelets, and induce neonatal alloimmune thrombocytopenia (NAIT. In a study of 100, 448 pregnant women in Norway during 1995–2004, 10.6% of HPA-1a negative women had detectable anti-HPA-1a antibodies. Design and Methods. A possible correlation between the maternal ABO blood group phenotype, or underlying genotype, and severe thrombocytopenia in the newborn was investigated. Results. We observed that immunized women with blood group O had a lower risk of having a child with severe NAIT than women with group A; 20% with blood group O gave birth to children with severe NAIT, compared to 47% among the blood group A mothers (relative risk 0.43; 95% CI 0.25–0.75. Conclusion. The risk of severe neonatal alloimmune thrombocytopenia due to anti-HPA-1a antibodies is correlated to maternal ABO types, and this study indicates that the observation is due to genetic properties on the maternal side.

  14. High Prevalence of Sinusitis in Children with Henoch-Schönlein Purpura

    Directory of Open Access Journals (Sweden)

    Haruna Nakaseko

    2011-01-01

    Full Text Available We evaluated the prevalence and the types of infectious foci in oral as well as ear, nose, and throat diseases, and we examined incidence of renal involvement with active treatment for focal infection in children with Henoch-Schönlein Purpura. A total of 96 children who presented at Aichi Children's Health and Medical Center and were diagnosed as having HSP were evaluated for infectious foci in the ear, nose, throat, and oral cavities. Seventy-one of 96 children (74.0% had some type of infectious lesion, such as sinusitis or tonsillitis, and the prevalence of sinusitis was the highest (51 cases, 53.7%. In 44 HSP patients without renal involvement at the first examination, the incidence of nephritis was lower (13.6% than in previous reports (17–54% due to our aggressive intervention for infectious foci.

  15. Thrombocytosis following splenectomy and aortic valve replacement for idiopathic thrombocytopaenic purpura with bicuspid aortic valve

    Directory of Open Access Journals (Sweden)

    Sarika Katiyar

    2015-01-01

    Full Text Available Idiopathic thrombocytopaenic purpura (ITP patients are at high risk for complications during and after cardiac surgeries involving cardiopulmonary bypass. The main clinical problem of primary ITP is an increased risk of bleeding although bleeding may not always be present. More recently, thrombosis has become appreciated as another potential complication of the procedure. We report a 22-year-old female patient with ITP with bicuspid aortic valve and splenomegaly, who underwent uncomplicated aortic valve replacement and splenectomy simultaneously. She was readmitted with chest pain due to coronary thrombosis following splenectomy which made the management difficult. We describe our experience in managing this patient who presented with thrombotic complication rather than bleeding in post-operative period and the challenges met in maintaining appropriate anticoagulation for aortic valve replacement as well as thrombosis, post-splenectomy

  16. Endoscopic findings in a patient with Henoch-Sch(o)nlein purpura

    Institute of Scientific and Technical Information of China (English)

    Ming-Jen Chen; Tsang-En Wang; Wen-Hsiung Chang; Shu-Jung Tsai; Wen-Shen Liao

    2005-01-01

    Henoch-Sch(o)nlein purpura (HSP) is a systemic vasculitis of the small vessels of the skin, joints, GI tract, and kidney.It preferentially affects children but may also occur in adults. We report a 60-year-old man with HSP who presented with colicky abdominal pain, bloody diarrhea, arthralgia, and skin rash. The gastrointestinal tract was viewed by upper endoscopy and colonoscopy. We found characteristic endoscopic findings in the stomach, cecum and sigmoid colon, the combination of which has rarely been demonstrated in one patient. Histologic examination of skin biopsy specimens revealed leukocytoclastic vasculitis with positive staining for IgA in the capillaries. Endoscopy appears to have substantial diagnostic utility in patients suspected of having HSP, especially when abdominal symptoms precede the cutaneous lesions.

  17. Pneumococcal Sepsis Complicated by Splenic Abscesses and Purpura Fulminans in a 15-Month-Old Child

    Directory of Open Access Journals (Sweden)

    Scott Pangonis MD

    2016-02-01

    Full Text Available Streptococcus pneumoniae is an invasive organism that causes a wide range of common diseases, including sinusitis, acute otitis media, and pneumonia. Splenic abscesses and purpura fulminans (PF are rare complications of pneumococcal disease. Splenic abscesses caused by S pneumoniae have only been reported in the adult literature. PF has been described in the pediatric population as a rare complication in patients with invasive pneumococcal disease (IPD with and without underlying immunological disorders such as asplenia. Here, we report a patient with IPD complicated by splenic abscesses and PF. Our patient initially presented with bacteremia, septic shock, and disseminated intravascular coagulation. She subsequently developed PF and splenic abscesses. She survived her illness after receiving a total of 8 weeks of antibiotic therapy. This case highlights 2 rare complications of IPD and demonstrates the need to keep pneumococcal disease in the differential diagnosis even in children whose vaccination status is up to date.

  18. Henoch schonlein purpura--a 5-year review and proposed pathway.

    Directory of Open Access Journals (Sweden)

    Louise Watson

    Full Text Available Henoch Schonlein Purpura (HSP is the commonest systemic vasculitis of childhood typically presenting with a palpable purpuric rash and frequently involving the renal system. We are the first group to clinically assess, critically analyse and subsequently revise a nurse led monitoring pathway for this condition.A cohort of 102 children presenting with HSP to a secondary/tertiary level UK paediatric hospital over a five year period, were monitored using a nurse led care pathway. Using this cohort, the incidence (6.21 cases per 100,000 children per year and natural disease course of HSP nephritis (46% initial renal inflammation; 9% subsequent renal referral; 1% renal biopsy and immunosuppression was determined. Older patients were at higher risk of requiring a renal referral (renal referral 12.3 (8.4-13.5 years vs. normal outcome 6.0 (3.7-8.5 years; p<0.01. A normal urinalysis on day 7 had a 97% (confidence interval 90 to 99% negative predictive value in predicting a normal renal outcome.Using this data and existing literature base, The Alder Hey Henoch Schonlein Purpura Pathway was developed, a revised pathway for the screening of poor renal outcome in HSP. This is based on a six-month monitoring period for all patients presenting with HSP, which importantly prioritises patients according to the urine findings on day 7 and thus intensively monitors those at higher risk of developing nephritis. The pathway could be easily adapted for use in different settings and resources.The introduction of a standardised pathway for the monitoring of HSP will facilitate the implementation of disease registries to further our understanding of the condition and permit future clinical trials.

  19. Expansion of Circulating T Follicular Helper Cells in Children with Acute Henoch-Schönlein Purpura

    OpenAIRE

    Jue Xie; Yan Liu; Lei Wang; Guoxiang Ruan; Huiming Yuan; Hong Fang; Jianyong Wu; Dawei Cui

    2015-01-01

    Henoch-Schönlein purpura (HSP) is a common systemic small vessel vasculitis in children with disorder autoimmune responses. T follicular helper (TFH) cells play crucial roles in regulating immune responses. The aim of our study was to investigate the probable role of TFH cells in the pathogenesis of children with HSP. In this study, the frequency of circulating CXCR5+CD4+TFH cells with inducible costimulator (ICOS) expression in the children with acute HSP was significantly higher than that i...

  20. Henoch–Schönlein purpura (HSP) and high-dose immunoglobulin treatment in patient with familiar prostatic adenocarcinoma

    OpenAIRE

    Sobieszczańska, Małgorzata; Tubek, Sławomir; Poplicha, Dagmara; Grabelus, Anna; Pawełczak, Jacek

    2014-01-01

    A 52-y old man was admitted to our Department because of abdominal pains and diarrhea with fresh blood, with concomitant purpura over the whole body and edema of the both tarsal joints. The medical history of the patient included skin changes of similar character identified once 10–12 y before. The family history revealed prostate cancer (brother and father) and pulmonary carcinoma (mother and mother's sister). An administration of immunoglobulins in the course of HSP is a non-standard clinic...

  1. [Henoch-Schönlein purpura in a cocaine consumer man with HIV infection and ANCA-p positivity].

    Science.gov (United States)

    De Paoli, María C; Moretti, Dino; Scolari Pasinato, Carlos M; Buncuga, Martín G

    2016-01-01

    The Henoch-Schönlein purpura (HSP) is a small vessel vasculitis with IgA immune complex deposition. The presentation in adults is rare and severe. Reported cases of HSP in patients infected with HIV are scarce. Neutrophil cytoplasmic antibodies (ANCA) are commonly found in other systemic vasculitis, but rarely in HSP and even more unusual the perinuclear pattern. Beside small vessel vasculitis, positivity of ANCA can be detected in a number of different pathological conditions in association with infectious processes, including HIV, or cocaine use, and especially the pattern of ANCA-p, associated with drugs, inflammatory bowel or autoimmune diseases. We report the case of a 35 years old man with toxic habits (cocaine, marijuana) who consulted for abdominal pain, hematochezia and purpura on lower extremities, and later fever, joint pain and progression of purpura associated with nephritic syndrome and ANCA-p (+). During hospitalization HIV infection was detected. Renal biopsy showed IgA nephropathy with favorable response to corticosteroid and antiproteinuric treatment. The communication of the case is due to the rarity of the presentation and therapeutic diagnostic challenge. It remains to elucidate the role of ANCA in the pathogenesis and management of adult PSH. PMID:27576285

  2. Anti-KEL7 (anti-Js(b)) alloimmunization diagnostic supported by molecular KEL*6,7 typing in a pregnant woman with previous intrauterine deaths.

    Science.gov (United States)

    Boturão-Neto, Edmir; Chiba, Akemi Kuroda; Oliveira Barros, Melca Maria; Barretto de Mello, Adriana; Fabron, Antonio; Orlando Bordin, José

    2006-12-01

    Anti-KEL7 (anti-Js(b)) is a rare antibody that has been related to haemolytic transfusion reactions and HDN. We report a case of anti-KEL7 alloimmunization detected in a pregnant woman who had an obstetric previous history of four miscarriages and one stillborn. Employing classical immunohematological techniques, we studied the propositus and her available relatives. Due to the unavailability of commercial anti-KEL6 and anti-KEL7 reagents, we used a KEL*6,7 genotyping method as an alternative tool to contribute with the identification of the alloantibody origin. The results of KEL genotyping showed that the propositus was KEL*6/6 homozygous, while her second partner was KEL*7/7 homozygous. PMID:17097348

  3. Characterization of a human platelet antigen-1a-specific monoclonal antibody derived from a B cell from a woman alloimmunized in pregnancy.

    Science.gov (United States)

    Eksteen, Mariana; Tiller, Heidi; Averina, Maria; Heide, Gøril; Kjaer, Mette; Ghevaert, Cedric; Michaelsen, Terje E; Ihle, Øistein; Husebekk, Anne; Skogen, Bjørn; Stuge, Tor B

    2015-06-15

    Human platelet Ag (HPA)-1a, located on integrin β3, is the main target for alloantibodies responsible for fetal and neonatal alloimmune thrombocytopenia (FNAIT) in the white population. There are ongoing efforts to develop an Ab prophylaxis and therapy to prevent or treat FNAIT. In this study, an mAb specific for HPA-1a, named 26.4, was derived from an immortalized B cell from an alloimmunized woman who had an infant affected by FNAIT. It is the only HPA-1a-specific human mAb with naturally paired H and L chains. Specific binding of mAb 26.4, both native and recombinant forms, to platelets and to purified integrins αIIbβ3 (from platelets) and αVβ3 (from trophoblasts) from HPA-1a(+) donors was demonstrated by flow cytometry and surface plasmon resonance technology, respectively. No binding to HPA-1a(-) platelets or integrins was detected. Moreover, the Ab binds with higher affinity to integrin αVβ3 compared with a second HPA-1a-specific human mAb, B2G1. Further in vitro experimentation demonstrated that mAb 26.4 can opsonize HPA-1a(+) platelets for enhanced phagocytosis by monocytes, inhibit binding of maternal polyclonal anti-HPA-1a Abs, and weakly inhibit aggregation of HPA-1a-heterozygous platelets, the latter with no predicted clinical relevance. Thus, mAb 26.4 is highly specific for HPA-1a and could potentially be explored for use as a prophylactic or therapeutic reagent for FNAIT intervention and as a phenotyping reagent to identify women at risk for immunization.

  4. Púrpura trombocitopênica e anemia hemolítica auto-imune em pacientes internados com lúpus eritematoso sistêmico juvenil Trombocytopenic purpura and autoimmune hemolytic anemia in hospitalized patients with juvenile systemic lupus erythematosus

    Directory of Open Access Journals (Sweden)

    Jochebed Kyoung Kim

    2007-02-01

    esplenectomia. CONCLUSÕES: o CHE isolado foi uma manifestação grave em pacientes internados com LESJ, habitualmente associado a uma doença ativa e sistêmica.OBJECTIVE: to evaluate the hematological involvement (HI in hospitalized patients with juvenile systemic lupus erythematosus (JSLE. METHODS: from 1994 to 2005, 195 admissions occurred in 77 JSLE patients (American College of Rheumatology criteria and were followed by the Pediatric Rheumatology Unit of the Instituto da Criança - University of São Paulo. These admissions were evaluated according to the presence of HI at onset or during the evolution of the disease: autoimmune hemolytic anemia (AHA or thrombocytopenic purpura. All patients performed at least two complete blood counts. AHA was defined by a fall in hemoglobin levels (beyond 2 g/dl, reticulocytosis, increase in lactate dehydrogenase (LDH and indirect bilirubin levels, and a positive Coombs test. The hematologic manifestations associated with infection, neoplasia and aplastic anemia were excluded. RESULTS: HI occurred in 14 patients (18.9%, with 15 admissions. Among these patients, 11 were female, 7 had trombocytopenic purpura, 5 AHA and 2 Evans syndrome. HI as onset and single manifestation of JSLE was observed in three patients. All the patients with trombocytopenic purpura presented cutaneous bleeding (petechia and/or ecchymosis. All had disease activity and simultaneously presented other manifestations of JSLE, particularly nephritis and vasculitis. Initially, all patients received pulsetherapy with methylprednisolone and prednisone later. In three patients the treatment aimed predominantly the control of hematologic manifestations, with intravenous gammaglobulin. The most used immunossupressive therapies were intravenous cyclophosphamide, cyclosporine and azathioprine. One patient died of central nervous system bleeding. No patient needed splenectomy. CONCLUSIONS: isolated HI was a severe manifestation in hospitalized patients with JSLE, generally

  5. Púrpura em paciente com estrongiloidíase disseminada Purpura in patient with disseminated strongiloidiasis

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    Luciano C. Ribeiro

    2005-05-01

    Full Text Available A infecção pelo Strongyloides stercoralis em associação com imunosupressão pode manifestar-se com lesões em múltiplos órgãos e sistemas, caracterizando a forma disseminada da doença. Lesões cutâneas não são freqüentemente relatadas e, se presentes, manifestam-se como rash e petéquias. Púrpuras bem definidas são pouco descritas. No presente trabalho é descrito um caso de estrongiloidíase disseminada, com acometimento cutâneo em forma de púrpura, que se desenvolveu em um paciente timectomizado e usuário crônico de corticosteróide devido à miastenia gravis.The association of systemic corticosteroid therapy and dissemination of Strongyloides stercoralis has been increasingly documented in the literature. Skin involvement in disseminated strongyloidiasis has been reported and the most commonly described cutaneous manifestations are rash and petechial eruptions. We present a case of an immunosuppressed man that developed disseminated strongyloidiasis with extensive purpura.

  6. Relationship between immune parameters and organ involvement in children with Henoch-Schonlein purpura.

    Directory of Open Access Journals (Sweden)

    Yan-xiang Pan

    Full Text Available Henoch-Schonlein purpura (HSP is the most common type of connective tissue diseases which increasingly occurs in children in recent years and its pathogenesis remains unclear. In order to explore the immune parameters and underlying pathogenesis mechanism of children with HSP, the study involved 1232 patients with HSP having different clinical symptoms and their laboratory indicators were evaluated. Th1/Th2 imbalance and overactivity of Th2 cells can cause increase in the synthesis and release of immunoglobulins in children with HSP. The number of red blood cells and white blood cells in urine was directly proportional to the level of IgA and inversely proportional to the level of serum complements (C3 and C4. Activation of these complements caused by immunoglobulin in patients with HSP plays an important role in renal injury. The urinary protein content in children with HSP along with proteinuria was positively correlated with IgE level, and IgE mediated type 1 hypersensitivity can cause increase in capillary permeability and weakened the charge barrier; hence, it could be considered as one of the causes of proteinuria in HSP. Additionally, the NK cells percentage was reduced and impaired immune function of NK cells were related to the immune injury of the digestive tract and kidney.

  7. Increased serum HMGB1 levels in patients with Henoch-Schönlein purpura.

    Science.gov (United States)

    Chen, Tao; Guo, Zai-Pei; Wang, Wen-Ju; Qin, Sha; Cao, Na; Li, Meng-Meng

    2014-06-01

    High-mobility group box-1 (HMGB1) has been implicated as a pro-inflammatory cytokine in the pathogenesis of various inflammatory and autoimmune diseases. However, information about HMGB1 in Henoch-Schönlein purpura (HSP) is still unclear. Herein, we investigated the role of HMGB1 in patients with HSP and the pro-inflammatory effects of HMGB1 on human dermal microvascular endothelial cell line (HMEC-1). Serum HMGB1 levels in patients with HSP together with patients with allergic vasculitis (AV) and urticarial vasculitis (UV) were detected by enzyme-linked immunosorbent assay (ELISA). HMEC-1 cells were treated with HMGB1 at concentrations ranging from 4 ng/ml to 100 ng/ml. Serum HMGB1 levels were significantly increased in patients with HSP, AV and UV, when compared with those in control group. Moreover, abundant cytoplasmic expression of HMGB1 was observed in endothelial cells in lesional skin of HSP patients. Using membrane cytokine antibody array, we indicate that HMGB1 markedly induced TNF-α and IL-6 release in cultured supernatant. Furthermore, by real-time quantitative PCR and ELISA, the effects of HMGB1 on these cytokines production in HMEC-1 cells were established. Finally, Western blot data revealed that HMGB1 can induce phosphorylation of inhibitor of κB-α (IκBα) and the nuclear translocation of nuclear factor-κB (NF-κB) p65 in HMEC-1 cells. In conclusion, this study provides first observations on the association of HMGB1 with HSP. We suggest that HMGB1 may be an important mediator of endothelial inflammation through the induction of TNF-α and IL-6 production and may play a crucial role in the pathogenesis of HSP. PMID:24758390

  8. Thirty-five Infantile Purpura Nephritis Patients Treated with Integrated Traditional Chinese and Western Medicine

    Institute of Scientific and Technical Information of China (English)

    2001-01-01

    @@  Thirty-five patients of infantile purpura nephritis (IPN) were treated with integrated traditional Chinese and western medicine (TCM-WM) from January 1994 to December 1998, with good efficacy obtained, and following is the report. METHODS Clinical Data   All the 65 cases were inpatients, and conformed to the “Standards of the Diagnosis and Therapeutical Effect of Hematological Diseases” (edited by ZHANG Zhi-nan. Tianjin: Tianjin Science and Technology Press, 1999∶235-239). They were randomly divided into two groups: Treated group: 35 cases, 23 males, 12 females; aged 4-13 years, 7 years on average; disease course 4-18 days, the mean 10 days; mild type 19 cases (asymptomatic hematuria, proteinuria), nephritis syndrome type 13 cases (hematuria, proteinuria, edema, hypertension), and nephrotic syndrome type 3 cases (typical manifestation of nephrosis complicated with renal insufficiency). Control group: 30 cases, 20 males, 10 females; aged 3-14 years, 6 years on average; disease course 3-16 days, the mean 9 days; mild type 16 cases, nephritis syndrome type 10 cases, nephrotic syndrome type 4 cases. The clinical manifestation, sex, age, hospitalisation time of these 2 groups showed insignificant difference. Method of Treatment   The control group: Adopting intravenous dripping of dexamethasone 0.3-0.5 mg*kg-1*d-1, consecutively for 5-7 days, which was then shifted to prednisone 1-1.5 mg*kg-1*d-1, altogether 2-3 weeks, with Vit C, persantin, chlorphenamine, calcium, etc., orally taken.   The treated (TCM-WM) group, on the basis of the above-mentioned WM treatment, had also TCM syndrome differentiation performed, and were classified into 3 types:

  9. Hemolytic disease of the newborn associated with anti-Jra alloimmunization in a twin pregnancy: the first case report in Korea.

    Science.gov (United States)

    Kim, Hyungsuk; Park, Min-Jeong; Sung, Tae-Jung; Choi, Ji Seon; Hyun, Jungwon; Park, Kyoung Un; Han, Kyou-Sup

    2010-10-01

    Jr(a) is a high-frequency antigen found in all ethnic groups. However, the clinical significance of the anti-Jr(a) antibody has remained controversial. Most studies have reported mild hemolytic disease of the newborn and fetus (HDNF) in Jr(a)-positive patients. Recently, fatal cases of HDNF have also been reported. We report the first case of HDNF caused by anti-Jr(a) alloimmunization in twins in Korea. A 33-yr-old nulliparous woman with no history of transfusion or amniocentesis was admitted at the 32nd week of gestation because of vaginal bleeding caused by placenta previa. Anti-Jr(a) antibodies were detected in a routine laboratory examination. An emergency cesarean section was performed at the 34th week of gestation, and 2 premature infant twins were delivered. Laboratory examination showed positive direct antiglobulin test and Jr(a+) phenotype in the red blood cells and the presence of anti-Jr(a) antibodies in the serum in both neonates. The infants underwent phototherapy for neonatal jaundice; this was followed by conservative management. They showed no further complications and were discharged on the 19th postpartum day. Preparative management to ensure the availability of Jr(a-) blood, via autologous donation, and close fetal monitoring must be performed even in cases of first pregnancy in Jr(a-) women.

  10. Hemolytic disease of the newborn associated with anti-Jra alloimmunization in a twin pregnancy: the first case report in Korea.

    Science.gov (United States)

    Kim, Hyungsuk; Park, Min-Jeong; Sung, Tae-Jung; Choi, Ji Seon; Hyun, Jungwon; Park, Kyoung Un; Han, Kyou-Sup

    2010-10-01

    Jr(a) is a high-frequency antigen found in all ethnic groups. However, the clinical significance of the anti-Jr(a) antibody has remained controversial. Most studies have reported mild hemolytic disease of the newborn and fetus (HDNF) in Jr(a)-positive patients. Recently, fatal cases of HDNF have also been reported. We report the first case of HDNF caused by anti-Jr(a) alloimmunization in twins in Korea. A 33-yr-old nulliparous woman with no history of transfusion or amniocentesis was admitted at the 32nd week of gestation because of vaginal bleeding caused by placenta previa. Anti-Jr(a) antibodies were detected in a routine laboratory examination. An emergency cesarean section was performed at the 34th week of gestation, and 2 premature infant twins were delivered. Laboratory examination showed positive direct antiglobulin test and Jr(a+) phenotype in the red blood cells and the presence of anti-Jr(a) antibodies in the serum in both neonates. The infants underwent phototherapy for neonatal jaundice; this was followed by conservative management. They showed no further complications and were discharged on the 19th postpartum day. Preparative management to ensure the availability of Jr(a-) blood, via autologous donation, and close fetal monitoring must be performed even in cases of first pregnancy in Jr(a-) women. PMID:20890084

  11. EULAR/PRINTO/PRES criteria for Henoch-Schönlein purpura, childhood polyarteritis nodosa, childhood Wegener granulomatosis and childhood Takayasu arteritis: Ankara 2008. Part II: Final classification criteria

    DEFF Research Database (Denmark)

    Ozen, Seza; Pistorio, Angela; Iusan, Silvia M;

    2010-01-01

    To validate the previously proposed classification criteria for Henoch-Schönlein purpura (HSP), childhood polyarteritis nodosa (c-PAN), c-Wegener granulomatosis (c-WG) and c-Takayasu arteritis (c-TA)....

  12. Clinical and misdiagnosis of abdominal type Henoch-Sch o nlein purpura purpura of child patients%儿童腹型过敏性紫癜临床及误诊分析

    Institute of Scientific and Technical Information of China (English)

    施亚娟

    2014-01-01

    Objective To analyze the clinical charscteristics and misdiagnosis of abdominal type Henoch-Sch o nlein purpura (HSP). Methods The clinical data of patients with Henoch-Schonlein purpura from January 2010 to August 2014 in our hospital was induced and analyzed. Results 297 patients (100%) had skin rash,among them,34 patients had no typical skin rash.For patients complicated with gastrointestinal symptom,189 (64%) were diagnosed as abdominal type HSP,136 cases of abdominal type HSP had the onset clinical manifestation of skin purpura.All patients of abdomi-nal type HSP had abdominal pain,some patients had digestive tract hemorrhage,vomiting and diarrhea,digestive tract symptom of patients is severe,abdominal sign is light,1 case occurred intestinal perforation,2 case s occurred intussus-ception.Endoscopy of 52 cases displayed that mucosal manifestations was mainly hyperemia,edema,hemorrhage point, ulcer. Conclusion Abdominal type HSP with gastrointestinal system was easily misdiagnosed,early endoscopy for early diagnosis,treatment had some help.%目的:探讨儿童腹型过敏性紫癜(HSP)的临床特点及误诊情况,提高对该病的认识。方法收集本院2010年1月~2014年8月住院的HSP患儿的临床资料,对其进行归纳和分析。结果297例(100%)HSP患儿均出现皮疹,其中34例皮疹不典型,合并消化道症状诊断为腹型HSP189例(64%),腹型组中136例患儿以皮肤紫癜首发,所有腹型HSP患儿均有腹痛,部分患儿合并消化道出血、呕吐、腹泻,患儿消化道症状重,腹部体征轻,1例合并肠穿孔,另2例合并肠套叠。52例患儿行胃镜检查:黏膜主要表现为充血、水肿、出血点、溃疡。结论以消化道症状首发的腹型HSP容易被误诊,尽早行内镜检查对早期诊断、治疗有一定的帮助。

  13. Co-stimulation with LPS or Poly I:C markedly enhances the anti-platelet immune response and severity of fetal and neonatal alloimmune thrombocytopenia.

    Science.gov (United States)

    Li, Conglei; Chen, Pingguo; Vadasz, Brian; Ma, Li; Zhou, Hui; Lang, Sean; Freedman, John; Ni, Heyu

    2013-12-01

    Fetal and neonatal alloimmune thrombocytopenia (FNAIT) is a life-threatening bleeding disorder caused by maternal antibodies against fetal/neonatal platelets. FNAIT is also linked with miscarriages, although the incidence and mechanisms of fetal death have not been well studied. IntegrinαIIbβ3 (GPIIbIIIa) and the GPIbα complex are major glycoproteins expressed on platelets and are also major antigens targeted in autoimmune thrombocytopenia (ITP), but reported cases of anti-GPIb-mediated FNAIT are rare. Bacterial and viral infections have been causally linked with the pathogenesis of immune-mediated thrombocytopenia (ITP); however, it is unknown whether these infections contribute to the severity of FNAIT. Here, immune responses against platelet antigens were examined by transfusing wild-type (WT) mouse platelets into β3-/- or GPIbα-/- mice. To mimic bacterial or viral infections, lipopolysaccharide (LPS) or polyinosinic:polycytidylic acid (Poly I:C) were injected intraperitoneally following platelet transfusions. The FNAIT model was established by breeding the immunised female mice with WT male mice. We demonstrated for the first time that the platelet GPIbα has lower immunogenicity compared to β3 integrin. Interestingly, co-stimulation with LPS or Poly I:C markedly enhanced the immune response against platelet GPIbα and caused severe pathology of FNAIT (i.e. miscarriages). LPS or Poly I:C also enhanced the immune response against platelet β3 integrin. Our data suggest that bacterial and viral infections facilitate the anti-platelet GPIbα response, which may lead to a severe non-classical FNAIT (i.e. miscarriage but not neonatal bleeding) that has not been adequately reported in humans.

  14. Trombocitopenia fetal/neonatal aloinmune: Revisión a propósito de un caso Foetal/neonatal alloimmune thrombocytopenia: A review and case report

    Directory of Open Access Journals (Sweden)

    P. Rodríguez Wilhelmi

    2008-12-01

    Full Text Available La trombocitopenia fetal/neonatal aloinmune (TFNA es la causa más frecuente de trombocitopenia grave en el recién nacido. Es un proceso agudo donde las plaquetas fetales son destruidas durante la gestación por un anticuerpo de tipo IgG presente en la madre aloinmunizada. En la raza caucásica, tiene especificidad frente al antígeno específico plaquetar HPA-1a en más del 80% de los casos. La hemorragia intracraneal, que ocurre hasta en un 30%, es la complicación más grave, con un 10% de mortalidad y un 20% de secuelas neurológicas irreversibles. El alto riesgo de repetición de hemorragia grave en futuras gestaciones obliga a plantearse profilaxis o tratamiento antenatal. El diagnóstico precoz puede permitir administrar un tratamiento eficaz basado en la transfusión de plaquetas de fenotipo HPA compatible o de inmunoglobulinas endovenosas. Presentamos el caso de una gestante de 27 años, que en la semana 35 de su segunda gestación fue diagnosticada por ecografía de hidrocefalia fetal bilateral. En la cesárea realizada en la semana 36, el neonato presentó hematomas en hombro y glúteo izquierdos, macrocefalia con fontanelas a tensión y salida de líquido cefalorraquídeo hemorrágico tras la colocación de un catéter de derivación externo. El hemograma reveló trombocitopenia grave (9 x 10(9/L. Ante sospecha clínica de TFNA, se transfundieron plaquetas de donante no fenotipado para el HPA-1a y se inició tratamiento con inmunoglobulinas endovenosas, con recuperación de la trombocitopenia, pero con secuelas neurológicas probablemente irreversibles. El estudio inmunohematológico confirmó el fenotipo materno HPA-1a negativo, el fenotipo neonatal HPA-1a positivo y la presencia de aloanticuerpos anti-HPA-1a en el suero materno. La profilaxis y el tratamiento continúan siendo, en la actualidad, motivo de discusión y controversia, así como la posibilidad de realizar un cribado antenatal.Foetal/neonatal alloimmune

  15. Experience of buffy coat pooling of platelets as a supportive care in thrombocytopenic dengue patients: A prospective study

    Directory of Open Access Journals (Sweden)

    Kabita Chatterjee

    2014-01-01

    Full Text Available Random donor platelet (RDP is not sufficient to improve the platelet count in most thrombocytopenic patients. Single donor platelet (SDP or buffy coat pooled platelet (BCPP are the two choices to provide a full therapeutic dose of platelets. However, there are constraints in the preparation of SDP due to stringent donor selection procedure, time required for procedure, and need of special expensive equipments and kits. BCPP is widely practiced, especially in the European countries, since 1995. In India, we decided to adopt the procedure of buffy coat pooling of platelets, especially for economically backward patients and for emergencies. This study was prospectively conducted from September 2009 to September 2010. A total of 129 units of BCPP [tested prior for viral markers by enzyme-linked immunosorbent assay (ELISA and individual donor nucleic acid amplification test (ID-NAT] were issued to 129 patients suffering from dengue and were included in this study. For comparison between efficacy of SDP and BCCP, patients were divided into two groups of 50 each. The post-transfusion platelet counts of the patients were noted after 2 hours of transfusion for each type of component. The platelet yield varied from 2.5 to 4.4 Χ 10ΉΉ in BCPP samples. The samples analyzed were sterile without any contamination. The different biochemical parameters were analyzed in detail. The observed post-transfusion platelet recovery and corrected count increment (CCI at 1 hour and 24 hours after BCPP transfusion were similar to that after SDP transfusion. Hence, we concluded that BCPP can be a low cost alternative to SDP in the times of emergencies like dengue and non-affordability by the patient for SDP.

  16. Henoch-Schönlein purpura in an older man presenting as rectal bleeding and IgA mesangioproliferative glomerulonephritis: a case report

    Directory of Open Access Journals (Sweden)

    Howarth Charles B

    2011-08-01

    Full Text Available Abstract Introduction Henoch-Schönlein purpura is the most common systemic vasculitis in children. Typical presentations are palpable purpura, abdominal pain, arthritis, and hematuria. This vasculitic syndrome can present as an uncommon cause of rectal bleeding in older patients. We report a case of an older man with Henoch-Schönlein purpura. He presented with rectal bleeding and acute kidney injury secondary to IgA mesangioproliferative glomerulonephritis. Case presentation A 75-year-old Polish man with a history of diverticulosis presented with a five-day history of rectal bleeding. He had first noticed colicky left lower abdominal pain two months previously. At that time he was treated with a 10-day course of ciprofloxacin and metronidazole for possible diverticulitis. He subsequently presented with rectal bleeding to our emergency department. Physical examination revealed generalized palpable purpuric rash and tenderness on his left lower abdomen. Laboratory testing showed a mildly elevated serum creatinine of 1.3. Computed tomography of his abdomen revealed a diffusely edematous and thickened sigmoid colon. Flexible sigmoidoscopy showed severe petechiae throughout the colon. Colonic biopsy showed small vessel acute inflammation. Skin biopsy resulted in a diagnosis of leukocytoclastic vasculitis. Due to worsening kidney function, microscopic hematuria and new onset proteinuria, he underwent a kidney biopsy which demonstrated IgA mesangioproliferative glomerulonephritis. A diagnosis of Henoch-Schönlein purpura was made. Intravenous methylprednisolone was initially started and transitioned to prednisone tapering orally to complete six months of therapy. There was marked improvement of abdominal pain. Skin lesions gradually faded and gastrointestinal bleeding stopped. Acute kidney injury also improved. Conclusion Henoch-Schönlein purpura, an uncommon vasculitic syndrome in older patients, can present with lower gastrointestinal bleeding

  17. A Case of Thrombotic Thrombocytopenia Purpura Associated with Systemic Lupus Erythematosus: Diagnostic Utility of ADAMTS-13 Activity

    Directory of Open Access Journals (Sweden)

    Risa Yamada

    2011-01-01

    Full Text Available Thrombotic thrombocytopenia purpura (TTP caused by a deficiency in ADAMTS-13 activity is considered to involve a subset of thrombotic microangiopathy (TMA. Although concept of TTP is included under the umbrella of TMA, discrimination of TTP from TMA is occasionally difficult in an autoimmune disorder. Herein, we report a case with TTP associated with systemic lupus erythematosus (SLE. In this case, it was difficult to discriminate TTP from TMA and the measurement of ADAMTS-13 activity was useful for obtaining an accurate diagnosis. SLE patients having thrombocytopenia in complication with anemia should be considered a monitoring of ADAMTS-13 activity even though the patients lacked symptoms of TTP related to the microvascular coagulation.

  18. Number and function of peripheral blood endothelial progenitor cells in Henoch-Schönlein purpura nephritis children with different degrees of renal vascular lesions

    OpenAIRE

    DANG, XI-QIANG; HE, XIAO-JIE; CHEN, HAI-XIA; HE, QING-NAN; Yi, Zhu-Wen

    2012-01-01

    The aim of this study was to explore the correlation between different degrees of renal vascular lesions in children with Henoch-Schönlein purpura nephritis (HSPN) and changes in progenitor cell number and function in peripheral blood. Forty-eight HSPN patients were divided into three groups, mild, moderate and severe, according to the degree of renal vascular lesions. Peripheral blood mononuclear cells were isolated and cultured. Endothelial progenitor cells (EPCs) were identified by immunof...

  19. Cocaine-associated retiform purpura: a C5b-9-mediated microangiopathy syndrome associated with enhanced apoptosis and high levels of intercellular adhesion molecule-1 expression.

    Science.gov (United States)

    Magro, Cynthia M; Wang, Xuan

    2013-10-01

    Cocaine-associated retiform purpura is a recently described entity characterized by striking hemorrhagic necrosis involving areas of skin associated with administration of cocaine. Levamisole, an adulterant in cocaine, has been suggested as the main culprit pathogenetically. Four cases of cocaine-associated retiform purpura were encountered in the dermatopathology practice of C. M. Magro. The light microscopic findings were correlated with immunohistochemical and immunofluorescence studies. All 4 cases showed a very striking thrombotic diathesis associated with intravascular macrophage accumulation. Necrotizing vasculitis was noted in 1 case. Striking intercellular adhesion molecule-1 (ICAM-1)/CD54 expression in vessel wall along with endothelial expression of caspase 3 and extensive vascular C5b-9 deposition was observed in all biopsies examined. Cocaine-induced retiform purpura is a C5b-9-mediated microvascular injury associated with enhanced apoptosis and prominent vascular expression of ICAM-1, all of which have been shown in prior in vitro and in vivo murine models to be a direct effect of cocaine metabolic products. Antineutrophilic cytoplasmic antibody and antiphospholipid antibodies are likely the direct sequelae of the proapoptotic microenvironment. The inflammatory vasculitic lesion could reflect the downstream end point reflective of enhanced ICAM-1 expression and the development of antineutrophilic cytoplasmic antibody. Levamisole likely works synergistically with cocaine in the propagation of this syndromic complex. PMID:23392134

  20. Haematology and neurology

    Science.gov (United States)

    Austin, Steven; Cohen, Hannah; Losseff, Nick

    2007-01-01

    This review aims to update the reader on advances in the understanding of haematological conditions that may arise in neurological practice. Thrombophilia, antiphospholipid antibody syndrome, thrombotic thrombocytopenic purpura, sickle cell and clonal disorders associated with neuropathy are discussed. PMID:17369588

  1. Ticlopidine

    Science.gov (United States)

    ... fever. This condition is called thrombotic thrombocytopenic purpura (TTP).Call your doctor immediately if you have yellowing ... ulcers, low blood cell counts (neutropenia, thrombocytopenia, anemia, TTP), kidney disease, high blood cholesterol, or high blood ...

  2. Alfalfa

    Science.gov (United States)

    Alfalfa is an herb. People use the leaves, sprouts, and seeds to make medicine. Alfalfa is used for kidney conditions, bladder and prostate ... bleeding disorder called thrombocytopenic purpura. People also take alfalfa as a source of vitamins A, C, E, ...

  3. Henoch-Schönlein purpura in children: its relation to oral and to oral and oral and dental health

    Directory of Open Access Journals (Sweden)

    Arlette Suzy Puspa Pertiwi

    2012-09-01

    Full Text Available > Background: Henoch-Schönlein purpura (HSP is a rare systemic small vessel vasculitis, which commonly occur in childrenbetween 2 and 10 years of age. The course of the disease is often self-limiting, although may manifest long-term renal morbidity. Theseverity of renal involvement decides about the prognosis of this disease. Many factors can trigger the disease attack, which is the mostcommon is bacterial invasion. Since the oral cavity is often refer as infectious foci to other part of the body, it seemed rationally tobe part that contribute the course of disease, thus management of these infectious foci, if possible, gives rise to an astoundingly goodprognosis. Purpose: This paper will describe a review on HSP and the possible association with oral and dental health since it mightbe related to the prognosis of HSP. reviews: Rashes in children are common; they may develop a rash after prescription of antibiotics.Nevertheless there are some childhood diseases that may manifest a rash presentation, such as HSP. It is important for pediatric dentistto have knowledge about HSP and consider the possibility of dental treatment or disease as potential triggers. Conclusion: Oral anddental condition may be the trigger cause of HSP attack. Therefore, it is important for pediatric dental practitioner to be aware of thecourse of the disease in order to limit the expanding complications.> latar belakang: Henoch-Schönlein purpura (HSP merupakan vaskulitis pembuluh darah kecil sistemik yang jarang terjadi danbiasanya menyerang anak usia 2 hingga 10 tahun. Penyakit tersebut seringkali dapat sembuh sendiri, tetapi pada jangka panjangdapat bermanifestasi dengan morbiditas ginjal. Keparahan keterlibatan ginjal menentukan prognosis penyakit. Banyak faktor yangdapat memicu serangan penyakit, tersering adalah invasi bakteri. Karena rongga mulut sering kali merupakan fokus infeksi terhadapbagian lain dari tubuh, maka mempunyai peluang sebagai faktor pemicu

  4. Changes of serum IL-6, IL-18 and IFN-γ in children with idiopathic thrombocytopenic purura%IL-6、IL-18和IFN-γ在特发性血小板减少性紫癜中的水平变化

    Institute of Scientific and Technical Information of China (English)

    路桂云

    2013-01-01

    目的 通过测定特发性血小板减少性紫癜(ITP)患儿血清中白细胞介素-6(IL-6)、白细胞介素-18(IL-18)和γ-干扰素(IFN-γ)的含量变化,探讨IL-6、IL-18和IFN-γ在ITP的发病机制中的作用.方法 选择ITP急性发作期患儿40例(急性组),经治疗后处于缓解期的患儿35例(缓解组),另选择30名健康儿童作为对照(对照组),采用酶联免疫法(ELISA)检测血清IL-6、IL-18和IFN-γ水平变化,并进行统计学分析比较.结果 ITP患儿血清IL-6、IL-18和IFN-γ水平急性期组明显高于缓解期组和对照组,缓解期组亦高于对照组,差异均有统计学意义(P均<0.05).结论 IL-6、IL-18和IFN-γ可能均参与了ITP的发病机制,通过测定血清IL-6、IL-18和IFN-γ水平可以用来判断ITP的病情和预后.%Objective To study the content changes of serum IL-6,IL-18 and IFN-γ in children with idiopathic thrombocytopenic purpura (ITP),and to investigate the role in the pathogenesis of IL-6,IL-18 and IFN-γin ITP.Methods Active group (n =40) and remission group (n =35) with ITR were picked up,and 30 healthy children served as normal controls.IL-6,IL-18 and IFN-γ levels were measured by ELISA,and the results were compared.Results The serum levels of IL-6,IL-18 and IFN-γ in children with active group were markedly higher than those in remission group and healthy controls,and the serum levels of IL-6,IL-18 and IFN-γin remission group were markedly higher than those in healthy controls (P < 0.05).Conclusions IL-6,IL-18 and IFN-γmaybe involved in the pathogenesis of ITP.Determination of IL-6,IL-18 and INF-γ levels can be used to judge the ITP' s condition and prognosis.

  5. The accuracy of platelet counting in thrombocytopenic blood samples distributed by the UK National External Quality Assessment Scheme for General Haematology.

    Science.gov (United States)

    De la Salle, Barbara J; McTaggart, Paul N; Briggs, Carol; Harrison, Paul; Doré, Caroline J; Longair, Ian; Machin, Samuel J; Hyde, Keith

    2012-01-01

    A knowledge of the limitations of automated platelet counting is essential for the effective care of thrombocytopenic patients and management of platelet stocks for transfusion. For this study, 29 external quality assessment specimen pools with platelet counts between 5 and 64 × 10(9)/L were distributed to more than 1,100 users of 23 different hematology analyzer models. The same specimen pools were analyzed by the international reference method (IRM) for platelet counting at 3 reference centers. The IRM values were on average lower than the all-methods median values returned by the automated analyzers. The majority (~67%) of the automated analyzer results overestimated the platelet count compared with the IRM, with significant differences in 16.5% of cases. Performance differed between analyzer models. The observed differences may depend in part on the nature of the survey material and analyzer technology, but the findings have implications for the interpretation of platelet counts at levels of clinical decision making.

  6. Expression of Interleukin—10 mRNA in Children with Different Clinical Types of Henoch—Schonlein Purpura Nephritis

    Institute of Scientific and Technical Information of China (English)

    WuYJ; ChenRH

    2002-01-01

    Objective To investigate the role of interleukin-10(IL-10) in the pathogenesis of different clinical types Henoch-Schonlein purpura nephritis(HSPN).Methods The IL-10 protein was determined by ELISA and the expression of IL-10 mRNA was measured by reverse transcription-polymerase chain reaction (RT-PCR) in phytohemagglutinin(PHA)-stimulating peripheral blood mononuclear cells(PBMCS) in 40 children with HSPN during their acute phase and remission stage.Results:(1)The levels of IL-10 mRNA and protein were significantly increased in patients in acute phase compared with controls[(0.48±0.12)vs(0.39±0.10)and(1023.90±158.78)pg/ml vs(895.51±141.06)pg/ml;P0.05 for both].(2)The IL-10 mRNA and protein of HSPN patients in acute phase with proteinuria,nephrotic syndrome or acute glomerulonephritis syndrome was signficantly higher than that in controls.There was no significant difference between the acute phase of HSPN patients with isolated hematuria and the controls.Conclusion The results suggested that the expression of IL-10 in PBMC was correlated to clinical type of HSPN.IL-10 has protective action in the pathogenesis of HSPN.

  7. Expansion of Circulating T Follicular Helper Cells in Children with Acute Henoch-Schönlein Purpura

    Directory of Open Access Journals (Sweden)

    Jue Xie

    2015-01-01

    Full Text Available Henoch-Schönlein purpura (HSP is a common systemic small vessel vasculitis in children with disorder autoimmune responses. T follicular helper (TFH cells play crucial roles in regulating immune responses. The aim of our study was to investigate the probable role of TFH cells in the pathogenesis of children with HSP. In this study, the frequency of circulating CXCR5+CD4+TFH cells with inducible costimulator (ICOS expression in the children with acute HSP was significantly higher than that in healthy controls (HCs but not CXCR5+CD4+TFH cells with programmed death-1 (PD-1 expression. Moreover, serum levels of IL-21 and IL-6 cytokines, IgA, and C3 in HSP children were also significantly higher than those in HCs. A positive correlation was observed between the frequencies of circulating ICOS+CXCR5+CD4+TFH cells and the serum IL-21 or IgA levels of acute HSP children, respectively. Additionally, the mRNA expression levels of interleukin- (IL- 21, IL-6, and transcriptional factors (B-cell lymphoma-6, Bcl-6 were also significantly increased in peripheral blood from acute HSP children compared to HCs. Taken together, these findings suggest that TFH cells and associated molecules might play critical roles in the pathogenesis of HSP, which are possible therapeutic targets in HSP children.

  8. Expansion of Circulating T Follicular Helper Cells in Children with Acute Henoch-Schönlein Purpura.

    Science.gov (United States)

    Xie, Jue; Liu, Yan; Wang, Lei; Ruan, Guoxiang; Yuan, Huiming; Fang, Hong; Wu, Jianyong; Cui, Dawei

    2015-01-01

    Henoch-Schönlein purpura (HSP) is a common systemic small vessel vasculitis in children with disorder autoimmune responses. T follicular helper (TFH) cells play crucial roles in regulating immune responses. The aim of our study was to investigate the probable role of TFH cells in the pathogenesis of children with HSP. In this study, the frequency of circulating CXCR5(+)CD4(+)TFH cells with inducible costimulator (ICOS) expression in the children with acute HSP was significantly higher than that in healthy controls (HCs) but not CXCR5(+)CD4(+)TFH cells with programmed death-1 (PD-1) expression. Moreover, serum levels of IL-21 and IL-6 cytokines, IgA, and C3 in HSP children were also significantly higher than those in HCs. A positive correlation was observed between the frequencies of circulating ICOS(+)CXCR5(+)CD4(+)TFH cells and the serum IL-21 or IgA levels of acute HSP children, respectively. Additionally, the mRNA expression levels of interleukin- (IL-) 21, IL-6, and transcriptional factors (B-cell lymphoma-6, Bcl-6) were also significantly increased in peripheral blood from acute HSP children compared to HCs. Taken together, these findings suggest that TFH cells and associated molecules might play critical roles in the pathogenesis of HSP, which are possible therapeutic targets in HSP children. PMID:26491701

  9. Antigenic Complementarity in the Origins of Autoimmunity: A General Theory Illustrated With a Case Study of Idiopathic Thrombocytopenia Purpura

    Directory of Open Access Journals (Sweden)

    Robert Root-Bernstein

    2006-01-01

    Full Text Available We describe a novel, testable theory of autoimmunity, outline novel predictions made by the theory, and illustrate its application to unravelling the possible causes of idiopathic thrombocytopenia purpura (ITP. Pairs of stereochemically complementary antigens induce complementary immune responses (antibody or T-cell that create loss of regulation and civil war within the immune system itself. Antibodies attack antibodies creating circulating immune complexes; T-cells attack T-cells creating perivascular cuffing. This immunological civil war abrogates the self-nonself distinction. If at least one of the complementary antigens mimics a self antigen, then this unregulated immune response will target host tissues as well. Data demonstrating that complementary antigens are found in some animal models of autoimmunity and may be present in various human diseases, especially ITP, are reviewed. Specific mechanisms for preventing autoimmunity or suppressing existing autoimmunity are derived from the theory, and critical tests proposed. Finally, we argue that Koch's postulates are inadequate for establishing disease causation for multiple-antigen diseases and discuss the possibility that current research has failed to elucidate the causes of human autoimmune diseases because we are using the wrong criteria.

  10. An unusual presentation of childhood vasculitis presenting in adulthood: A challenging diagnosis of Henoch-Schönlein Purpura

    Directory of Open Access Journals (Sweden)

    Charat Thongprayoon

    2014-01-01

    Full Text Available Context: Henoch-Schφnlein purpura (HSP, a systemic IgA vascultitis, is uncommon in adults, with an incidence rate of 0.1 to 1.2 per million in adults over 20 years old. This vasculitic syndrome can present as an uncommon cause of intestinal obstruction in older patients. We report a case of an older woman with HSP presenting with small bowel obstruction and vasculitic rash. Case Report: We report a 67-year-old woman who presented with small bowel obstruction and skin rash. Skin biopsy revealed leukocytoclastic vasculitis with +IgA granular deposition within the walls of superficial dermal vessels. Kidney biopsy confirmed the diagnosis of HSP with mild mesangial proliferative IgA nephropathy. Her abdominal pain and small bowel obstruction were improved with conservative treatment. She continued to do well with normal kidney function at a 3-month follow-up visit. Conclusion: HSP, a systemic IgA vasculitis, is a predominantly pediatric vasculitis and is uncommon in adults. In adults, the disease process is identical to that in children. However, gastrointestinal manifestation is less common in older patients, and bowel perforation and obstruction are rare. Intestinal obstruction with skin rash and renal involvement should raise suspicions of HSP.

  11. 儿童过敏性紫癜饮食影响因素分析%Analysis of dietary effect factors of childhood allergic purpura

    Institute of Scientific and Technical Information of China (English)

    宇丽; 汤玉霞; 牛霞; 周其莲; 余淼; 谢伦芳

    2015-01-01

    目的 探讨儿童过敏性紫癜( HSP)饮食习惯中可能的影响因素,为HSP的防治提供建议和指导. 方法 采用成组设计的病例对照研究方法,选取114 例首发HSP儿童为病例组,以同期住院的性别、年龄匹配的132 例诊断为上呼吸道感染的儿童为对照组,通过问卷回顾性调查两组儿童发病前1个月饮食行为相关信息. 进行单因素与多因素非条件Logistic回归分析. 结果 单因素分析结果显示,常吃快餐、常喝白开水/果汁、经常摄入鱼虾等海产品、零食以肉类制品为主及进餐不定量等5个因素与儿童HSP有关( P<0. 1);多因素分析结果显示,常喝白开水/果汁为保护因素.常吃鱼虾等海产品及进餐不定量是儿童HSP的重要危险因素(P<0. 05). 结论 健康饮食、减少过敏原接触、培养良好饮食习惯等对预防儿童HSP的发生有重要意义.%Objective To find out the dietary influence factors of childhood allergic purpura and to provide advice and guidance for the prevention of childhood allergic purpura. Methods A case-control study was conducted on two groups of children. The case group contained 114 children with allergic purpura and the control group contained 132 children with upper respiratory tract infection. Questionnaire included eating behaviors and other relevant infor-mation. The data were analyzed by mono-factorial and multi-factorial unconditional logistic regression. Results Factors that were independently associated with the development of allergic purpura,would include eating fast food, fish and shrimp,meat snacks,not quantitative dining,drinking water and fruit juice. Mutivariate analyses showed that drinking water and fruit juice were protective factors;meanwhile,eating fish and shrimp,not quantitative dining were the independent risk factors of allergic purpura ( P<0. 05 ) . Conclusion The healthy diet and reducing al-lergen contact have important significance for protecting children from

  12. 腹型过敏性紫癜患儿临床及胃镜特点%Abdominal type Henoch purpura in children with clinical and endoscopic characteristics

    Institute of Scientific and Technical Information of China (English)

    蔡洁; 胡波; 桂明

    2013-01-01

    目的 探讨腹型过敏性紫癜患儿的临床特征、胃镜特点及其与幽门螺杆菌的关系,为疾病早期诊断提供依据.方法 回顾性分析460例腹型过敏性紫癜患儿的临床特征,以及其中69例行胃镜检查患儿的胃镜特点、122例幽门螺杆菌感染检测结果.结果 患儿均有腹部疼痛,腹痛部位不固定,症状重而腹部体征较轻,腹痛发生1/2~45 d后皮肤出现紫癜.胃镜检查见胃肠黏膜充血、水肿、出血点、糜烂、溃疡,病变以胃窦、胃体、十二指肠降部及球部为主,但十二指肠黏膜改变最严重.采用黏膜尿素酶试验或免疫印迹法检测幽门螺杆菌阳性54例.结论 腹型过敏性紫癜临床表现无特异性.胃及十二指肠黏膜类似皮肤紫癜样病变是其常见的镜下特点,有早期诊断价值.过敏性紫癜与幽门螺杆菌感染的关系有待进一步研究.%Objective To discuss the clinical features and endoscopic characteristics of abdominal type Henoch purpura in children and its relationship with Helicobacter pylori to provide the basis for early diagnosis. Methods Retrospective analysis of 460 cases of six years of Henoch purpura in children with abdominal clinical features,as well as 69 children affected by endoscopy and endoscopic characteristics of H. pylori infection in routine testing of 122 test results. Results All children experienced abdominal pain,the location was not fixed,and the symptoms were serious,but the signs were light. After the abdominal pain for 1/2 ~45 days,the skin presented the purpura. Gastroscopic features included hyperemia and edema of gastrointestinal mucosa, hemorrhagic spots, erosion and ulceration, lesions mainly in the gastric antrum,body,descending duodenum and bulbar,but the duodenum mucous membrane changed in the most serious. Mucosal urease test or Western blot detection showed H. pylori-positive in 54 cases. Conclusion Abdominal type Henoch purpura presented no specific clinical

  13. The Thinking and Method of TCM in Treating Allergic Purpura%中医药治疗过敏性紫癜的思路与方法

    Institute of Scientific and Technical Information of China (English)

    夏小军; 段赟

    2016-01-01

    The causes and the pathogenesis of allergic purpura were explored from five aspects: innate defi-ciency, invasion of external pathogen, irregular diet, insect bites,Qideficiency failing to control, blood loss,Yin-de-ficiency and fire hyperactivity, fire burning blood vessels, blood stasis obstructing the collaterals, blood failing to stay in the meridians, allergic purpura were divided into four patterns: heat hurting blood vessels,Yin-deficiency and fire hyperactivity,Qifailing to control blood, and treated by selfmade purpura prescriptions series, simultaneously, the patterns were differentiated according to the causes′to clear heat, detoxify, dispel the wind, promote blood circu-lation and eliminate blood stasis, invigorate spleen-stomach.%从禀赋薄弱、感受外邪,饮食不节、昆虫叮咬,气虚不摄、血溢脉外,阴虚火旺、灼伤血络,瘀血阻络、血不归经5方面探讨过敏性紫癜的病因病机,并辨证为热伤血络、阴虚火旺、瘀血阻络、气不摄血4种分型,用自拟紫癜系列方加减治疗,并注重审证求因,清热解毒祛风,并活用活血化瘀方法,同时勿忘健运脾胃。

  14. Autologous Peripheral Blood Stem Cell Transplantation in Patients With Life Threatening Autoimmune Diseases

    Science.gov (United States)

    2005-06-23

    Purpura, Schoenlein-Henoch; Graft Versus Host Disease; Anemia, Hemolytic, Autoimmune; Rheumatoid Arthritis; Churg-Strauss Syndrome; Hypersensitivity Vasculitis; Wegener's Granulomatosis; Systemic Lupus Erythematosus; Giant Cell Arteritis; Pure Red Cell Aplasia; Juvenile Rheumatoid Arthritis; Polyarteritis Nodosa; Autoimmune Thrombocytopenic Purpura; Takayasu Arteritis

  15. Guidelines on the use of intravenous immune globulin for hematologic conditions.

    Science.gov (United States)

    Anderson, David; Ali, Kaiser; Blanchette, Victor; Brouwers, Melissa; Couban, Stephen; Radmoor, Paula; Huebsch, Lothar; Hume, Heather; McLeod, Anne; Meyer, Ralph; Moltzan, Catherine; Nahirniak, Susan; Nantel, Stephen; Pineo, Graham; Rock, Gail

    2007-04-01

    Canada's per capita use of intravenous immune globulin (IVIG) grew by approximately 115% between 1998 and 2006, making Canada one of the world's highest per capita users of IVIG. It is believed that most of this growth is attributable to off-label usage. To help ensure IVIG use is in keeping with an evidence-based approach to the practice of medicine, the National Advisory Committee on Blood and Blood Products of Canada (NAC) and Canadian Blood Services convened a panel of national experts to develop an evidence-based practice guideline on the use of IVIG for hematologic conditions. The mandate of the expert panel was to review evidence regarding use of IVIG for 18 hematologic conditions and formulate recommendations on IVIG use for each. A panel of 13 clinical experts and 1 expert in practice guideline development met to review the evidence and reach consensus on the recommendations for the use of IVIG. The primary sources used by the panel were 3 recent evidence-based reviews. Recommendations were based on interpretation of the available evidence and where evidence was lacking, consensus of expert clinical opinion. A draft of the practice guideline was circulated to hematologists in Canada for feedback. The results of this process were reviewed by the expert panel, and modifications to the draft guideline were made where appropriate. This practice guideline will provide the NAC with a basis for making recommendations to provincial and territorial health ministries regarding IVIG use management. Specific recommendations for routine use of IVIG were made for 7 conditions including acquired red cell aplasia; acquired hypogammaglobulinemia (secondary to malignancy); fetal-neonatal alloimmune thrombocytopenia; hemolytic disease of the newborn; HIV-associated thrombocytopenia; idiopathic thrombocytopenic purpura; and posttransfusion purpura. Intravenous immune globulin was not recommended for use, except under certain life-threatening circumstances, for 8 conditions

  16. 过敏性紫癜患儿血清过敏原检测应用%Application of serum allergen detection for children with allergic purpura

    Institute of Scientific and Technical Information of China (English)

    曾子坤

    2016-01-01

    Objective To investigate clinical significance by serum allergen detection in diagnosis of children with allergic purpura. Methods A total of 120 children with allergic purpura received serum allergen detection of immune globulin E (IgE) antibody for allergen identification. Results The 120 cases had total positive rate of inhaled allergen as 54.17% (65 cases), with proportion as dermatophagoides farina > mould > tree pollen > house dust > hair and scurf of cat and dog > common ragweed artemisia, cockroach, mosquito. Their total positive rate of food allergen was 35.00% (42 cases), with proportion as peanut, soybean > shrimp, crab > pork, beef, mutton > egg > fish > milk. Conclusion Under certain circumstance, allergic purpura can be induced by various allergen. Therefore, both convention therapy and allergen detection are necessary in clinical treatment to identify allergen and restrain occurrence of allergic purpura.%目的:探究血清过敏原检测在过敏性紫癜患儿诊断中的临床意义。方法120例过敏性紫癜患儿,检测其血清中过敏原特异性免疫球蛋白 E(IgE)抗体等,并找出过敏原。结果120例患儿中,其中总吸入过敏原阳性率为54.17%(65例),过敏原占比由高到低排列为:尘粉螨>霉菌>树花粉>屋尘>猫狗毛发皮屑>普通豚草蒿属及蟑螂、蚊子;总食物过敏原阳性率为35.00%(42例),过敏原占比由高到低排列为:花生、大豆>虾、蟹>猪肉、牛肉、羊肉>蛋类>鱼>牛奶。结论多种过敏原在一定条件下均可诱发过敏性紫癜,因此,在临床治疗中既需要进行常规治疗,也应进行过敏原检测,以查找出过敏原,减少紫癜的发生。

  17. Adult Henoch-Schönlein purpura associated with small cell lung cancer: A case report and review of the literature

    OpenAIRE

    Zhang, Xue-De; YANG, SHUAN-YING; Li, Wei; Ming, Zong-Juan; Hou, Yan-Li; Niu, Ze-Qun; Zhang, Yu-ping

    2013-01-01

    The present study reports the case of a 53-year-old male who had been suffering from coughing and the presence of a blood-streaked sputum for >1 month. Chest computed tomography (CT) and a bronchoscopic brush smear were performed. The patient was subsequently diagnosed with small cell lung cancer (limited stage). The patient developed polyarthritis, abdominal pain, diarrhea and a purpuric rash at 14 days post thoracotomy surgery for lung cancer. Henoch-Schönlein purpura (HSP) was diagnosed ba...

  18. Detection of plasmacytoid dendritic cell (pDC) content in peripheral blood and renal tissue of children with henoch-schonlein purpura and its clinical value

    Institute of Scientific and Technical Information of China (English)

    Rong-Mei Xiang

    2016-01-01

    Objective:To study the plasmacytoid dendritic cell (pDC) content in peripheral blood and renal tissue of children with henoch-schonlein purpura and its clinical value.Methods:30 cases of henoch-schonlein purpura children with renal damage were enrolled in HSPN group, 30 cases of henoch-schonlein purpura children without renal damage were enrolled in NHSPN group, and 30 cases of healthy volunteers were enrolled in the control group. Then contents of pDC, Th2 cell, IL-4, IL-5, IL-10 and IL-13 in peripheral blood as well as contents of pDC, Th17 cell, IL-17, IL-21 and IL-23 in renal tissue of three groups were detected.Results: (1) pDC contents in peripheral blood of HSPN group and NHSPN group were lower than those of control group and the decrease of pDC contents in peripheral blood of HSPN group was more obvious; CD304 contents in renal tissue of HSPN group and NHSPN group were higher than those of control group and the increase of CD304 contents in renal tissue of HSPN group was more obvious; (2) Th2 cell as well as IL-4, IL-5, IL-10 and IL-13 contents in peripheral blood of HSPN group and NHSPN group were higher than those of control group and the increase of related indexes in peripheral blood of HSPN group was more obvious; Th17 cell as well as IL-17, IL-21 and IL-23 contents in kidney tissue of HSPN group were higher than those of NHSPN group; (3) in peripheral blood, pDC content was negatively correlated with Th2 cell level as well as IL-4, IL-5, IL-10 and IL-13 contents, and in renal tissue, pDC content was positively correlated with Th17 cell level as well as IL-17, IL-21 and IL-23 contents. Conclusions:Abnormal pDC content correlates with the pathogenesis of henoch-schonlein purpura, pDC content decreases in peripheral blood and will result in enhancement of Th2 cell function, and pDC content increases in kidney and will result in enhancement of Th17 cell function.

  19. Clinicopathological features and prognosis of membranoproliferative-like Henoch-Schönlein purpura nephritis in children

    Institute of Scientific and Technical Information of China (English)

    Yan-Jie Huang; Ying Ding; Xiao-Qing Yang; Wen-Sheng Zhai; Xian-Qing Ren; Qing-Yin Guo; Xia Zhang; Meng Yang; Tatsuo Yamamoto; Yuan Sun

    2015-01-01

    Background: The aim of this retrospective study was to defi ne the clinical manifestations, pathological features and prognosis of children with membranoproliferative-like Henoch-Schönlein purpura nephritis (HSPN), representing International Study of Kidney Disease in Children (ISKDC) grade VI. Methods: Among 245 patients with HSPN treated in our hospital between 2008 and 2010, nine patients (3.7%) were diagnosed with HSPN of ISKDC grade VI (males=5, females=4, age: 9.5±2.03 years, mean±SD). The clinical features, laboratory and pathologicalfi ndings, treatment and outcome of the 9 patients were retrospectively analyzed. Results: Of the 9 patients, 7 (78%) presented with hematuria and nephrotic syndrome, and were treated with steroids (oral prednisone or intravenous methylprednisolone pulse therapy) and immunosuppressants (oral tripterygium glycosides or intravenous cyclophosphamide pulse therapy). One (11%) patient had hematuria and nephrotic range proteinuria (>50 mg/kg per 24 hours) and was treated with oral prednisone and tripterygium glycosides. Another (11%) patient presented with hematuria and moderate proteinuria (25-50 mg/kg per 24 hours) and was treated with oral tripterygium glycoside only. Histopathological examination showed diffuse glomerular mesangial and endocapillary proliferation, mesangial interposition, double-contour formation, podocyte hypertrophy, shedding, and cytoplasmic absorption droplets. The percentages of glomeruli with small cellular crescents varied from 4%-25% in 6 of 9 patients. Follow-up for 2 to 4 years showed excellent recovery in all patients. Conclusions: The main clinical feature of ISKDC grade VI HSPN in children is a nephrotic syndrome with hematuria. The excellent prognosis of the disease was probably related to early diagnosis and treatment with steroids and/or immunosuppressants, and mild degree of glomerulosclerosis and tubulointerstitial damage.

  20. Kampo medicines improved blood test and QOL in two vasculitis cases of Churg–Strauss syndrome and Henoch–Shönlein purpura after inadequate treatment with conventional therapies

    Directory of Open Access Journals (Sweden)

    Yasuyo Hijikata

    2010-02-01

    Full Text Available Yasuyo Hijikata1, Yumiko Ikemoto2, Urara Kohdera31Toyodo Hijikata Clinic, Osaka, Japan; 2St Mary’s Hospital, Himeji, Japan; 3Nakano Children’s Hospital, Osaka, JapanObjective: Based on the tenets of traditional Chinese medicine (TCM theory, Kampo medicines were selected and applied to two cases of Churg–Strauss syndrome and Henoch–Shönlein purpura. Two vasculitis syndrome patients exhibited persistent symptoms and abnormal blood tests after treatment with conventional therapies.Methods: As the two cases had “blood stagnation” and “damps and heat” and one had a “yang deficiency” in terms of TCM theory, we applied certain selected Kampo medicines.Results: In case 1, the patient presented with hypereosinophilia, venous thrombosis, pulmonary infarction, decreased platelet count, ulner nerve palsy and Raynaud’s phenomena, which led to a diminished quality of life. After starting the Kampo medicines, the patient improved quickly and recovered within 11 months. In case 2, persistent purpura, abdominal pain, and bloody feces quickly improved and disappeared after Kampo treatment. After starting the Kampo medicines, prednisolone was stopped at 21 days without any sign of relapse to date.Conclusion: Kampo medicines helped clear the persistent abnormal symptoms and laboratory findings of vasculitis syndromes, Churg–Strauss syndrome and Henoch–Shönlein purpura, which had responded inadequately to the conventional therapies administered.Keywords: Kampo medicine, vasculitis, Churg–Strauss syndrome, Henoch–Shönlein purpura

  1. Investigations of the capacity of synthesizing 3β-sterols in mollusca—VI. The biosynthesis and composition of 3β-sterols in the neogastropods Purpura lapillus and Murex brandaris

    NARCIS (Netherlands)

    Voogt, P.A.

    1972-01-01

    1. 1. It is shown that Purpura lapillus and Murex brandaris are capable of synthesizing sterols from mevalonate and acetate. The biosynthesis of sterols from acetate proceeds only at a slow rate. This phenomenon is compared with that observed in Natica cataena. 2. 2. The composition of the sterol m

  2. EULAR/PRINTO/PRES criteria for Henoch-Schönlein purpura, childhood polyarteritis nodosa, childhood Wegener granulomatosis and childhood Takayasu arteritis: Ankara 2008. Part I: Overall methodology and clinical characterisation

    DEFF Research Database (Denmark)

    Ruperto, Nicolino; Ozen, Seza; Pistorio, Angela;

    2010-01-01

    To report methodology and overall clinical, laboratory and radiographic characteristics for Henoch-Schönlein purpura (HSP), childhood polyarteritis nodosa (c-PAN), c-Wegener granulomatosis (c-WG) and c-Takayasu arteritis (c-TA) classification criteria....

  3. Hematologic complications of pregnancy.

    Science.gov (United States)

    Townsley, Danielle M

    2013-07-01

    Pregnancy induces a number of physiologic changes that affect the hematologic indices, either directly or indirectly. Recognizing and treating hematologic disorders that occur during pregnancy is difficult owing to the paucity of evidence available to guide consultants. This review discusses specifically the diagnosis and management of benign hematologic disorders occurring during pregnancy. Anemia secondary to iron deficiency is the most frequent hematologic complication and is easily treated with oral iron formulations; however, care must be taken not to miss other causes of anemia, such as sickle cell disease. Thrombocytopenia is also a common reason for consulting the hematologist, and distinguishing gestational thrombocytopenia from immune thrombocytopenia (ITP), preeclampsia, HELLP syndrome (hemolysis, elevated liver enzymes, and low platelets), or thrombotic thrombocytopenic purpura (TTP) is essential since the treatment differs widely. Occasionally the management of mother and infant involves the expeditious recognition of neonatal alloimmune thrombocytopenia (NAIT), a condition that is responsible for severe life-threatening bleeding of the newborn. Additionally, inherited and acquired bleeding disorders affect pregnant women disproportionately and often require careful monitoring of coagulation parameters to prevent bleeding in the puerperium. Finally, venous thromboembolism (VTE) during pregnancy is still largely responsible for mortality during pregnancy, and the diagnosis, treatment options and guidelines for prevention of VTE during pregnancy are explored. PMID:23953339

  4. EB病毒感染伴特发性血小板减少性紫癜患儿细胞免疫功能研究%The investigation of lymphocyte subsets in Epstein-Barr virus-related idiopathic thrombocytopenic purpura

    Institute of Scientific and Technical Information of China (English)

    田厚荣; 王文娟; 祝绚

    2013-01-01

    目的 观察EB病毒感染伴特发性血小板减少性紫癜(ITP)患儿外周血淋巴细胞亚群变化特点,探讨其与ITP的关系及临床意义.方法 采用流式细胞技术检测不伴EB病毒感染的ITP患儿、健康体检儿童的各淋巴细胞亚群的水平.结果 ITP伴EB病毒感染组(ITP-EBV组)与ITP非EB病毒感染组(ITP-N组)、对照组比较,CD3+T细胞、CD4+/CD8+比值、NK细胞减少,CD3+CD8+T细胞、CD19+B细胞增加(P<0.05).结论 ITP伴EB病毒感染存在T淋巴细胞免疫功能异常,应同时进行抗病毒和免疫治疗.

  5. Study on changes and clinical significance of lymphocyte subtypes in children with idiopathic thrombocytopenic purpura%儿童特发性血小板减少性紫癜患者外周血T淋巴细胞亚群变化

    Institute of Scientific and Technical Information of China (English)

    陈叶龙; 寿黎红

    2012-01-01

    目的 研究特发性血小板减少性紫癜(ITP)患者T淋巴细胞亚群的变化,了解其在ITP发病机制中的作用.方法 以30例ITP确诊患者为观察组,其中慢性ITP(CITP)组18例,急性ITP(AITP)组12例,以20例体检健康者为对照组,应用流式细胞术检测受试对象外周血T淋巴细胞亚群,并用统计学分析结果.结果 ITP组CD3+、CD4+细胞百分比及CD4+/CD8+比值比正常对照组明显减低(P<0.05),CD8+细胞百分比比正常对照组明显升高(P<0.05),CITP组变化幅度大于AITP组(P<0.05).结论 ITP患儿存在细胞免疫功能紊乱,可能与ITP免疫发病机制有关.

  6. IFN-γ+874A/T polymorphism and its risk for immune thrombocytopenic purpura%IFN-γ+874A/T位点多态性与免疫性血小板减少症患者发病的相关性研究

    Institute of Scientific and Technical Information of China (English)

    徐建芬; 赵海丰

    2013-01-01

    目的 探讨IFN-γ+874A/T位点单核苷酸多态性与免疫性血小板减少症(ITP)患者发病的相关性.方法 采用序列特异性引物聚合酶链反应(PCR-SSP)对100例ITP患者和100例健康(对照组)进行IFN-γ+874A/T基因定型.结果 当ITP患者作为一个整体,IFN-γ+874A/T与ITP患者发病风险没有相关性.根据年龄,将ITP患者分为儿童ITP和成人ITP,分别与对照组比较,该基因位点的基因型和等位基因频率差异无统计学意义.根据年龄及病程,将ITP患者分为急性儿童ITP,慢性儿童ITP,急性成人ITP和慢性成人ITP,分别与对照组比较,该基因位点的基因型和等位基因频率差异无统计学意义.结论 IFN-γ+874A/T基因多态性没有参与ITP的易感性.

  7. 伴紫癜、抽搐和甲硫氨酸血症的citrin缺陷病患儿临床表型与基因突变分析%Clinical investigation and mutation analysis of a child with citrin deficiency complicated with purpura,convulsive seizures and methioninemia

    Institute of Scientific and Technical Information of China (English)

    温鹏强; 王国兵; 陈占玲; 刘晓红; 崔冬; 赏月; 李成荣

    2013-01-01

    Objective To analyze the clinical features and SLC25A13 gene mutations of a child with citrin deficiency complicated with purpura,convulsive seizures and methioninemia.Methods The patient was subjected to physical examination and routine laboratory tests.Blood amino acids and acylcarnitines,and urine organic acids and galactose were analyzed respectively with tandem mass spectrometry and gas chromatographic mass spectrometry.SLC25A13 gene mutation screening was conducted by high resolution melt (HRM) analysis.Results The petechiae on the patient's face and platelet count (27 × 109/L,reference range 100 × 109/L 300 × 109/L) supported the diagnosis of immunologic thrombocytopenic purpura (ITP).Laboratory tests found that the patient have abnormal coagulation,cardiac enzyme,liver function and liver enzymes dysfunction.Tandem mass spectrometry also found methionine to be increased (286 μmol/L,reference ranges 8 μmol/L 35 μmol/L).The patient did not manifest any galactosemia,citrullinemia and tyrosinemia.Analysis of SLC25A13 gene mutation found that the patient has carried IVS16ins3kb,in addition with abnormal HRM result for exon 6.Direct sequencing of exon 6 revealed a novel mutation c.495delA.The same mutation was not detected in 100 unrelated healthy controls.Further analysis of her family has confirmed that the c.495delA mutation has derived from her farther,and that the IVS16ins3kb was derived from her mother.Conclusion The clinical features and metabolic spectrum of citrin deficiency can be variable.The poor prognosis and severity of clinical symptoms of the patient may be attributed to the novel c.495delA mutation.%目的 分析1例伴紫癜、抽搐和甲硫氨酸血症为主要表现的citrin缺陷病患儿的临床特征,并探讨其SLC25A13基因突变类型.方法 对患儿进行体格与一般实验室项目检查;应用串联质谱分析血氨基酸和肉碱浓度,气相色谱质谱法检测尿有机酸及半乳糖含量;应用高分辨率熔

  8. The interaction between circulating complement proteins and cutaneous microvascular endothelial cells in the development of childhood Henoch-Schonlein Purpura.

    Directory of Open Access Journals (Sweden)

    Yao-Hsu Yang

    Full Text Available In addition to IgA, the deposition of complement (C3 in dermal vessels is commonly found in Henoch-Schönlein purpura (HSP. The aim of this study is to elucidate the role of circulating complement proteins in the pathogenesis of childhood HSP.Plasma levels of C3a, C4a, C5a, and Bb in 30 HSP patients and 30 healthy controls were detected by enzyme-linked immunosorbent assay (ELISA. The expression of C3a receptor (C3aR, C5a receptor (CD88, E-selectin, intercellular adhesion molecule 1 (ICAM-1, C3, C5, interleukin (IL-8, monocyte chemotactic protein (MCP-1, and RANTES by human dermal microvascular endothelial cells (HMVEC-d was evaluated either by flow cytometry or by ELISA.At the acute stage, HSP patients had higher plasma levels of C3a (359.5 ± 115.3 vs. 183.3 ± 94.1 ng/ml, p < 0.0001, C5a (181.4 ± 86.1 vs. 33.7 ± 26.3 ng/ml, p < 0.0001, and Bb (3.7 ± 2.6 vs. 1.0 ± 0.6 μg/ml, p < 0.0001, but not C4a than healthy controls. Although HSP patient-derived acute phase plasma did not alter the presentation of C3aR and CD88 on HMVEC-d, it enhanced the production of endothelial C3 and C5. Moreover, C5a was shown in vitro to up-regulate the expression of IL-8, MCP-1, E-selectin, and ICAM-1 by HMVEC-d with a dose-dependent manner.In HSP, the activation of the complement system in part through the alternative pathway may have resulted in increased plasma levels of C3a and C5a, which, especially C5a, may play a role in the disease pathogenesis by activating endothelium of cutaneous small vessels.

  9. Research of complement activity in children with anaphylactoid purpura%儿童过敏性紫癜补体活性研究

    Institute of Scientific and Technical Information of China (English)

    肖珊珊; 刘丽晓; 张佳慧; 范慧子

    2012-01-01

    Objective To investigate the complement C3, C4 activity in children with anaphylactoid purpura of acute and convalescent phase. Methods 97 children with anaphylactoid purpura treated in our hospital from October 2009 to November 2011 were selected as research objects. According to the clinical manifestation, all patients were divided into the skin-type group, abdominal-type group, kidney-type group, joint-type group and mixed-type group. According to the progression disease, patients were divided into the acute phase and the convalescent phase. At the same time, the complement levels were compared with that 32 cases of healthy children, who were taken as the control group. Then the children' serum C3, C4 level were detected by immune scattering rate turbidimetric method using Array 360 scattering immune turbidimetric instrument produced in Beckman-coulter company. Results In the acute and convalescent phase of anaphylactoid purpura children, the serum C3 and C4 levels among different clinical type groups had no difference (P > 0.05). The serum C4 level in acute, convalescent phase groups and control group had no statistically significant difference (P > 0.05); but compared with the control group, the C3 level in acute phase reduced (P 0.05);急性期、恢复期与对照组相比,血清补体C4水平差异无统计学意义(P > 0.05);与对照组相比,急性期血清补体C3水平降低(P < 0.05),恢复期C3水平相对急性期升高,但仍较对照组低(P < 0.05).结论 不同临床分型过敏性紫癜患儿其血清补体水平无明显差异;而相对于健康患儿其急性期补体C3水平降低,恢复期相对回升,而血清补体C4水平无变化.

  10. A case of neonatal alloimmune thrombocytopenia in the presence of both anti-HPA-4b and anti-HPA-5b antibody: clinical and serological analysis of the subsequent pregnancy.

    Science.gov (United States)

    Kiyokawa, Tomoko; Koh, Yangsook; Mimura, Kazuya; Nakayama, Kotarosumitomo; Hosokawa, Mika; Sakuragi, Mikiko; Morikawa, Tamayo; Nakao, Mayumi; Aochi, Hiroshi; Fukumori, Yasuo; Kanagawa, Takeshi; Nagamine, Keisuke; Kimura, Tadashi; Tomiyama, Yoshiaki

    2014-10-01

    Neonatal alloimmune thrombocytopenia (NAIT) is induced by maternal alloantibodies raised against fetal platelet antigens inherited from the paternal parent. In contrast to Caucasians, in Asians, predominantly in Japanese, most frequently detected antibodies in NAIT are anti-HPA-4b and anti-HPA-5b. In some NAIT cases multiple alloantibodies are detected. In such cases it is very difficult to determine which antibody is the dominant antibody in NAIT. In this case report, we describe a NAIT case (first sibling) with severe thrombocytopenia and cephalhematoma in the presence of both anti-HPA-4b and anti-HPA-5b antibodies in the maternal serum. We carefully examined titers of anti-HPA antibodies during the subsequent pregnancy with HPA-4b-positive and HPA-5b-negative fetus determined by amniocentesis at gestational week 16. We administered IVIG (1 g/kg/w) to the mother from gestational week 32 to 35. The mother subsequently delivered a second sibling with normal platelet count by cesarean section. Although we could not completely rule out the involvement of anti-HPA-4b, our findings suggested that anti-HPA-5b was implicated in the NAIT in the first sibling.

  11. 过敏性紫癜的非典型临床表现(综述)%The atypical clinical manifestations in Henoch-Schonlein purpura

    Institute of Scientific and Technical Information of China (English)

    汪晓红; 季必华

    2014-01-01

    过敏性紫癜除典型的临床表现外,还可因累及神经、心脏、肺脏、胰腺、肝脏、胆囊、生殖器等器官出现一些非典型的临床表现。该文对此作一综述。%The majority of patients with Henoch-Schonlein purpura show the classic clinical manifestations,but a small number can appear atypical clinical manifestations,when their other organs are involved,such as nervous sys-tem,heart,lungs,pancreas,hepar,gallbladder,genital organ.This review summarizes that.

  12. Progressive Pigmentary Purpura

    Science.gov (United States)

    ... Derm App Skin Facts Aging and Sun Damage Beauty Myths Preventing Sun Damage Skin Cancer Detection Skin ... patient is unique. PPP usually is only a cosmetic problem, but some people has severe itching, PPP ...

  13. 成人腹型过敏性紫癜临床表现及内镜特点分析26例%Clinical manifestations and endoscopic characteristics of adult abdominal type allergic purpura: An analysis of 26 cases

    Institute of Scientific and Technical Information of China (English)

    刘立玺; 朱方擎; 林浩; 文萍; 文剑波

    2013-01-01

    AIM:To summarize the clinical and endoscopic features of abdominal type allergic purpura in adult patients.METHODS:Clinical and endoscopic data for 26adult patients with abdominal type allergic purpura were analyzed retrospectively.RESULTS:All patients had abdominal pain,and 13 patients had digestive tract hemorrhage.Endoscopy revealed hyperaemia,edema,bleeding spots,erosion and ulcer in the gastrointestinal mucosa.Severe mucosa] lesions were often found in the duodenum,ileum and caecum.CONCLUSION:Gastrointestinal endoscopy is helpful for early diagnosis of adult abdominal type allergic purpura.%目的:总结成人腹型过敏性紫癜的临床特点、内镜下改变,为该病早期诊断提供依据.方法:回顾性分析2000-01/2012-12我院收治的26例腹型过敏性紫癜的临床表现及内镜资料.结果:26例患者均有腹痛,消化道出血13例,内镜检查可见胃肠黏膜充血水肿、出血点、糜烂、溃疡,以十二指肠、回肠末段为主.结论:胃肠镜有助于腹型过敏性紫癜的早期诊断.

  14. Alloimmunization screening after transfusion of red blood cells in a prospective study Pesquisa de aloimunização após transfusão de concentrados de hemácias em um estudo prospectivo

    Directory of Open Access Journals (Sweden)

    Vitor Mendonça Alves

    2012-01-01

    Full Text Available BACKGROUND: Several irregular red blood cell alloantibodies, produced by alloimmunization of antigens in transfusions or pregnancies, have clinical importance because they cause hemolysis in the fetus and newborn and in transfused patients. OBJECTIVE: a prospective analysis of patients treated by the surgical and clinical emergency services of Hospital de Clínicas of the Universidade Federal do Triângulo Mineiro (HC/UFTM, Brazil was performed to correlate alloimmunization to clinical and epidemiological data. METHODS: Blood samples of 143 patients with initial negative antibody screening were collected at intervals for up to 15 months after the transfusion of packed red blood cells. Samples were submitted to irregular antibody testing and, when positive, to the identification and serial titration of alloantibodies. The Fisher Exact test and Odds Ratio were employed to compare proportions. RESULTS: Fifteen (10.49% patients produced antibodies within six months of transfusion. However, for 60% of these individuals, the titers decreased and disappeared by 15 months after transfusion. Anti-K antibodies and alloantibodies against antigens of the Rh system were the most common; the highest titer was 1:32 (anti-K. There was an evident correlation with the number of transfusions. CONCLUSIONS: Given the high incidence of clinically important red blood cell alloantibodies in patients transfused in surgical and clinical emergency services, we suggest that phenotyping and pre-transfusion compatibilization for C, c, E, e (Rh system and K (Kell system antigens should be extended to all patients with programmed surgeries or acute clinical events that do not need emergency transfusions.OBJETIVO: Vários aloanticorpos irregulares antieritrocitários, produzidos por sensibilizações a antígenos estranhos durante gestações ou transfusões, possuem importância clínica por provocarem hemólise no feto ou recém-nascido e/ou no receptor de sangue. Diante

  15. Progress in treatment of pediatric Henoch-Schönlein purpura nephritis%儿童紫癜性肾炎治疗进展

    Institute of Scientific and Technical Information of China (English)

    叶乐珍; 周江瑾(综述)

    2014-01-01

    Henoch-Schönlein purpura nephritis (HSPN) is the most common secondary glomerular disease in children. The clinical manifestations of HSPN vary from microscopic hematuria, microalbuminuria to renal dysfunction even end stage renal disease which is needed to rely on long-term renal replacement therapy, thus affecting the quality of children life seriously. In recent years, the incidence of Henoch-Schönlein purpura continues to increase, which should be paid more attention. There are many studies about the treatment of HSPN both at home and abroad, yet no certain conclusion is drawn because of the inconsistent results. We advocate stepped therapies, that is, the appropriate regimens are chosen based on the clinical manifestations and renal pathology in children. For severe HSPN, we recommend multi-drug intensive therapy combined with other methods such as plasma exchange to alleviate the symptoms.%紫癜性肾炎是最常见的儿童继发性肾小球疾病。其临床表现不一,轻者仅表现为镜下血尿、微量蛋白尿,重者可逐渐进展为肾功能不全,直至终末期肾脏病而需要依靠长期肾脏替代治疗,严重影响患儿生存质量。近年来过敏性紫癜发生率不断提高,需予以重视。关于紫癜性肾炎的治疗,目前国内外有较多的研究,但结果不尽一致,因此至今未有定论。我们提倡阶梯式疗法,即根据患儿临床表现、肾脏病理选择合适的方案,而对于重症紫癜性肾炎,我们推荐多药强化治疗并联合血浆置换等方法以缓解病情。

  16. Physiopathology of blood platelets and development of platelets substitutes. Progress report, August 1, 1976--October 31, 1977. [/sup 51/Cr

    Energy Technology Data Exchange (ETDEWEB)

    Baldini, M G

    1977-07-31

    Progress is reported on the following research projects: the effect of estrogen on platelet aggregability and thrombus formation; the antithrombotic effect of platelet inhibiting agents in a bench model of artificial kidney; the arrest of hemorrhage in severely alloimmunized thrombocytopenic patients; and in vivo elution of /sup 51/Cr from labeled platelets induced by antibody. (HLW)

  17. Analysis of Clinical Laboratory of Peripheral Blood IL-16,IL-18 Level in Children with Henoch Schonlein Purpura%过敏性紫癜患儿外周血IL-16、IL-18水平的临床实验分析

    Institute of Scientific and Technical Information of China (English)

    许亚红; 张金秀; 郭特鎏; 何业结

    2015-01-01

    Objective To investigate the application value of IL-16,IL-18 in children with Henoch Schonlein purpura. Methods Analysis of 50 cases of al ergic purpura in children with serum IL-16,IL-18 levels by solid phase enzyme immunoassay. Results Henoch Schonlein purpura in acute phase serum IL-16,in IL-18 patients than in the healthy control group significantly increased ( O.05),al ergic purpura Hui son IL-16 was positively cor elated with IL-18.Conclusion IL-16,IL-18 may be involved in the pathogenesis of vasculitis al ergic purpura,there is some relationship between the detection of IL-16,IL-18 and al ergic purpura disease development.%目的探讨IL-16、IL-18在过敏性紫癜患儿中的应用价值。方法采用固相酶联免疫分析法检测50例过敏性紫癜患儿血清IL-16、IL-18水平。结果过敏性紫癜急性期患儿血清IL-16、IL-18较健康对照组明显升高(0.05),过敏性紫癜患儿IL-16与IL-18呈正相关性。结论 IL-16、IL-18可能参与过敏性紫癜血管炎的发病,IL-16、IL-18的检测与过敏性紫癜病情发展有一定的关系。

  18. Evaluation of the diagnostic performance of platelet-derived indices for the differential diagnosis of thrombocytopenia in pediatrics

    Directory of Open Access Journals (Sweden)

    Nelson Hernando Aponte-Barrios

    2014-10-01

    Full Text Available Background. Platelet-derived indices have a well-established correlation with the differential diagnosis of thrombocytopenia in adult-based research. These indices include mean platelet volume, platelet distribution width, and platelet-large cell ratio. Objective. To determine the values of platelet-derived indices in a pediatric population with diagnoses of thrombocytopenia and their etiologic correlation. Materials and methods. Analytic observational diagnostic-test study. The population for this analytical study was pediatric patients between 6 months and 18 years of age who had thrombocytopenia (<100x10(9/L. The study period was 18 months long. Results. Of 54 subjects, 18 (33.3% were diagnosed with idiopathic thrombocytopenic purpura, and 36 (66.7% were diagnosed with acute leukemia. Mean age was 7.4 years and 6.8 years for immune thrombocytopenic purpura and acute leukemia, respectively. Mean platelet distribution width values for immune thrombocytopenic purpura and acute leukemia were 15.08 fL and 10.73, respectively. Mean MPV for immune thrombocytopenic purpura and acute leukemia was 11.7 fL and 9.8 fL, respectively. Mean platelet-large cell ratio was 38.26% and 24.97% for idiopathic thrombocytopenic purpura and acute leukemia, respectively. Differences in these three distinct platelet indices between idiopathic thrombocytopenic purpura and acute leukemia were statistically significant (p=0.00. The area under the ROC curve for platelet-derived indices showed that they were adequate for defining the causes of thrombocytopenia. MPV and platelet-large cell ratio had an area under the curve of 0.89 and 0.88, respectively, while platelet size deviation width had an area under the curve of 0.903. Conclusions. Platelet-derived indices could be useful in the initial approach for the differential diagnosis of pediatric patients with thrombocytopenia.

  19. Intracranial Hemorrhage as a Source of Headache in a Patient with Postpartum

    OpenAIRE

    Jamshid Vafaeimanesh; Ali Mehrafshan; Azam Nazeri; Fatemeh Hosseinzadeh

    2014-01-01

    Background: Postpartum thrombotic thrombocytopenic purpura (TTP) should be considered in thrombocytopenic patients. Case report: The patient was a 28-year-old pregnant woman referred for cesarean section. After CS, hysterectomy was done due to uncontrolled vaginal bleeding. Peripheral blood smear confirmed the TTP diagnosis. Plasmapheresis was initiated and platelets were infused. Six hours after infusion, tonic-colonic seizure, left hemiplegia and bell's palsy appeared. Bra...

  20. 2) 当院における特発性血小板減少性紫斑病(ITP)の治療成績と長期予後(新潟血栓止血研究会10周年記念特別例会記録)

    OpenAIRE

    高井, 和江; 真田, 雅好; Takai, Kazue; Sanada, Masayoshi

    1991-01-01

    We report the results of treatments and prognosis for idiopathic thrombocytopenic purpura (ITP) of adults in our hospital. Mild thrombocytopenic patients could be observed with no therapy and no risk of bleeding. Fifty cases were treated with corticosteroid, but 16 cases of them were splenectomized and 4 cases were administered immunosuppressants because of inadequate effect of steroid. Steroid were effective in 23 cases (46%) of 50 cases finally. Splenectomy was performed in 19 cases and it ...

  1. Çocukluk dönemi akut idyopatik immün otoimmün trombositopenik purpurasmın insan parvovirus B19 virüsü ile ilişkisi

    OpenAIRE

    Ahmet KARATAŞ; Kadıoğlu, İsmail; Güray, Atilla; Şalcıoğlu, Zeki; Öztürk, Haydar

    2001-01-01

    Immunologic factors play the most important part in idiopathic thrombocytopenic purpura Viral agents also cause the disease by this way Human parvovirus B19 has been shown to play a part as a viral etiologic agent in thrombocytopenia In the present study 29 idiopathic thrombocytopenic purpura patients were examined Parvovirus B1 9 IgM was present in 4 of 29 patients 13 7 and parvovirus B19 IgG positivity in 13 44 8 In the control group parvovirus B19 IgG positivity was 43 In 4 patients with p...

  2. Association between RAS Gene Polymorphisms (ACE I/D, AGT M235T and Henoch-Schönlein Purpura in a Turkish Population

    Directory of Open Access Journals (Sweden)

    Sinem Nalbantoglu

    2013-01-01

    Full Text Available Henoch-Schönlein purpura (HSP is a small-vessel vasculitis of autoimmune hypersensitivity, and renin-angiotensin system (RAS regulates vascular homeostasis and inflammation with activation of cytokine release. Thus, we aimed to investigate the association between HSP and ACE I/D and AGT M235T polymorphisms. Genotyping was determined by allele specific PCR and PCR-RFLP. We obtained a significant difference in genotype distribution (p = 0.003 and allele frequencies (p 0.05 and allele frequencies (p > 0.05 of the AGT M235T polymorphism. Risk assessment showed significant risk for HSP in the subjects both with the ID + DD genotype (p = 0.019, OR: 2.288, 95% CI: 1.136–4.609 and D allele (OR: D vs. I: 2.0528, 95% CI: 1.3632–3.0912, p = 0.001 while no significant risk was obtained for HSP in the subjects both with the MT + TT genotype (p = 0.312, OR: 1.3905, 95% CI: 0.7326–2.6391 and T allele (OR: T vs. M: 1.065, 95% CI: 0.729–1.557, p = 0.743. Furthermore, when patients were stratified by the presence of certain systemic complications of HSP, no significant association was detected with ACE I/D, and AGT M235T polymorphisms. Our findings suggest that ACE I/D polymorphism is significantly associated with HSP susceptibility.

  3. Acupoint implantable drug-line treatment for allergic purpura%穴位埋植药线治疗过敏性紫癜效果观察

    Institute of Scientific and Technical Information of China (English)

    郑丽丽; 马卫东; 施文洁; 马萍萍

    2014-01-01

    目的:观察穴位埋植药线治疗过敏性紫癜的临床疗效。方法:将过敏性紫癜患儿200例随机分为观察组和对照组各100例。对照组采用传统治疗方法即对症治疗+肾上腺糖皮质激素应用,观察组采用双侧足三穴位埋植药线的方法。即患儿取坐位或仰卧位,在常规消毒和局麻下,用无菌手术缝合针引线,一侧足三里穴埋植卡介菌多糖浸泡的肠线,另一侧埋植核酸注射液浸泡肠线。双侧穴位埋植药线的深度均要达肌层,然后将露出表皮的线头用手术剪剪除,在用无菌敷料包扎固定。7d治疗1次,2次为1个疗程,连续治疗3个疗程。上述两组患者均治疗40d后评定整体疗效及典型症状消失的时间。数据进行统计学处理(x2检验,t检验)。结果:两组患者整体疗效比较,提示观察组优于对照组(P<0.01),具有非常显著性差异。其典型症状如皮肤紫癜、消化道症状、关节症状、肾损害症状比较,也具有非常显著性差异(P<0.01),观察组所用的时间均较对照组短。结论:采用足三里穴位埋植药线治疗过敏性紫癜效果好。%Objective:To observe the clinical efficacy of the the acupoint implants the drug line for allergic purpura. Methods:200 children were randomly divided into observation group and control group of 100 patients, control group took traditional treatment methods that symptomatic treatment +glucocorticoid. The observation group was treated with bilateral foot three acupuncture points implantable drug line. Children take sitting or supine position, routine disinfection and under local anesthesia, sterile surgical suture needle leads the side of the foot the the Zusanli implants card BCG polysaccharide soaked catgut, the other side of the implant nucleic acid injection soak catgut. The bilateral acupoint implantable drug line depth of myometrial, then the exposed epidermis thread cut off with

  4. Total lymphoid irradiation in alloimmunity and autoimmunity

    Energy Technology Data Exchange (ETDEWEB)

    Strober, S.

    1987-12-01

    Total lymphoid irradiation has been used as an immunosuppressive regimen in autoimmune disease and organ transplantation. The rationale for its use originated from studies of patients with Hodgkin disease, in whom this radiotherapy regimen was noted to induce profound and long-lasting immune suppression and yet was well tolerated, with few long-term side effects. Total lymphoid irradiation is a unique immunosuppressive regimen that produces a selective (and long-lasting) reduction in the number and function of helper T cells and certain subsets of B cells. Conventional immunosuppressive drugs show little selectivity, and their effects are short-lived. The most important aspect of total lymphoid irradiation is the potential for achieving transplantation tolerance and permanent remissions in autoimmune disease in laboratory animals. Attempts are being made to achieve similar goals in humans given total lymphoid irradiation, so that immunosuppressive drugs can be ultimately withdrawn from transplant recipients and patients with lupus nephritis. 28 references.

  5. Rituximab in the treatment of patients with systemic Iupus erythematosus-associated thrombotic thrombocytopenic purpura with normaI ADAMTS-13 activity:three cases and Iiterature review%利妥昔单抗治疗ADAMTS-13活性正常系统性红斑狼疮相关血栓性血小板减少性紫癜三例并文献复习

    Institute of Scientific and Technical Information of China (English)

    李嗣钊; 卢昕; 周丽静; 陈亚宁; 尚健; 姚合斌

    2016-01-01

    biopsy which revealed typeⅡ lupus nephritis (LN) and typeⅣ andⅤ LN with renal thrombotic microangiopathy respectively. All 3 patients received methylprednisolone pulse and intravenous immunoglobulin or in combination with mycophenolate mofetil therapy before SLE-associated TTP was diagnosed, however they got worse. As the diagnosis was considered, 2 patients received plasma exchange (PE) combined with glucocorticoids and/or mycophenolate mofetil (MMF), but there were still no improvement. Then 3 patients were rescued by RTX therapy for 2 to 4 episode, after that they achieved complete remission. With mean follow-up of 14 months they showed a sustained remission. 1 patient died from severe pneumonia at 6 months of follow-up. From literature review, a total of 6 cases including our cas suffered from SLE-associated TTP with normal ADAMTS-13 activity, some of them were treated by glucocorticoids combined with PE and/or immunosuppressive failure, then switched to RTX therapy, some of them received RTX in combination with glucocorticoids and PE as an initial treatment, they all got complete remission.ConcIusion SLE-associated TTP should be considered when SLE complicated with fever, anemia accompanied with thrombocytopenia and high LDH level. Increased peripheral broken red cells count and renal biopsy are necessary for the diagnosis of this disease. Patients with SLE-associated TTP with normal ADAMTS-13 activity usually perform high disease activity, severe nervous system and kidney damage. RTX combined with glucocorticoids and/or PE and immunosuppressive may significantly improve effectiveness of treatment and the prognosis of these patients.%目的:探讨利妥昔单抗(RTX)治疗血管性血友病因子裂解蛋白酶(ADAMTS-13)活性正常的系统性红斑狼疮(SLE)相关血栓性血小板减少性紫癜(TTP)的疗效。方法分析中国人民解放军海军总医院内分泌风湿免疫科先后应用 RTX 治疗3例ADAMTS-13活性正常的SLE相关 TTP 患者的临床特征,随访患者治疗后的转归,并总结相关文献。结果3例 SLE 相关 TTP患者均有不同程度的发热、血小板减少和微血管病性溶血性贫血,伴神经系统和肾损害,血乳酸脱氢酶(LDH)明显升高,外周血涂片可见破碎红细胞,而 ADAMTS-13活性正常。2例患者行肾活检显示1例为狼疮肾炎Ⅳ+Ⅴ型伴血栓性微血管病,1例为狼疮肾炎Ⅱ型。3例患者在确诊SLE相关TTP前均接受甲泼尼龙冲击联合静脉注射免疫球蛋白和吗替麦考酚酯(MMF)治疗,但病情加重,确诊后其中2例患者联合血浆置换治疗病情仍无改善,给予RTX治疗;1例患者在确诊后直接联合RTX治疗。经上述治疗后3例患者病情均达到完全缓解,平均随访14个月呈持续缓解,其中1例在随访6个月时因重症肺炎死亡。回顾文献结合本文系列报道,共6例应用RTX治疗ADAMTS-13活性正常的SLE相关TTP,显示所有患者均在糖皮质激素联合血浆置换和(或)联合免疫抑制剂治疗失败后加用RTX,或在初始治疗的同时即加用RTX,病情得到缓解。结论 SLE患者出现发热、贫血、血小板减少及LDH升高可能提示SLE相关TTP,而外周血涂片和肾活检检查有助于确诊。ADAMTS-13活性正常的SLE相关TTP患者神经系统和肾损害发生率高,病情重,对血浆置换联合糖皮质激素和(或)免疫抑制剂的治疗反应差,而RTX对这类患者有效,可改善患者预后。

  6. Platelets are versatile cells: New discoveries in hemostasis, thrombosis, immune responses, tumor metastasis and beyond.

    Science.gov (United States)

    Xu, Xiaohong Ruby; Zhang, Dan; Oswald, Brigitta Elaine; Carrim, Naadiya; Wang, Xiaozhong; Hou, Yan; Zhang, Qing; Lavalle, Christopher; McKeown, Thomas; Marshall, Alexandra H; Ni, Heyu

    2016-12-01

    Platelets are small anucleate blood cells generated from megakaryocytes in the bone marrow and cleared in the reticuloendothelial system. At the site of vascular injury, platelet adhesion, activation and aggregation constitute the first wave of hemostasis. Blood coagulation, which is initiated by the intrinsic or extrinsic coagulation cascades, is the second wave of hemostasis. Activated platelets can also provide negatively-charged surfaces that harbor coagulation factors and markedly potentiate cell-based thrombin generation. Recently, deposition of plasma fibronectin, and likely other plasma proteins, onto the injured vessel wall has been identified as a new "protein wave of hemostasis" that may occur even earlier than the first wave of hemostasis, platelet accumulation. Although no experimental evidence currently exists, it is conceivable that platelets may also contribute to this protein wave of hemostasis by releasing their granule fibronectin and other proteins that may facilitate fibronectin self- and non-self-assembly on the vessel wall. Thus, platelets may contribute to all three waves of hemostasis and are central players in this critical physiological process to prevent bleeding. Low platelet counts in blood caused by enhanced platelet clearance and/or impaired platelet production are usually associated with hemorrhage. Auto- and allo-immune thrombocytopenias such as idiopathic thrombocytopenic purpura and fetal and neonatal alloimmune thrombocytopenia may cause life-threatening bleeding such as intracranial hemorrhage. When triggered under pathological conditions such as rupture of an atherosclerotic plaque, excessive platelet activation and aggregation may result in thrombosis and vessel occlusion. This may lead to myocardial infarction or ischemic stroke, the major causes of mortality and morbidity worldwide. Platelets are also involved in deep vein thrombosis and thromboembolism, another leading cause of mortality. Although fibrinogen has been

  7. Early Digestive Endoscopy Diagnosis Value of Abdominal Type Allergic Purpura%腹型过敏性紫癜的早期消化内镜诊断价值

    Institute of Scientific and Technical Information of China (English)

    周传波

    2015-01-01

    目的:探讨腹型过敏性紫癜的早期消化内镜诊断价值。方法:2012年1月-2014年6月笔者所在医院对收治的26例腹型过敏性紫癜患者行早期消化内镜检查,分析其临床特点及内镜表现。结果:本组26例患者的胃肠道黏膜均出现不同程度的水肿、充血、大小不一的出血点、红斑、糜烂和溃疡,其中十二指肠黏膜(13/26)和回肠末端(2/6)发生病变的几率最高。结论:采用消化内镜检查腹型过敏性紫癜,真实可靠,准确性高,有利于疾病的早期诊断和治疗,具有重要的临床意义。%Objective:To investigate the early diagnostic value of digestive endoscopic for abdominal type allergic purpura.Method:26 patients with abdominal type allergic purpura from January 2012 to June 2014 in our hospital were checked by early gastrointestinal endoscopy,its clinical characteristics and endoscopic features were analysed.Result:Of the 26 patients the gastrointestinal mucosa showed varying degrees of edema,hyperemia,the size of the bleeding point,erythema,erosion and ulcer,duodenal mucosa(13/26) and the terminal ileum(2/6) lesions were the highest probability.Conclusion:The use of digestive endoscopic examination of abdominal type allergic purpura is reliable,high accuracy,it can facilitate earlier diagnosis and treatment of the disease,and has important clinical significance.

  8. Lenalidomide-Associated ITP

    Directory of Open Access Journals (Sweden)

    Christina I. Herold

    2011-01-01

    Full Text Available Lenalidomide is a potent immunomodulatory agent being used increasingly for treatment of hematologic malignancies including multiple myeloma and myelodysplasia. The common toxicities of lenalidomide, including dose-limiting myelosuppression, are well described. However, the immunomodulatory properties of lenalidomide may give rise to unexpected autoimmune complications. Herein, we describe a case of immune thrombocytopenic purpura (ITP associated with use of lenalidomide.

  9. Patofysiologien ved primær immun trombocytopeni

    DEFF Research Database (Denmark)

    Gudbrandsdottir, Sif; Frederiksen, Henrik; Birgens, Henrik Sverre;

    2011-01-01

    Primary immune thrombocytopenia (ITP)--formerly known as idiopathic thrombocytopenic purpura--is an autoimmune disorder characterized by immune-mediated thrombocytopenia. The aetiology of ITP remains unknown, but studies have shown that multiple immunological mechanisms are involved...... in the pathogenesis of ITP.This article aims to provide an overview of our knowledge of the pathogenesis of ITP....

  10. Possible lower rate of chronic ITP after IVIG for acute childhood ITP an analysis from registry I of the Intercontinental Cooperative ITP Study Group (ICIS)

    NARCIS (Netherlands)

    Tamminga, Rienk; Berchtold, Willi; Bruin, Marrie; Buchanan, George R.; Kuehne, Thomas

    2009-01-01

    P>In children, one-third of immune thrombocytopenic purpura (ITP) patients follow a chronic course. The present study investigated whether treatment with intravenous immunoglobulin (IVIG) at the time of diagnosis of ITP is of prognostic significance, using data from 1984 children entered in Registry

  11. Drug: D05777 [KEGG MEDICUS

    Lifescience Database Archive (English)

    Full Text Available D05777 Drug Ruplizumab (USAN/INN) Treatment of immune thrombocytopenic purpura and ...receptor signaling pathway Target-based classification of drugs [BR:br08310] Cyto...kines TNF family CD40L (CD154) [HSA:959] [KO:K03161] Ruplizumab D05777 Ruplizumab (USAN/INN) CAS: 220651-94-5 PubChem: 47207438 ...

  12. Thrombotic microangiopathy associated with bortezomib treatment in a patient with relapsed multiple myeloma

    Directory of Open Access Journals (Sweden)

    Urpu Salmenniemi

    2012-06-01

    Full Text Available Thrombotic thrombocytopenic purpura (TTP and hemolytic-uremic syndrome (HUS describe microvascular occlusive disorders characterized by thrombocytopenia due to increased platelet aggregation and fragmentation hemolysis. We report here what to our knowledge is the second case of TTP/HUS associated with bortezomib treatment.

  13. Drug: D09348 [KEGG MEDICUS

    Lifescience Database Archive (English)

    Full Text Available D09348.gif Treatment of rheumatoid arthritis, immune thrombocytopenic purpura, and B-cell lymphoma spleen ty...D09348 Drug Fostamatinib disodium (USAN) C23H24FN6O9P. 6H2O. 2Na 732.1756 732.5148

  14. Survival curves study of platelet labelling with 51Cr

    International Nuclear Information System (INIS)

    Platelet kinetics and idiopathic thrombocytopenic purpura were researched in the literature. An 'in vitro' platelet labelling with 51Cr procedure in implementation has been evaluated in human beings. Functions used for fitting considered the cases whether the curve was linear or exponential as well as the presence of hematies. (author)

  15. Characterization of human antibodies against ADAMTS13 that develop in patients with acquired TTP

    NARCIS (Netherlands)

    Pos, W.

    2010-01-01

    Thrombotic thrombocytopenic purpura (TTP) is a thrombotic micro-angiopathy characterized by the absence or dysfunction of the von Willebrand factor cleaving protease ADAMTS13. Functional absence of ADAMTS13 results in impaired cleavage of newly released ultra large von Willebrand factor (UL-VWF) mul

  16. Acquired TTP: ADAMTS13 meets the immune system

    NARCIS (Netherlands)

    N. Sorvillo

    2013-01-01

    Autoantibodies directed against ADAMTS13 prohibit the processing of VWF multimers, initiating a rare and life-threatening disorder called acquired thrombotic thrombocytopenic purpura (TTP). At present it is not clear why previously healthy individuals develop anti-ADAMTS13 antibodies. The studies de

  17. HUS AND TTP

    OpenAIRE

    Trachtman, Howard

    2013-01-01

    This review will describe the epidemiology, pathophysiology, presentation, clinical causes, treatment, and long-term prognosis of pediatric patients who present with thrombotic microangiopathy (TMA). The focus will be on hemolytic uremic syndrome (HUS) and thrombotic thrombocytopenic purpura (TTP), the most common phenotypes of TMA.

  18. Rituximab treatment for symptomatic chronic ITP

    NARCIS (Netherlands)

    Tamminga, Rienk Y. J.; Bruin, Marrie C. A.

    2006-01-01

    About 20% of the children diagnosed with acute idiopathic thrombocytopenic purpura (ITP) will run a chronic course. Only in a minority of these, platelet-count-enhancing treatments are indicated. Most treatment options are directed at decreasing platelet destruction including corticosteroids, intrav

  19. Klinisch denken en beslissen in de praktijk. Een man met icterus, nierfunctiestoornissen, trombocytopenie, spierpijn en verwardheid

    NARCIS (Netherlands)

    Huits, R M; van der Werf, T S; Zijlstra, J G

    2004-01-01

    A 51-year-old man became jaundiced, after experiencing flu-like symptoms for a week. On admission anaemia, thrombocytopenia, and hepatic and renal function disorders were present. One month previously he had had sinusitis. Thrombotic thrombocytopenic purpura (TTP)/haemolitic-uraemic syndrome (HUS) w

  20. Gastro-peritoneo-cutaneous fistula following splenectomy

    OpenAIRE

    BAYRAKÇI, Berna; ORUÇ, Nevin; TEKİN, Fatih; Elmas, Nevra; ÖZÜTEMİZ, A. Ömer

    2009-01-01

    Splenectomy operation is usually indicated for treatment of hematological disorders or splenic trauma. Splenectomy complications including gastric injury and peritoneal abscess formation were rarely reported. Forty seven years old male patient diagnosed with immune thrombocytopenic purpura and had splenectomy operation. Abdominal pain and cutaneous fistula was developed after the operation. Further investigations revealed gastric fistula opening endoscopically and presence of intraabdominal a...

  1. COMPARATIVE ANALYSIS OF SERUM IGE IN PATIENTS WITH ANAPHYLACTOID PURPURA, ECZEMA AND URTICARIA%过敏性紫癜、湿疹、荨麻疹患者血清IgE对比分析

    Institute of Scientific and Technical Information of China (English)

    韩晨珠; 陈丽莉; 梁官钊; 李保强

    2015-01-01

    目的::对比分析过敏性紫癜、湿疹、荨麻疹三种过敏性疾病患者血清中变应原特异性IgE和总IgE水平。方法:采用免疫印迹法检测341例过敏性疾病患者血清中总IgE和特异性IgE抗体水平。结果:过敏性紫癜、湿疹、荨麻疹患者总IgE阳性率分别为54.84%、65.79%、70.48%,以荨麻疹的阳性率最高。三种过敏性疾病吸入性变应原均以霉菌组合的阳性率最高;食入性变应原过敏性紫癜和湿疹牛奶的阳性率最高,荨麻疹以腰果类阳性率较高。结论:通过对三种过敏性疾病血清IgE的测定和对比分析,进一步证实Ⅰ型变态反应部分参与了过敏性紫癜和湿疹的致病;通过特异性变应原的测定,可了解引起患者过敏的变应原,为过敏性疾病的治疗和预防提供帮助。%Objective:To investigate the allergen-specific IgE and total IgE level in serum of patients with anaphylactoid purpura, eczema and urticaria.Methods: Immunoblotting was used to detect the allergen-speciifc IgE and total IgE level in serum of 341 cases allergic diseases patients.Results:The total IgE positive rate of anaphylactoid purpura, eczema and urticaria was respectively 54.84%, 65.79% and 70.48%; Among them, the positive rate of urticaria was the highest. For inhalant allergen, the positive rate of mold mixture was the highest in 3 allergic diseases. For food allergen, the positive rate of milk was the highest in anaphylactoid purpura and eczema; cashew allergies was the highest in urticaria. Conclusions:Comparative analysis of IgE level in serum of patients with 3 allergic diseases further conifrms that type I allergic reaction partly involves in allergic purpura and eczema. Determination of allergen can give a hand for treating and preventing allergic diseases.

  2. 儿童过敏性紫癜心脏损害分析%Analysis of heart damage of Henoch-Schonlein purpura in children

    Institute of Scientific and Technical Information of China (English)

    张才江

    2013-01-01

    目的:观察儿童过敏性紫癜合并心脏损害的发病率及临床特点。方法对162例过敏性紫癜患儿在诊断同时行心肌酶谱、心电图检查,根据心脏、胃肠道病变进一步行心脏超声、24小时动态心电图和腹部平片、CT检查。结果162例过敏性紫癜患者中有39例(24.1%)合并心脏损害,主要表现为各种类型心电图改变、心肌酶谱增高及心包积液。74例伴胃肠道损害病例中合并心脏损害51例(68.9%),其中7例外科急腹症合并有严重的心脏病变。对所有心脏损害病例应用肾上腺皮质激素、保护心肌及对症治疗后均治愈。结论过敏性紫癜患儿合并心脏损害是临床较常见的表现之一,同时胃肠道损害者大部分合并心脏损害,早期识别并积极治疗,可改善预后。%Objective To investigate the incidence and clinical characterics of heart involvement in children with Henoch-Schonlein purpura(HSP). Methods Myocardial enzymes and electrocardiogram were detected in 162 chlidren with HSP, and then further examitations of ultrasonic cardiogram, 24-h Holter monitoring, abdominal plain X rays and CT scans were processed according to heart and gastrointestinal lesions. Results Among 162 cases, heart involvement was found in 39 cases (24.1%), characterized by al sorts of ECG changes, increased myocardial enzymes and pericardial effusion. Among 74 cases with gastrointestine involvement, combined heart damage was found in 51 cases (68.9%), 7 of which were surgical acute abdomen combined with severe heart abnormalities. Patients with heart damage treated with adrenal cortical hormone and symptomatic therapy such as protecting the myocardium could be cured. Conclusions Combined with heart damage is one of the common clinical manifestations in chlidren with HSP, and most cases with gastrointestine involvement are combined with heart damage. It is benefit for improving prognosis to identify early and

  3. Annular purpura and step aerobics.

    Science.gov (United States)

    Allan, S J; Humphreys, F; Buxton, P K

    1994-09-01

    Step aerobic classes are at present one of the most popular forms of exercise undertaken by young adults. To date no dermatological abnormalities have been described in people regularly performing step aerobics. We describe a case in which a healthy young woman developed an extensive pigmented purpuric eruption 4 weeks after commencing regular step aerobic classes. The eruption resolved completely 8 weeks after regular exercise was ceased. PMID:7955503

  4. 儿童紫癜性肾炎诊治现状多中心回顾性调查分析%Multicenter investigation of diagnosis and treatment of Henoch-Schonlein purpura nephritis in childhood

    Institute of Scientific and Technical Information of China (English)

    全国儿童常见肾脏病诊治现状调研工作组

    2013-01-01

    目的 探讨我国住院儿童紫癜性肾炎的临床特点、病理表现及治疗现状.方法 回顾性分析全国40所医院2008年7月1日至2011年6月30日收治的新发过敏性紫癜、经临床或病理确诊为紫癜性肾炎并在调查单位接受治疗的紫癜性肾炎患儿的病历资料,并填写统一的调查表,收集相关资料,病例登记表经参研单位初步汇总后,由中华医学会儿科学分会肾脏学组最终汇总,由专门数据录入人员录入和核对,并随机抽样进行数据核实,最后对儿童紫癜性肾炎的临床特点、病理表现及治疗现状进行统计分析.结果 此次调研最终符合入选条件且病例完整可供分析者共4863例患儿,其中男2935例,女1928例,男女比例为1.52∶1,6~13岁为发病高峰.4702例(96.7%)患儿于过敏性紫癜起病6个月内确诊肾损害.临床分型最常见为血尿和蛋白尿型(2831例,58.2%).其中1625例患儿行肾活检治疗,最常见肾活检指征为蛋白尿合并镜下血尿(1149例,70.7%).1448例肾活检患儿提供病理分级,以Ⅲ级和Ⅱ级为多见.4863例患儿中接受激素及其他免疫抑制剂治疗者共3677例(75.6%).最常见的治疗方案为单用激素1655例(34.0%);单纯血尿型患儿(362例,56.2%)多未予特殊治疗,血尿和蛋白尿型患儿最常选择治疗方案仍为单用激素治疗.肾组织病理Ⅰ级和Ⅱ级以单用激素或联合雷公藤多甙治疗为主,病理Ⅲ级和Ⅳ级以激素+环磷酰胺+甲泼尼龙冲击治疗为主.结论 本研究调查分析了我国住院紫癜性肾炎患儿的临床诊治现状,目前多根据临床分型及参照病理分级选择治疗方案,但免疫抑制剂及非特异性治疗的选择和使用方式多样,仍有待进一步临床多中心研究结果验证并加以规范.%Objective To retrospectively investigate the current diagnosis and treatment of children with Henoch-Schonlein purpura nephritis in hospital,to survey the

  5. 孟鲁司特对过敏性紫癜患儿血浆细胞因子水平的疗效观察%The Efficacy of Montelukast in Children with Allergic Purpura of Plasma Cytokines Levels

    Institute of Scientific and Technical Information of China (English)

    刘芳; 钟日英; 涂燕青; 周莲珠; 王景香

    2015-01-01

    Objective:To study the clinical efficacy of montelukast in children with allergic purpura of plasma cytokine levels .Method:100 children with allergic purpura were selected in our hospital as research subjects,and were randomly divided into two groups,the control group of children were given anti-histamine drugs,anti-infective drugs and calcium,vitamin C and other conventional drugs.The observation group was treated with montelukast chewable tablets treatment on the basis of conventional therapy. In two weeks for a course of treatment,the clinical effect of plasma cytokine levels in patients before and after treatment were compared and observed.Result:IL-6,IL-8 and TNF-αexpression levels observed before the treatment,two groups of patients showed no significant difference(P>0.05);after treatment,the expression levels of three indicators had decreased significantly,and compared with before the treatment,the differences were statistically significant(P0.05);治疗后,三项指标的表达水平都有显著降低,且与治疗前相比差异均有统计学意义(P<0.05);观察组患者各项指标的下降程度超过对照组患者,两组比较差异具有统计意义(P<0.05)。观察组和对照组患者的临床治疗总有效率分别是96.0%和78.0%,两组比较差异有统计学意义(P<0.05)。结论:孟鲁司特治疗儿童过敏性紫癜具有临床疗效确切、安全性好的优点,可以对患儿血浆内的细胞因子进行调节,促进患儿免疫功能的改善,值得临床推广应用。

  6. Studies on the Relationship of the Syndromes and the TCM Constitution of the Henoch-Schonlein Purpura Nephritis Patients%紫癜性肾炎患者证候与中医体质类型关系研究

    Institute of Scientific and Technical Information of China (English)

    乔会秀

    2012-01-01

    Objective: To survey the TCM constitutional characteristics and syndromes in the patients with the henoch-schonlein purpura nephritis, study the relationships between constitutions and syndromes and provide the basis for preventing and treating henoch-schonlein purpura nephritis with Chinese medicine. Methods: The constitutional types were investigated by applying the standard in Classification and Determination of TCM constitutions. The questionnaire designed by assigned physicians was used to survey TCM syndrome types. The correlation between constitutional characteristics and TCM syndromes was analysed. Results: Among 120 patients with Henoch-schonlein purpura nephritis, the wind-heat invading collaterals syndrome for 32.5%, Bleeding due to blood-heat syndrome 26.7%, hyperactivity of fire due to yin deficiency syndrome23.3%, Qi deficiency bleeding syndrome 17.5%, allergic constitution 21.6%, damp-heat constitution 19.2%, yin asthenia constitution 17.5%, qi asthenia constitution 13.3%, blood stasis constitution 11.7%. The constitution of phlegm. The wind-heat invading collaterals syndrome was correlated significantly to the allergic constitution ( P<0.01 ). And bleeding due to blood-heat syndrome was correlated significantly to the blood stasis constitution and damp-heat constitution ( P<0.05 ) . The hyperactivity of fire due to yin deficiency syndrome was correlated to the yin asthenia constitution,( P<0.01 ) . The Qi deficiency bleeding syndrome was correlated significantly to the qi asthenia constitution (P<0.01 ). Conclusion: The constitutional types of the patients with the henoch-schonlein purpura nephritis mostlv allergic constitution and damp-heat constitution. The syndrome types are mostly the syndrome of the wind-heat invading collaterals syndrome and bleeding due to blood-heat syndrome. There is a correlation between the the wind-heat invading collaterals syndrome and the allergic constitution, bleeding due to blood-heat syndrome and the blood stasis

  7. IgA Nephropathy and Henoch-Schoenlein Purpura Nephritis: Aberrant Glycosylation of IgA1, Formation of IgA1-Containing Immune Complexes, and Activation of Mesangial Cells

    DEFF Research Database (Denmark)

    Novak, J.; Moldoveanu, Z.; Renfrow, M.B.;

    2007-01-01

    IgA1 in the circulation and glomerular deposits of patients with IgA nephropathy (IgAN) is aberrantly glycosylated; the hinge-region O-linked glycans are galactose-deficient. The circulating IgA1 of patients with Henoch-Schoenlein purpura nephritis (HSPN) has a similar defect. This aberrancy...... at specific sites. We sought to define the aberrant glycosylation of a galactose-deficient IgA1 myeloma protein and analyze the formation of the immune complexes and their biological activities. Supplementation of serum or cord-blood serum with this IgA1 protein resulted in formation of new IgA1 complexes...... determined the O-glycosylation sites in the hinge region of the IgA1 myeloma protein and IgA1 proteins from sera of IgAN patients. The IgA1 myeloma protein had galactose-deficient sites at residues 228 and/or 230 and 232. These sites reacted with IgG specific to galactose-deficient IgA1. IgA1 from the Ig...

  8. 62例成人紫癜性肾炎临床与病理分析%Clinical and pathological manifestations of 62 patients with Henoch-Schonlein Purpura nephritis

    Institute of Scientific and Technical Information of China (English)

    范俊英; 王彩丽; 刘丽萍; 王慧

    2012-01-01

    目的 分析成人紫癜性肾炎患者的临床表现和肾脏病理特征.方法 回顾收集62例成人紫癜性肾炎患者的临床资料,分析其临床表现与病理分级分布情况.结果 肾病综合征组与血尿伴蛋白尿组、单纯血尿或蛋白尿组血比较,肌酐水平差异有统计学意义[(203± 132) μmol/L,(79±32) μmol/L,(81 ±24) μmol/L].补体C3、血沉和IgA水平在3组之间差异无统计学意义.血尿伴蛋白尿者病理分型以Ⅱ级(12例)和Ⅲa级(16例)为主,Ⅳ级4例;单纯血尿或蛋白尿者以Ⅱ级和Ⅲb级为主(各4例),Ⅲa级和Ⅵa级各2例;肾病综合征以Ⅲa级和Ⅳa级为主(各4例),Ⅲb、Ⅳb、Ⅴ级各2例,Ⅵ级4例;肾衰竭8例,均为肾病综合征表现,肾病综合征组与其他两组病理分级比较,病理改变较重.结论 成人紫癜性肾炎临床表现与病理分型多种多样,血清肌酐与蛋白尿在一定程度上可以反映病情,肾病综合征、肾小球新月体是紫癜性肾炎预后不良的危险因素.%Objective To analyze the clinical manifestations and pathological features of Henoch -Schonlein purpura nephritis patients. Methods The clinical data of 62 patients with purpura nephritis were analyzed restrospectively. And the clinical manifestations, the grade and distributions of renal pathology were analyzed. Results Patients were divided into the nephritic syndrome group, hematuria combined with proteinuria group, simple hematuria group, and the simple proteinuria group. The serum creatinin level was significantly different between the nephritic syndrome group and the hematuria combined with proteinuria group, the simple hematuris group and proteinuria group[(79?32)u,mol/L], the simple hematuria or simple proteinuria group [(81?4)u,mol/LJ, but the nephrotic syndrome group [(203 ?132) u,mol/L], but there was no significant difference in serum C3 level, ESR and IgA level between the groups. The most common pathological manifestations were Grade II

  9. Intracranial Hemorrhage as a Source of Headache in a Patient with Postpartum

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    Jamshid Vafaeimanesh

    2014-06-01

    Full Text Available Background: Postpartum thrombotic thrombocytopenic purpura (TTP should be considered in thrombocytopenic patients. Case report: The patient was a 28-year-old pregnant woman referred for cesarean section. After CS, hysterectomy was done due to uncontrolled vaginal bleeding. Peripheral blood smear confirmed the TTP diagnosis. Plasmapheresis was initiated and platelets were infused. Six hours after infusion, tonic-colonic seizure, left hemiplegia and bell's palsy appeared. Brain CT Scan revealed intracranial hemorrhage. 28 plasmapheresis sessions were performed and finally, she was discharged with good general condition.

  10. Clinical and Endoscopic Analysis of Adult Abdominal-type Henoch-schonlein Purpura%成人腹型过敏性紫癜的临床和内镜分析

    Institute of Scientific and Technical Information of China (English)

    张磊; 董向前; 张颖慧

    2013-01-01

    [Objective] To summarize clinical characteristics and endoscopic manifestations of abdominal-type Henoch-schonlein purpura(HSP) in adult patients in order to provide the basis for the early diagnosis of the disease. [Methods] Clinical characteristics and endoscopic manifestations of 35 adult patients with abdominal-type HSP were analyzed retrospectively. [Results] All 35 patients were cured within 2~6 weeks. All patients had abdominal pain including peri-umbilical abdominal pain (48. 6%), middle-upper abdominal pain (37.1%) and lower abdominal pain(14. 3%). Twelve patients(34. 2%) were complicated with nausea and vomiting. Sixteen patients(45. 7%) had digestive tract bleeding. Purpura of low limbs was found 2~14 days after the occurrence of abdominal pain. Kndoscopic manifestations were diffused congestive edema of gastrointestinal mucosa, widespread hemorrhagic spots, erythema, erosion and ulceration. Duodenal, ileum, caecum and colon ascendens had more severe mucous lesions, but the esophageal mucosas in all patients were normal. Histological manifestations showed massive neutrophilic infiltration in mucosa and submucosa and fibrotic necrosis of small vessels. Spasmolytics and antiacids were ineffective, while adrenal cortical hormone was effective. [Conclusion]Small bowel lesions are severer than those of stomach and colon in abdominal-type HSP patients. Clinical and endoscopic manifestations are helpful for the early diagnosis.%[目的]总结成人腹型过敏性紫癜(HSP)的临床特点与内镜表现,为该病早期诊断提供依据.[方法]对35例成人腹型HSP的临床特点与内镜表现进行回顾分析.[结果]3所有患者均有腹痛,其中脐周腹痛17例(48.6%)、中上腹痛13例(37.1%)、下腹痛5例(14.3%),12例(34.2%)患者伴恶心、呕吐,16例(45.7%)患者有消化道出血,腹痛发生后2~14 d出现下肢紫癜.内镜表现为胃肠道黏膜弥漫性充血水肿,广泛多发的出血点、红斑、糜烂、溃疡,以十二指肠

  11. 血小板活化因子乙酰水解酶与过敏性紫癜肾脏损害的关联性%Correlation of Platelet Activating Factor Acetylhydrolase and Henoch-Schonlein Purpura Nephritis

    Institute of Scientific and Technical Information of China (English)

    陆彪; 刘静

    2012-01-01

    Objective To explore the correlation of platelet activating factor acetylhydrolase(PAF - AH) and Henoch - Schonlein pur-pura nephritis ( HSPN). Methods Seventy children with Henoch - Schonlein purpura ( HSP) who were admitted into the General Hospital of Ningxia Medical University from Oct. 2010 to Apr. 2011 were selected,and according to whether they had the kidney damage,they were divided into non - HSPN group and HSPN group. Fifty healthy children were collected as healthy control group. And real - time fluorescent quanti-tive polymerase chain reaction was used to detect the expression of PAF - AH mRNA. Serum PAF - AH, vascular endothelial growth factor ( VEGF) ,and heat shock protein 70( HSP70) levels were detected by using enzyme - linked immunosorbent assay. SPSS 13.0 software was used to analyze the data. Results 1. The expressions of PAF - AH mRNA and protein, VEGF and HSP70 in HSPN group were higher than those in non- HSPN group and healthy control group, which were significantly positive(Pa <0.01). The expressions of PAF - AH protein, VEGF and HSP70 in non - HSPN group were higher than those in healthy control group,which were significantly positive(Pa <0.01).2. PAF-AH was positively correlated with VEGF and HSP70 in HSPN group( Pa < 0.01). Conclusion PAF - AH is involved in the pathogenesis of HSP,which has relevance with kidney damage.%目的 探讨血小板活化因子乙酰水解酶(PAF-AH)与过敏性紫癜(HSP)肾脏损害的相关性.方法 收集2010年10月-2011年4月在宁夏医科大学总医院住院的HSP患儿70例,并按有无肾脏损害将其分为非紫癜性肾炎(HSPN)组和HSPN组.另选体检健康的50例儿童为健康对照组.采用实时荧光定量PCR技术检测其PAF-AH mRNA表达,同时采用双抗体夹心酶联免疫吸附法检测其血清PAF-AH、血管内皮生长因子(VEGF)、热休克蛋白70 (HSP70)水平变化.采用SPSS 13.0软件进行数据处理.结果 1.HSPN组PAF-AH mRNA及蛋白表达、VEGF及HSP70表达

  12. Correlation between food intolerance and Henoch-Sch(o)nlein purpura in children%食物不耐受与儿童过敏性紫癜的相关性

    Institute of Scientific and Technical Information of China (English)

    张建江; 史佩佩; 张利果; 陆凤霞; 赵丽丽

    2011-01-01

    Objective To investigate the correlation between food intolerance and Henoch-Sch(o)nlein purpura (HSP) in children and the efficacy of food forbidden or alternative therapy. Methods The levels of IgG against several common food in serum obtained from 40 children with HSP were measured by ELISA. The efficacy of food forbidden or alternative food therapy was assessed after 3 months. Results Total positive rate of serum food-intolerant IgG antibodies in HSP children was 92.5%. Among these 14 intolerant foods, the positive percentage of egg was the highest (33.8%), followed by tomato (14.9%), milk (13.5%) and morrhua (12.2%). Significant differences of the sort and degree of food intolerance existed among different age groups (t=2.257, P=0.045), but not between boys and girls (t=1.053, P=0.315), city and countryside (t= 1.388, P=0.193). There was no linear correlation between total food intolerance and serum IgG level (t=0.793, P=0.445). Food intolerance had no direct relation to immune complex deposition in kidneys of HSP nephritis (r =-0.262, P =0.387). The efficacy of adjusting diet was 95% . Conclusions HSP is closely related to food intolerance. Egg is the most common intolerant food. Food forbidden or alternative therapy shows acceptable efficacy in the treatment of most Henoch-Sch(o)nlein purpura children.%目的 探讨食物不耐受与儿童过敏性紫癜的相关性及不耐受食物禁食或轮替疗法对过敏性紫癜患儿的疗效.方法 ELISA法检测40例过敏性紫癜患儿血清14种食物特异性IgG浓度及相关生化指标,同时进行3个月不耐受食物禁食或轮替疗法,观察疗效.结果 过敏性紫癜息儿食物不耐受阳性率为92.5%,鸡蛋的阳性率最高,其次为西红柿、牛奶、鳕鱼等.食物不耐受种类在男女、城乡分布间差异无统计学意义(t=1.053,P=0.315;t=1.388,P=0.193),但不同年龄间差异有统计学意义(t=2.257,P=0.045).食物不耐受累计敏感度与血

  13. Henoch-Schönlein purpura in adults: a case series from a multidisciplinary study group Púrpura de Henoch-Schönlein em adultos: uma série de casos de um grupo de estudo multidisciplinar

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    Boris A. Cruz

    2006-12-01

    Full Text Available BACKGROUND: Henoch-Schönlein purpura (HSP is a systemic vasculitis involving small vessels with the deposition of immune complexes containing IgA. It has been extensively studied in children, but in adults, its natural history is much less known. OBJECTIVES: to report a series of patients with HSP presenting in their adulthood. PATIENTS AND METHODS: the Minas Gerais Vasculitis Study Group´s Members (MGVSG were invited to report patients with HSP who appeared in their adulthood. A standardized retrospective chart review was done. RESULTS: eleven patients, two male and nine female, age 39.4 +/- 20.1 yearsold were studied. Nine patients presented purpura, seven presented arthritis, four patients had gastrointestinal involvement and ten patients (91% presented glomerulonephritis (GN. Eight patients were subjected to renal biopsies. Six of them presented endocapillary proliferative GN and only two of them had minimal mesangial proliferation. In the other three patients, HSP was confirmed by skin biopsies. All patients received steroids, in five of them steroids were combined with other immunosuppressive agents. After a follow-up of 39.0 +/- 64.6 months, four patients (36% presented impairment of renal function, but only one (9% developed end stage renal disease and was successfully appeared to renal transplantation. At the end of follow-up, seven patients (64% are in complete remission and four in partial remission. CONCLUSION: in adulthood, HSP represents a distinct clinical syndrome with a higher frequency of renal involvement and more severe systemic vasculitis. Nevertheless, the final outcome in this series was as good as reported in children, maybe due to aggressive immunosuppressive therapy.INTRODUÇÃO: púrpura de Henoch-Schönlein (PHS é uma vasculite sistêmica que acomete vasos de pequeno calibre com depósitos de imunocomplexos contendo IgA. Esta vasculite já foi extensamente estudada em crianças, mas sua história natural em adultos

  14. Clinical observation of dipyridamole combined with methylprednisolone in treatment of infantile allergic purpura%双嘧达莫联合甲基强的松治疗小儿过敏性紫癜的疗效观察

    Institute of Scientific and Technical Information of China (English)

    贺金娥

    2015-01-01

    Objective To study the clinical effect of dipyridamole combined with methylprednisolone in treatment of infantile allergic purpura.Methods Patients (80 cases) with allergic purpura in Department of Pediatrics of Yanan University Affiliated Hospital from August 2012 to August 2014 were randomly divided into control and treatment groups, and each group had 40 cases. The patients in the control group were iv administered with Methylprednisolone Sodium Succinate for injection 20 mg/(kg·d) which was added into 5% glucose solution 150 mL in 2 h for the first day to the third day, and the dosage was in half every 3 d. They werepo administered with Prednisone Acetate Tablets 2.0 mg/(kg·d)until the children were in stable condition, and the dosage was 1.0 mg/(kg·d) after two weeks. The patients in the control group werepo administered with Dipyridamole Tablets on the basis of the control group, 3.0 mg/(kg·d). Two groups were treated for six months. After treatment, the efficacy was evaluated, and the changes of kidney damage disappearance time, abdominal pain disappearance time, joint swelling and pain disappeared time, gastrointestinal bleeding subsided time, rash subsided time, and hospital stay time in two groups were compared.Results After treatment, the efficacies in the control and treatment groups were 75.0% and 97.5%, respectively, and there were differences between two groups (P < 0.05). After treatment, kidney damage disappearance time, abdominal pain disappearance time, joint swelling and pain disappeared time, gastrointestinal bleeding subsided time, rash subsided time, and hospital stay time in treatment group were shorter than those in the control group, with significant difference between two groups (P < 0.05).ConclusionDipyridamole combined with methylprednisolone has good clinical curative effect in treatment of infantile allergic purpura, and can effectively improve the clinical symptoms and signs, also can improve the overall efficiency, which has a

  15. AMEGAKARYOCYTIC THROMBOCYTOPENIC PURPURA‎: A FIFTEEN YEAR EXPERIENCE

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    M. Bakhshi

    2006-07-01

    Full Text Available Totally implantable venous access devices (TIVAD or implantable catheter ports are devices which can be implanted subcutaneously. They enable prolonged and repeated access to the vascular system, into the peritoneal cavity or intravertebral space. This device is particularly useful for repeated medical injection, for blood sampling or transfusion of blood and blood derivatives and for total parenteral nutrition (TPN. Although many patients benefit from the insertion of TIVAD without any secondary effects, any surgical implantation can nevertheless lead to complications. ‎In this study, we investigated the advantages and disadvantages of TIVAD catheter in pediatric age group. A total of 94 cases, 2 to 14 years old, were included in our study. We implanted TIVAD in these patients for chemotherapy in 83 cases (88.29%, for prolonged TPN in 6 cases (6.38%, for corticosteroid and antibiotic therapy after ‎Kasai operation in 2 cases (2.12%, for intermittent IV therapy in 2 cases (2.12% and for need to partial parenteral nutrition in 1 case (1.06%. Out of 94 cases, 14 cases (15% had some kind of complications and 80 cases (85% had no complication. There was no mortality. Most patients and their parents (82 cases, 87.23% were satisfied from TIVAD. ‎It seems that TIVAD can be a useful device for many chronic patients who need an IV access for multiple injections.

  16. Detection of food specific IgG and observation of the therapeutic effect of fast/alternate treatment in patients with henoch-schomlein purpura%过敏性紫癜食物特异性IgG检测及禁食/轮替治疗疗效观察

    Institute of Scientific and Technical Information of China (English)

    陈小红; 许飏; 黎昌强; 柳青

    2013-01-01

    Objective To detect 14 kind of food specific IgG in patients with simple type henoch-schonlein purpura,and observe the recurrence rate after fast/alternate treatment.Methods 14 kind of food specific IgG levels in 62 cases of simple type henochschonlein purpura and 60 healthy people were tested by ELISA.The 62 simple type HSP patients were divided into the treatment group(32 cases) and control group(30 cases).Treatment group underwent fast/alternate treatment,and the control group underwent conventional therapy,IgG level and the recurrence rate 6 months after treatment in groupswere analyzed.Results IgG levels of in patients with simple type henoch-schonlein purpura was higher than that of healthy group,with significant difference(P< 0.05).The level of IgG in patients with simple type henoch-schonlein purpura decreased 6 months after treatment,and the level of IgG in treatment group was lower than that of control group,with significant difference(P<0.05).The two groups have questionnaire after six months.Recurrence rate of the treatment group was 6.67% and that of control group was 33.33%,which showed significant difference(P<0.05).Conclusion The food specific IgG level has correlation with simple type henoch-schonlein purpura.Fast/alternate treatment is of good value for this disease.%目的 检测单纯型过敏性紫癜患者血清14种食物特异性免疫球蛋白G(IgG),并应用禁食/轮替治疗观察复发率.方法 采用酶联免疫吸附法(ELISA)检测62例单纯型过敏性紫癜及60例健康对照组血清14种食物特异性IgG抗体水平,将62例单纯型过敏性紫癜分为治疗组32例及对照组30例,治疗组行禁食/轮替治疗,对照组行未控制饮食的常规治疗,6个月后观察复发率并检测二组IgG水平.结果 单纯型过敏性紫癜患者IgG水平高于健康对照组,两组差异有统计学意义(P<0.05);单纯型过敏性紫癜患者治疗6个月后IgG均下降,且治疗组较对照组下降明

  17. Frequency of anti-glycoprotein Ia/IIa (anti-HPA-5b,-5a and anti-glycoprotein IIb/IIIa (anti-HPA-1a,-3a,-4a alloantibodies in multiparous women of African descent

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    Zaccheaus A Jeremiah

    2010-05-01

    Full Text Available Zaccheaus A Jeremiah1, Justina E Oburu2, Osaro Erhabor1, Fiekumo I Buseri1, Teddy C Adias31Haematology and Blood Transfusion Science Unit, Department of Medical Laboratory Sciences, College of Health Sciences, Niger Delta University, Wilberforce Island, Nigeria; 2Department of Haematology and Blood Transfusion, University of Port Harcourt Teaching Hospital, Port Harcourt, Nigeria; 3Rivers State University of Science and Technology, Port Harcourt, NigeriaBackground: Human platelet antibodies are often implicated in some disease conditions, such as neonatal alloimmune thrombocytopenia (NAIT, idiopathic thrombocytopenic purpura (ITP and platelet refractoriness. The frequencies of these alloantibodies have not been reported in Nigeria and West Africa.Methods: Screening for allontibodies to human platelet antigens (HPA was undertaken using the GTI PakPlus® qualitative solid phase ELISA reagent. Platelet count was done using the ICSH approved procedure using 1% ammonium oxalate reagent.Study design: A cross-section of apparently healthy adult Nigerian multiparous non-pregnant women, who were staff of a tertiary health facility in the Niger Delta, Nigeria, were screened for alloantibodies to human platelet antigens.Results: Of the one hundred (100 women screened, the prevalence of anti-glycoprotein IIb/IIIa (anti-HPA-Ia,-3a,-4a was zero percent (0%, anti-glycoprotein Ia/IIa (anti-HPA-5b accounted for 30% of results, while anti-glycoprotein Ia/IIa (anti-HPA-5a was 18%. Parity was found to exert significant influence on the development to HPA antibodies (Fisher’s Exact Test = 11.683, P < 0.05; 13.577, P < 0.01. The platelet count of the women did not appear to exert any influence on the development of the antibodies (P > 0.05.Conclusion: This study has observed a high prevalence of anti-HPA-5b in our sample population. The prevalence of alloantibodies to HPA antigens was found to associate strongly with parity. These results indicate that there is a

  18. Uso de Terapia VAC® en paciente pediátrico con exposición ósea postamputación tras púrpura fulminans VAC® Therapy in pediatric patient with bone exposition after purpura fulminans

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    J. Navarro Cecilia

    2011-12-01

    Full Text Available La sepsis meningocóccica se asocia con un alto índice de mortalidad. Los pacientes afectados pueden mostrar signos de coagulación intravascular diseminada (CID y necrosis de partes blandas debidas a baja perfusión tisular. Los procedimientos quirúrgicos precoces suelen demorarse en estos casos, pues inicialmente es difícil determinar el grado de la lesión. Una vez que el paciente está estable, es esencial el desbridamiento de todo tejido necrótico, teniendo como objetivo el preservar la máxima cantidad de tejido posible, especialmente las articulaciones. Muchos niños sobreviven a esta patología, pero sufren gran morbilidad: amputación de una o varias extremidades y otras complicaciones derivadas como exposiciones óseas. Existen pocas publicaciones que hagan referencia al tratamiento no quirúrgico de este tipo de lesiones. Presentamos un caso de aplicación del sistema de terapia de presión negativa VAC® en paciente en edad pediátrica tras pérdida de sustancia como resultado de una púrpura fulminans secundaria a meningococcemia.Meningococcal sepsis is associated with a high mortality rate. These patients may show severe disseminated intravascular coagulation (DIC and skin, subcutaneous tissue and muscle necrosis due to poor tissue perfusion. It is rare that early surgical intervention be required, as it is relatively difficult to determine the degree of tissue loss early on. Once the patient is stable, debridement of all necrotic tissue is essential, and the goals are still to preserve as much tissue as possible, specially the joints. Many children survive the disease, but suffer major morbidities such as extremities amputation, which may result of complications like bone exposure. There is very little published experience regarding the non-surgical treatment of this complication. We report a new possible application of the vacuum-assisted closure system (VAC® in very young patients with loss of tissue as a result of purpura

  19. 肝素钠治疗小儿过敏性紫癜的护理体会%Nursing Experience on Treatment of the Children’s Anaphylactoid Purpura with Heparin Sodium

    Institute of Scientific and Technical Information of China (English)

    2013-01-01

    Objective:To explore how nurses improve their skills efficiently by summarizing the nursing experience on treatment of the children’s anaphylactoid purpura with heparin sodium with the use of heparin sodium in author’s department.Method:11 cases of children who suffered AP in our pediatrics dept from 1st Jan.2011 to 30 Nov.2012 were collected.Combined with characteristics of young patients,the nursing strategies were raised with analyzing of nursing interventions.Result:The HS treatment to children’s AP was highly safe and convenient.Apart from the general nursing cares,the study of HS’nursing essentials was necessary.Conclusion:In order to have more and more patients healed,it’s imperative to familiarize its medicinal properties and operational skills.%  目的:总结本科在使用肝素钠(Heparin sodium,HS)治疗儿童过敏性紫癜(Anaphylactoid purpura,AP)的护理体会,探讨护士如何有效地提高自己的技术水平。方法:收集本科自2011年1月1日-2012年11月30日以来发作 AP 的患儿11例,结合本科患者年纪小的特点,并对这些病例的护理措施加以分析,并提出相应的护理对策。结果:HS 治疗 AP 安全性高且使用方便,除了一般的护理要素,掌握该药物的护理要点有十分必要性。结论:为了使该药物能更好地为患者服务,熟练掌握其药物特性及操作技能势在必行。

  20. Dapsone Induced Methaemoglobinemia: Early Intraoperative Detection by Pulse Oximeter Desaturation

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    Mahmood Rafiq

    2008-01-01

    Case signifies the importance of knowledge of any preoperative drug intake and its anaesthetic implications. Also patients on dapsone therapy especially children should be monitored for methaemoglobin levels. Since children with immune thrombocy-topenic purpura are being treated with dapsone these days and many of these patients would be planned for splenectomy, monitoring of preoperative methaemoglobin levels and methaemoglobinemia as a cause of intraoperative pulse oximeter desaturation should be kept in mind.

  1. Nye behandlingsmuligheder ved primær immun trombocytopeni

    DEFF Research Database (Denmark)

    Gudbrandsdottir, Sif; Frederiksen, Henrik; Birgens, Henrik Sverre;

    2011-01-01

    Primary immune thrombocytopenia (ITP)--formerly known as idiopathic thrombocytopenic purpura--is an autoimmune disorder characterized by immune mediated thrombocytopenia. The aetiology of ITP remains unknown, but studies have shown that multiple immunological mechanisms are involved...... in the pathogenesis of ITP. This article aims to provide an overview of current treatment options, with particular emphasis on new biological therapies: rituximab, a monoclonal anti-CD20 antibody, and the thrombopoietin receptor agonists romiplostim and eltrombopag....

  2. Oral vesiculobullous lesions: Consider the platelets

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    Clare Steel

    2014-01-01

    Full Text Available Oral vesiculobullous lesions or "blood blisters" can be found on a routine dental examination and may have many causes. Trauma is often the first diagnosis followed by a variety of bullous conditions, such as pemphigus and pemphigoid. Using a case report, we highlight the other, more serious, possibility of idiopathic thrombocytopenic purpura (ITP to raise awareness to the General Dental Practitioners and the need for prompt treatment.

  3. Pregnancy-Associated Hemolytic Uremic Syndrome Revisited in the Era of Complement Gene Mutations

    OpenAIRE

    Fakhouri, Fadi; Roumenina, Lubka; Provot, François; Sallée, Marion; Caillard, Sophie; Couzi, Lionel; Essig, Marie; Ribes, David; Dragon-Durey, Marie-Agnès; Bridoux, Frank; Rondeau, Eric; Frémeaux-Bacchi, Veronique

    2010-01-01

    In contrast to pregnancy-associated thrombotic thrombocytopenic purpura, the pathogenesis and presentation of pregnancy-associated atypical hemolytic uremic syndrome (P-aHUS) remain ill-defined. We conducted a retrospective study to assess the presentation and outcomes of patients presenting with P-aHUS and the prevalence of alternative C3 convertase dysregulation. P-aHUS occurred in 21 of the 100 adult female patients with atypical HUS, with 79% presenting postpartum. We detected complement ...

  4. Lack of Clinically Significant Pharmacokinetic Interaction between the Thrombopoietin Receptor Agonist Eltrombopag and Hepatitis C Virus Protease Inhibitors Boceprevir and Telaprevir

    OpenAIRE

    Wire, Mary Beth; Fang, Lei; Hussaini, Azra; Kleha, Joseph F.; Theodore, Dickens

    2014-01-01

    Eltrombopag is an orally bioavailable thrombopoietin receptor agonist approved for the treatment of thrombocytopenia associated with chronic immune (idiopathic) thrombocytopenic purpura and chronic hepatitis C virus (HCV) infection. This study evaluated the potential drug-drug interactions between eltrombopag and the HCV protease inhibitors boceprevir and telaprevir. In this open-label, 3-period, single-sequence, and crossover study, 56 healthy adult subjects were randomized 1:1 to cohort 1 (...

  5. Assessment of the Pharmacokinetic Interaction between Eltrombopag and Lopinavir-Ritonavir in Healthy Adult Subjects

    OpenAIRE

    Wire, Mary B.; McLean, Heidi B.; Pendry, Carolyn; Theodore, Dickens; Park, Jung W.; Peng, Bin

    2012-01-01

    Eltrombopag is an orally bioavailable thrombopoietin receptor agonist that is approved for the treatment of chronic idiopathic thrombocytopenic purpura. It is being developed for other medical disorders that are associated with thrombocytopenia. Patients with human immunodeficiency virus (HIV) may suffer from thrombocytopenia as a result of their HIV disease or coinfection with hepatitis C virus (HCV). HIV medications, particularly ritonavir (RTV)-boosted HIV protease inhibitors, are involved...

  6. Marked Improvement in Refractory TTP Directly after H. pylori Eradication Therapy

    OpenAIRE

    Irina Gringauz; Narin Nard Carmel-Neiderman; Tobin Mangel; Orith Portnoy; Gad Segal; Idan Goren

    2016-01-01

    Thrombotic thrombocytopenic purpura (TTP) is a life-threatening disorder involving thrombotic microangiopathy and is characterized by increased platelet aggregation throughout the body. Acquired TTP can be triggered by a variety of conditions including infections. We hereby describe a case report of an 81-year-old female presenting to the internal medicine department with TTP and active chronic gastritis, positive for Helicobacter pylori (H. pylori) on biopsy. The TTP was highly resistant to ...

  7. Therapeutic dilemma in the management of a patient with the clinical picture of TTP and severe B12 deficiency

    OpenAIRE

    Walter, Kara; Vaughn, Jennifer; Martin, Daniel

    2015-01-01

    Background Idiopathic thrombotic thrombocytopenic purpura (TTP) is a rare hematological emergency characterized by the pentad of microangiopathic hemolytic anemia, thrombocytopenia, neurological symptoms, renal injury, and fever that is invariably fatal if left untreated. Prompt intervention with plasma exchange minimizes mortality and is the cornerstone of therapy. Rare reports have described “pseudo-TTP” driven by extreme hematologic abnormalities resulting from deficiency of vitamin B12. D...

  8. Thrombotic Microangiopathy with Skin Localization Secondary to Cytarabine-Daunorubicin Association: Report of a Case

    Directory of Open Access Journals (Sweden)

    S. Regragui

    2012-01-01

    Full Text Available The thrombotic microangiopathy is a syndrome characterized by the combination of mechanical hemolytic anemia, peripheral thrombocytopenia, and organ failure of variable severity. In addition to the idiopathic form, several cases are identified as secondary to pregnancy, infections, disease systems, organ transplants, and cancer. Other forms are secondary to drugs including antimitotics. We report the case of a patient followed for acute myelogenous leukemia. She received induction chemotherapy combining daunorubicin and cytarabine, complicated by thrombotic thrombocytopenic purpura.

  9. Multiple tuberculous aneurysms of the aorta.

    Science.gov (United States)

    Pierret, Charles; Tourtier, Jean-Pierre; Grand, Bertrand; Boddaert, Guillaume; Laurian, Claude; de Kerangal, Xavier

    2011-06-01

    Tuberculous aneurysms of the aorta are quite rare, but are exceptional when found in multiple locations. We report the case of multiple tuberculous aortic aneurysms of the thoracic and abdominal aorta in a 19-year-old female discovered when she consulted for thrombocytopenic purpura. The treatment for both locations included prolonged antituberculous therapy and surgical resection with cryopreserved aortic allograft patch for the reconstruction.

  10. Priporočila za uporabo in zdravljenje s svežo zmrznjeno plazmo: Recommendations for the usage and treatment with fresh frozen plasma:

    OpenAIRE

    Domanovič, Dragoslav; Stecher, Adela; Zver, Samo

    2012-01-01

    Fresh frozen plasma (FFP) is a blood component obtained from processed whole blood or collected by plasmapheresis and afterwards stored in frozen state. Indications for the clinical use of FFP are single coagulation factor deficiencies (such as F V and F XI), multiple coagulation factors deficiency (most frequent as a consequence of massive hemorrhage), coagulopathy caused byadvanced liver disease, warfarin mediated coagulopathy and thrombotic thrombocytopenic purpura. Standard FFP treatment ...

  11. 5-Aminolevulinic acid regulates the inflammatory response and alloimmune reaction.

    Science.gov (United States)

    Fujino, Masayuki; Nishio, Yoshiaki; Ito, Hidenori; Tanaka, Tohru; Li, Xiao-Kang

    2016-08-01

    5-Aminolevulinic acid (5-ALA) is a naturally occurring amino acid and precursor of heme and protoporphyrin IX (PpIX). Exogenously administrated 5-ALA increases the accumulation of PpIX in tumor cells specifically due to the compromised metabolism of 5-ALA to heme in mitochondria. PpIX emits red fluorescence by the irradiation of blue light and the formation of reactive oxygen species and singlet oxygen. Thus, performing a photodynamic diagnosis (PDD) and photodynamic therapy (PDT) using 5-ALA have given rise to a new strategy for tumor diagnosis and therapy. In addition to the field of tumor therapy, 5-ALA has been implicated in the treatment of inflammatory disease, autoimmune disease and transplantation due to the anti-inflammation and immunoregulation properties that are elicited with the expression of heme oxygenase (HO)-1, an inducible enzyme that catalyzes the rate-limiting step in the oxidative degradation of heme to free iron, biliverdin and carbon monoxide (CO), in combination with sodium ferrous citrate (SFC), because an inhibitor of HO-1 abolishes the effects of 5-ALA. Furthermore, NF-E2-related factor 2 (Nrf2), mitogen-activated protein kinase (MAPK), and heme are involved in the HO-1 expression. Biliverdin and CO are also known to have anti-apoptotic, anti-inflammatory and immunoregulatory functions. We herein review the current use of 5-ALA in inflammatory diseases, transplantation medicine, and tumor therapy. PMID:26643355

  12. Platelet transfusion and alloimmunization : clinical and laboratory studies

    NARCIS (Netherlands)

    K. Sintnicolaas (Krijn)

    1996-01-01

    textabstractThrombocytopenia as a result of decreased platelet production may be treated with platelet transfusions. Decreased bone marrow function resulting in thrombocytopenia may be seen in hematopoietic diseases as leukemia, myelodysplastic syndrome or conditions of infiltration of bone marrow w

  13. Platelet transfusion and alloimmunization : clinical and laboratory studies

    OpenAIRE

    Sintnicolaas, Krijn

    1996-01-01

    textabstractThrombocytopenia as a result of decreased platelet production may be treated with platelet transfusions. Decreased bone marrow function resulting in thrombocytopenia may be seen in hematopoietic diseases as leukemia, myelodysplastic syndrome or conditions of infiltration of bone marrow with solid tumor metastases. Thrombocytopenia also is a frequent side effect of cancer therapy. The application of high doses of chemotherapy and/or radiotherapy may result in periods of severe bone...

  14. Immunoregulatory Effects of Everolimus on In Vitro Alloimmune Responses

    Science.gov (United States)

    Levitsky, Josh; Miller, Joshua; Huang, Xuemei; Gallon, Lorenzo; Leventhal, Joseph R.; Mathew, James M.

    2016-01-01

    Everolimus (EVL) is a novel mTOR-inhibitor similar to sirolimus (SRL) that is used in organ transplant recipients, often in combination with tacrolimus (TAC) or mycophenolate (MPA). The current study aims to determine its effects on regulatory T cells. Increasing concentrations of EVL, MPA and TAC alone or in combination were added to MLRs of healthy volunteers. Lymphoproliferation by 3H-TdR incorporation and the percentage of newly generated CD4+CD127-CD25+FOXP3+ (total Treg) and CD4+CD127-CD25HighFOXP3+ (natural Treg) in CFSE labeled responder cells were assessed by flow cytometry. In comparison to medium controls, EVL and other agents dose-dependently inhibited 3H-TdR incorporation in HLA-2DR-matched and HLA-mismatched MLRs (n = 3–10). However, EVL significantly amplified newly generated total and natural Tregs in CFSE labeled responder cells (p SRL> MPA > TAC. If the results from these in vitro studies are extrapolated to clinical transplantation, it would suggest EVL plus low concentrations of MPA may be the most tolerogenic combination. PMID:27275747

  15. Immunoregulatory Effects of Everolimus on In Vitro Alloimmune Responses.

    Directory of Open Access Journals (Sweden)

    Josh Levitsky

    Full Text Available Everolimus (EVL is a novel mTOR-inhibitor similar to sirolimus (SRL that is used in organ transplant recipients, often in combination with tacrolimus (TAC or mycophenolate (MPA. The current study aims to determine its effects on regulatory T cells. Increasing concentrations of EVL, MPA and TAC alone or in combination were added to MLRs of healthy volunteers. Lymphoproliferation by 3H-TdR incorporation and the percentage of newly generated CD4+CD127-CD25+FOXP3+ (total Treg and CD4+CD127-CD25HighFOXP3+ (natural Treg in CFSE labeled responder cells were assessed by flow cytometry. In comparison to medium controls, EVL and other agents dose-dependently inhibited 3H-TdR incorporation in HLA-2DR-matched and HLA-mismatched MLRs (n = 3-10. However, EVL significantly amplified newly generated total and natural Tregs in CFSE labeled responder cells (p SRL> MPA > TAC. If the results from these in vitro studies are extrapolated to clinical transplantation, it would suggest EVL plus low concentrations of MPA may be the most tolerogenic combination.

  16. Curative effect observation of glucocorticoid combined with oral cyclophosphamide in the treatment of henoch-schonlein purpura in 63 cases%糖皮质激素与口服环磷酰胺联合治疗过敏性紫癜63例疗效观察

    Institute of Scientific and Technical Information of China (English)

    马春华

    2013-01-01

    Objective To discuss the clinical therapeutic effect of glucocorticoid combined with oral cyclophosphamide in the treatment of henoch-schonlein purpura. Methods The therapeutic effect of 63 children with henoch-schonlein purpura who were treated with glucocorticoid combined with oral cyclophosphamide in our hospital were retrospectively analyzed. Results The skin purpura, abdominal pain and joint pain symptoms of all children at 5-10 days after glucocorticoid combined with oral cyclophosphamide treatment disappeared completely, proteinuria and hematuria disappeared within 3-12 weeks, and the total effective rate was 100%;12 cases relapsed within a year after discontinue medication, all relapsed cases had not been used cyclophosphamide treatment and were given cyclophosphamide combined with prednisone treatment. After 4-8 weeks of the treatment, all children had been cured, and no relapse occurred within 1 year follow-up. In this study, there were 51 cases(80.95%) of recovery and 12 cases(19.05%) in effective, no a case invalid. Conclusion Glucocorticoid combined with oral cyclophosphamide in the treatment of henoch-schonlein purpura is safe, reliable and worthy of clinical widely popularization.%目的探讨糖皮质激素与口服环磷酰胺联合使用对过敏性紫癜的临床治疗效果。方法回顾分析我院收治的63例发生过敏性紫癜的患儿临床采用糖皮质激素与口服环磷酰胺进行治疗的效果。结果本研究选取的所有患儿在经过糖皮质激素或环磷酰胺治疗5~10d后,皮肤紫癜、腹痛、关节痛等症状均已完全消失,3~12周内蛋白尿、血尿消失,总有效率为100%;停药后1年内有12例患儿复发,所有复发者均没有采用环磷酰胺进行治疗,对于复发的患儿均采用环磷酰胺加泼尼松进行联合治疗,经过4~8周治疗后,所有患儿均已痊愈,随访1年,均没有发生复发,在本研究中,痊愈患儿有51例,占80.95%;有效12

  17. 紫癜性肾炎患儿医院感染相关因素分析及护理%Related factors of nosocomial infections in children with Henoch-Schonlein purpura nephritis and the nursing countermeasures

    Institute of Scientific and Technical Information of China (English)

    王妍炜

    2013-01-01

    目的 分析紫癜性肾炎患儿医院感染的特点及相关因素,以制定相应对策,从而有效降低医院感染发生率.方法 回顾性分析2011年1月-2012年2月儿科住院的178例紫癜性肾炎患儿的临床资料.结果 178例患儿发生医院感染37例,感染率为20.8%;感染部位前3他为呼吸道、泌尿道和皮肤软组织,呼吸道感染最高,占40.5%;检出病原菌以革兰阴性杆菌为主占68.4%;侵入性操作、激素和免疫抑制剂的使用、住院时间与医院感染的关系密切.结论 重点关注病情严重、病程长、免疫力低下、肾脏穿刺的患儿,加强病房管理和环境监测,建立侵入性操作的护理规范,严格执行无菌操作,加强洗手消毒,做好健康宣教,防止交叉感染等均是预防医院感染的关键.%OBJECTIVE To analyze the characteristics and related factors of the nosocomial infections in the children with Henoch-Schonlein purpura nephritis (HSPN) so as to put forward the corresponding countermeasures to reduce the incidence of the nosocomial infections.METHODS The clinical data of 178 cases of HSPN chilren who hospitalized in the pediatrics department from Jan 2011 to Feb2012 were retrospectively analyzed.RESULTS of totally 178 cases of children investigated,the nosocomial infections occurred in 37 case-time of patients with the case-time infection rate of 20.8%.The respiratory tract,urinary tract,and the skin soft tissue were the top three infection sites,the cases with the respiratory tract infections accounted for 68.4%.The invasive operation,use of hormones and immunosuppressive agents,and hospitalization duration were closely related to the nosocomial infections.CONCLUSION Paying attention to the children with severe condition,long treatment course,low immunity,and renal biopsy,strengthening the ward management and monitoring of environment to establish the nursing standards of the invasive operation,strictly implementing the aseptic

  18. The correlation between HLA-A allele and anaphylactoid purpura in children of Mongolia%蒙古族儿童过敏性紫癜与HLA-A基因的关联性

    Institute of Scientific and Technical Information of China (English)

    刘春枝; 任少敏; 马超

    2010-01-01

    目的 探索内蒙地区蒙古族儿童过敏性紫癜(AP)与HLA-A基因的关联性.可望找出相关基因,以探寻其部分发病机理及防治前景.方法 采用病例对照研究策略,引入PCR-SSO技术,在祖籍三代居住内蒙地区,无血缘关系,无与异族通婚史及其他免疫性疾病史和家族史的蒙古族人群中,选择儿童AP病例组56例和健康儿童对照组66名,作HLA-A等位基因的型别分析.基因频率比较在单因素四格表χ2或Fisher检验的基础上,作以Logistic多因素回归.结果 病例组HLA-A*11基因频率为16.1%,与对照组的9.1%比较,Wald为3.954,P为0.047,差异有统计学意义.B为0.844>0,OR为2.325>1,促进发病,其95%可信区间为1.012~5.340,其内不包含1,与P意义相符,有统计学意义.EF为0.342>0.结论 HLA-A*11等位基因可能是内蒙地区蒙古族儿童AP发病单体型中的一个遗传易感基因.%Objective To explore the association between HLA -A gene and anaphylactoid purpura(AP) in children of Mongolia in Inner Mongolia. To find correlated genes and study part of pathogenesis and the method of prevention and cure of AP. Methods The method of case control was adopted and selected 56 children with AP as case group and 66 health children as control group in Mongolia,who had resided in Inner Mongolia three generations without consanguinity, history of mixed, marriages, other medical history , and family history of immunity,led into polymerase chain reaction sequence specific oligonucleotide probes technique, analyzed the type of HLA-A gene. The compare of gene frenquency made with logistic regression after χ2 or Fisher test. Results The gene frenquency of HLA- A * 11 ( 16. 1% ) allele in case group compared to that of control group( 9. 1% ) ,Wald of HLA-A * 11 gene was 3. 954 ,P =0. 047, the difference had statistical significance. B = 0. 844 > 0, OR = 2. 325 > 1, it helped development of the disease,which 95%confident interval was 1. 012-5.340,which did not

  19. Clinical Manifestations and Endoscopic and Pathological Features of Abdominal Type Henoch-Schönlein Purpura in Adults%成人腹型过敏性紫癜的临床、内镜及病理学特点

    Institute of Scientific and Technical Information of China (English)

    孟灵梅; 周丽雅; 丁士刚; 金珠

    2015-01-01

    目的:探讨成人腹型过敏性紫癜的临床表现和内镜特点,提高其诊断水平。方法回顾性分析我院1994年1月~2014年9月收治的98例成人腹型过敏性紫癜的临床、内镜及病理学资料,其中58例接受胃镜检查,54例接受肠镜检查。结果①症状主要为腹痛98例(100%)、皮肤紫癜98例(100%)、消化道出血51例(52%),伴关节肿痛23例(23%)。②实验室检查主要为外周血白细胞增高(55例,56%),C反应蛋白升高(20例,20%),白蛋白降低(10例,10%)。20%(20/98)B超提示腹腔积液,100%(32/32)CT提示肠壁增厚。③58例胃镜下主要表现:53例(91%)为略高出黏膜的点状出血和淤斑或成片状,39例(67%)糜烂或多发溃疡伴出血,7例(12%)颗粒状或结节状增生,7例(12%)弥漫性出血。病变在十二指肠降部出现率最高且最严重(39例,67%),其次为十二指肠球部(35例,60%)、胃(23例,40%)。病理组织学呈毛细血管炎表现12例(21%),3例(5%)伴幽门螺杆菌感染,其余均为黏膜慢性炎,无特异性表现。④54例肠镜下主要表现:42例(78%)为略高出黏膜的点状出血和淤斑或成片状,16例(30%)糜烂或多发溃疡伴出血,9例(17%)弥漫性出血。病变在回肠末端出现率最高且最严重(38例,70%),其次为直肠(16例,30%)、盲肠(11例,20%)、乙状结肠(4例,7%)、全结肠(4例,7%)、回盲瓣(2例,4%)。组织病理学均为黏膜慢性炎,无特异性表现。结论十二指肠降部、回肠末端病变在成人过敏性紫癜常见,内镜检查对成人以腹痛为主要表现的过敏性紫癜有较高的诊断价值。%Objective To discuss the clinical manifestations and endoscopic and pathological features of abdominal type Henoch-Schönlein purpura ( HSP) in adults

  20. Henoch-Schönlein Purpura

    Science.gov (United States)

    ... term complication. In adults, HSP can lead to chronic kidney disease (CKD) and kidney failure, described as end-stage ... a pathologist—a doctor who specializes in diagnosing diseases. A kidney ... uses imaging techniques such as ultrasound or a computerized tomography ...