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Sample records for alloimmune thrombocytopenic purpura

  1. 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 ...

  2. 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 ...

  3. 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, ...

  4. 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.

  5. Atorvastatin-related thrombocytopenic purpura

    OpenAIRE

    Narayanan, Deepa; Kilpatrick, Eric S.

    2010-01-01

    A 44-year-old male patient with a single vessel ischaemic heart disease was referred to the lipid clinic for management of hypercholesterolaemia after an episode of admission with thrombocytopenic purpura secondary to atorvastatin. Atorvastatin was discontinued and his platelet counts improved gradually with steroids. He is now established on a different statin with no further episodes of thrombocytopenia. Though a drug challenge was never done, an idiosyncratic reaction to the initial statin...

  6. 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

  7. 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 ...

  8. 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

  9. Acute Pancreatitis-Induced Thrombotic Thrombocytopenic Purpura

    Directory of Open Access Journals (Sweden)

    Mohan Gurjar

    2012-01-01

    Full Text Available Context Acute pancreatitis due to thrombotic thrombocytopenic purpura is a well recognized condition. Here, we are reporting a rare converse phenomenon, in which thrombocytopenic purpura occurred secondary to acute pancreatitis. Case report A 19-year-old male referred to our intensive care unit with diagnosis of acute pancreatitis with multi-organ dysfunction. He had history of severe abdominal pain and recurrent vomiting about one month ago, requiring hospital admission. There, on diagnostic work-up at admission, abdominal ultrasonography was suggestive of pancreatitis. His serum amylase and lipase were 1,900 and 1,582 U/L, respectively. Other laboratory parameters were within normal limits. He was managed conservatively with intravenous fluids, antibiotics and analgesics; and discharged after about 2 weeks One week after discharge he was readmitted in same hospital with abdominal pain, multiple episodes of bilious vomiting and abdominal distention. Later on he was referred to our intensive care unit; having classical pentad of thrombocytopenic purpura, i.e., thrombocytopenia, micro-angiopathic hemolytic anemia, renal failure, encephalopathy, and fever. His condition improved with plasma exchange therapy and transferred out from our ICU to ward after 10 days of stay. Conclusion Thrombocytopenic purpura may be precipitate by acute pancreatitis due to multiple mechanisms. A high clinical suspicion is required to make an early diagnosis and allow early initiation of plasma exchange therapy, resulting in a goodprognosis.

  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. Idiopathic thrombocytopenic purpura and MMR vaccine

    OpenAIRE

    Miller, E; Waight, P; Farrington, C.; Andrews, N.; Stowe, J; Taylor, B

    2001-01-01

    A CAUSAL ASSOCIATION BETWEEN MEASLES—mumps-rubella (MMR) vaccine and idiopathic thrombocytopenic purpura (ITP) was confirmed using immunisation/hospital admission record linkage. The absolute risk within six weeks of immunisation was 1 in 22 300 doses, with two of every three cases occurring in the six week post-immunisation period being caused by MMR. Children with ITP before MMR had no vaccine associated recurrences.



  13. 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

  14. [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...

  15. Idiopathic Thrombocytopenic Purpura Misdiagnosed as Hereditary Angioedema

    Science.gov (United States)

    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 however not always have to be caused by angioedema but can relate to other concomitant disorders. In this report we are focusing on misdiagnosis in a patient with known hereditary angioedema, whose bleeding episode caused by idiopathic thrombocytopenic purpura was mistaken for an acute attack of hereditary angioedema. The case illustrates how clinicians can have difficulties in handling patients with rare diseases, especially in the emergency care setting. PMID:26819784

  16. 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...... however not always have to be caused by angioedema but can relate to other concomitant disorders. In this report we are focusing on misdiagnosis in a patient with known hereditary angioedema, whose bleeding episode caused by idiopathic thrombocytopenic purpura was mistaken for an acute attack of...... hereditary angioedema. The case illustrates how clinicians can have difficulties in handling patients with rare diseases, especially in the emergency care setting....

  17. 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

  18. 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

  19. 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

  20. 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

  1. 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.

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

    Directory of Open Access Journals (Sweden)

    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. 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...

  4. 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

  5. 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...

  6. 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.



  7. Pregnancy outcomes in women with idiopathic thrombocytopenic purpura

    OpenAIRE

    Yassaee, Fakhrolmolouk; Eskandari, Roghieh; Amiri, Zohreh

    2012-01-01

    Background: Idiopathic thrombocytopenic purpura (ITP) is a disease that commonly affects women of reproductive age and is associated with maternal and fetal complications. Objective: The aim of the present study was to report the perinatal outcome in pregnant women with ITP. Materials and Methods: Twenty one pregnant women with ITP admitted in a teaching hospital in Tehran, from October 2008 to February 2010, were enrolled in this prospective historical cohort study; course and perinatal outc...

  8. Perioperative Care of a Patient with Refractory Idiopathic Thrombocytopenic Purpura Undergoing Total Knee Arthroplasty

    OpenAIRE

    Singhal, Rohit; Gudimetla, Veera; Stewart, Andrew; Luscombe, Karen L; Charalambous, Charalambos P.

    2012-01-01

    Idiopathic thrombocytopenic purpura (ITP) is an autoimmune disorder leading to low platelet count and an increased risk of bleeding. Major joint replacement surgery in a patient with ITP can be associated with severe postoperative bleeding. We present our experience of perioperative management in a patient with severe refractory chronic idiopathic thrombocytopenic purpura who successfully underwent a cemented total knee replacement.

  9. Immune thrombocytopenic purpura in a child with acute lymphoblastic leukemia and mumps.

    Science.gov (United States)

    Kurekci, A Emin; Atay, A Avni; Demirkaya, Erkan; Sarici, S Umit; Ozcan, Okan

    2006-03-01

    Immune thrombocytopenic purpura in childhood is characterized by a typical history of acute development of purpura and bruising in an otherwise healthy child. In children it usually follows a viral infection (eg, mumps, rubella) or immunization. We report for the first time a child with acute lymphoblastic leukemia who developed immune thrombocytopenic purpura due to mumps during the maintenance phase of acute lymphoblastic leukemia treatment. PMID:16679943

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

    DEFF Research Database (Denmark)

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

    2009-01-01

    Thrombotic thrombocytopenic purpura (TTP) is a thrombotic microangiopathic disease. TTP is due to reduced activity of the von Willebrand factor which cleaves ADAMTS13. The disease is characterized by thrombocytopenia (<20 billion/l) intravascular Coombs-negative haemolysis and schistocytes in blood...... 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...

  11. [An unusual coincidence of thrombotic thrombocytopenic purpura and pernicious anemia].

    Science.gov (United States)

    Zamir, D; Polychuck, I; Reitblat, T; Leibovitz, I; Lugassy, G

    2002-08-01

    A 52 year old man was admitted for hospitalization due to dizziness and weakness that appeared in the previous 2 weeks. Anemia and thrombocytopenia, as well as elevated levels of lactic dehydrogenase, reticulocytosis and schistocytes on blood smear, all suggested thrombotic thrombocytopenic purpura. However, B12 deficiency was also diagnosed. The diagnosis of pernicious anemia was reassured by both fundic biopsy and the existence of antiparietal cells antibodies and anti-intrinsic cells antibodies. A few courses of plasmapheresis along with parenteral B12 stabilized his physical condition and he was released with no need for further treatment, and only required ambulatory follow-up. PMID:12222131

  12. A Case of Immune Thrombocytopenic Purpura After Rabies Vaccination.

    Science.gov (United States)

    Fulbright, Joy M; Williams, Sarah E; Pahud, Barbara A

    2015-10-01

    We describe a case of immune thrombocytopenic purpura (ITP) occurring 15 days after the first dose of a 4-dose rabies vaccination series. ITP is thought to be an immune-mediated process triggered by an infection or toxin. There is little evidence in the literature beyond case reports of an association of ITP with vaccines other than with the measles, mumps, and rubella vaccine. This is the third reported case of ITP associated with rabies vaccination. Because of the rare occurrence of this adverse event relative to the severity of rabies infection, the benefits of rabies vaccination, when indicated, outweigh the low and possible risk of ITP. PMID:26165405

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

    Directory of Open Access Journals (Sweden)

    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.

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

    Directory of Open Access Journals (Sweden)

    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

  15. 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

  16. Treatment of immune thrombocytopenic purpura: focus on eltrombopag

    Directory of Open Access Journals (Sweden)

    Lawrence Rice

    2009-03-01

    Full Text Available Lawrence RiceWeill Cornell Medical College, The Methodist Hospital, Houston, Texas, USAAbstract: Immune thrombocytopenic purpura (ITP is a relatively common autoimmune disorder in which antibodies are produced to circulating platelets. Symptoms can be mild, but for most patients the risk of severe bleeding is unacceptable and treatment is required. Glucocorticoids followed by splenectomy had been the mainstays of therapy. High dose intravenous immunoglobulin and anti-RhD therapy are available for patients with severe illness, but produce only temporary benefit. Rituximab may provide more durable responses, danazol may be underutilized, and immunosuppressants and cytotoxic agents are less often required. Recently the pathophysiology of ITP has been more clearly elucidated, particularly the importance of decreased production of platelets in most patients and the very blunted rise that occurs in serum thrombopoietin (TPO. The isolation of TPO and better understanding of its role in thrombopoiesis has led to the development of new highly effective treatments. TPO analogs had some successes in treating highly refractory ITP patients but were taken out of development due to TPO-antibody induction. Two second-generation TPO-mimetics, romiplostim and the orally available eltrombopag, have recently been licensed in some territories for the treatment of ITP. Approval of eltrombopag was based on results from Phase II and III placebo-controlled clinical trials and a long-term extension study. About 80% of patients achieve significant increases in platelet count (11% of placebo patients, with reduced bleeding and reduced use of concomitant medications; responses are often durable with no tachyphylaxis. The side effects of eltrombopag are generally mild and not worse than placebo, although there are concerns about hepatic dysfunction, and the potentials for thromboses, marrow reticulin fibrosis, rebound thrombocytopenia and cataracts. This is an important new

  17. [Bronchiolitis obliterans with organizing pneumonia associated with idiopathic thrombocytopenic purpura].

    Science.gov (United States)

    Presas, J L; Piriz, J; Serra, S L; Paz, E D; Allievi, A; Kartin, D; Olmedo, G

    1998-01-01

    We report a case of a 35 year-old woman with idiopathic thrombocytopenic purpura (ITP) who, under treatment with immunosuppressive drugs, developed bilateral interstitial pulmonary disease. Previously she had been splenectomized and treated with corticosteroids and cyclosporin. During the clinical course, the patient developed alterations of the hepatogram and presented a positive serology for Epstein-Barr virus. The lung biopsy showed the histologic pattern of obliterative bronchiolitis, interstitial inflammatory infiltration and intraalveolar pneumonia (BOOP). We could not find in the literature a previous report in which ITP was associated with BOOP. Of interest was the spontaneous remission of the pulmonary disease after suppression of cyclosporin and positive serology for Epstein-Barr virus. PMID:9674210

  18. 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

  19. Postinfluenza Vaccination Idiopathic Thrombocytopenic Purpura in Three Elderly Patients

    Directory of Open Access Journals (Sweden)

    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.

  20. Dengue Virus Infection Triggering Thrombotic Thrombocytopenic Purpura in Pregnancy.

    Science.gov (United States)

    Deepanjali, Surendran; Naik, Raghuramulu R; Mailankody, Sharada; Kalaimani, Sivamani; Kadhiravan, Tamilarasu

    2015-11-01

    We report a case of thrombotic thrombocytopenic purpura (TTP) that immediately followed symptomatic dengue virus infection in a pregnant lady. The patient developed dengue fever at 16 weeks of gestation, resulting in spontaneous abortion. Subsequently, fever reappeared with persistent thrombocytopenia and jaundice. Investigations revealed microangiopathic hemolysis; there was no evidence of disseminated intravascular coagulation. The TTP episode resolved after six cycles of therapeutic plasma exchange with fresh-frozen plasma. An ADAMTS13 (a disintegrin and metalloprotease with thrombospondin type 1 motif 13 repeats) activity assay, done during convalescence, showed normal activity. The patient had an uneventful second pregnancy and has remained free of TTP recurrence for more than 2 years now. We review the pathophysiological basis of TTP in dengue infection, and suggest that jaundice with disproportionate elevation of serum aspartate aminotransferase level in a patient with dengue should arouse the suspicion of TTP. PMID:26283741

  1. Successful management of thrombotic thrombocytopenic purpura associated with pregnancy.

    Science.gov (United States)

    Uğur Bilgin, Aynur; Karaselek, Mehmet Ali; Camlı, Kazım

    2014-06-01

    Thrombotic thrombocytopenic purpura (TTP) is an uncommon, severe, potentially life-threatening disease characterized by microangiopathic hemolytic anemia, thrombocytopenia, altered mental status, fever, and renal abnormalities. It can be seen at any age or sex but affects women of childbearing age more commonly. Pregnancy is known as one of the most common precipitating events for the onset of TTP and occurs mostly in the late third trimester or during the puerperium. Because of relatively low prevalence of pregnancy-related TTP, here we report the clinical characteristics and successful outcomes of 7 women with pregnancy-related TTP. Median age of patients was 25 (19-32). While 4 out of 7 women were primiparous, others were multiparous. Total plasma exchange (TPE) procedure was started within 24h after admission to our hospital. All patients got into complete remission without any maternal mortality. Fetal mortality was found to be 28%. Pregnancy-related TTP is still associated with high maternal and fetal mortality rates. However, the prognosis of TTP has improved dramatically with early diagnosis and plasma- based therapies. PMID:24667160

  2. Pulmonary Hyalinizing Granuloma Associated with Idiopathic Thrombocytopenic Purpura

    Directory of Open Access Journals (Sweden)

    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.

  3. 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.

  4. 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

  5. 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

  6. 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.

  7. Improvement of thrombocytopenia after treatment for Helicobacter pylori in a patient with immunologic thrombocytopenic purpura

    Directory of Open Access Journals (Sweden)

    Aline Maia Rocha

    2014-04-01

    Full Text Available Immune thrombocytopenic purpura is the most common autoimmune hematologic disease, affecting individuals of different ages. Recently, the bacterium Helicobacter pylori entered the list of causes of immune thrombocytopenic purpura. Here we present the case of a 55-year-old female patient with low platelet counts initially attributed to chronic vaginal bleeding. As corticosteroid therapy was ineffective she was treated for H. pylori infection. Within four weeks the patient had a platelet count of 87.17 ×109/L accompanied by clinical improvement of the symptoms.

  8. 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

  9. 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.

  10. 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.

  11. Low serum levels of interleukin-6 in children with post-infective acute thrombocytopenic purpura.

    Science.gov (United States)

    Gangarossa, S; Romano, V; Munda, S E; Sciotto, A; Schilirò, G

    1995-08-01

    Interleukin-6 plays an important role in host defense mechanisms and it appears to be a major mediator of the acute-phase response. IL-6 is also an important thrombocytopoietic factor. High serum levels of IL-6 are present in reactive thrombocytosis. The number and function of circulating platelets are the major factors that affect megakaryocytopoiesis by thrombopoietin. High levels of thrombopoietin have been observed in patients with thrombocytopenic purpura. To evaluate a possible thrombopoietin-like function of IL-6, we measured IL-6 levels in the serum of patients affected by post-infective acute thrombocytopenic purpura using a sensitive ELISSA assay. As controls, we studied normal subjects and patients with reactive thrombocytosis. No significant difference was observed between thrombocytopenic patients and normal controls. High IL-6 levels were present in patients with reactive thrombocytosis. In conclusion, we had not observed high levels of IL-6 in acute thrombocytopenic purpura and, very probably, IL-6 is not involved in the regulation of platelet mass for the hemostatic function. The thrombocytopoietic activity of IL-6 is another acute-phase response and it is consistent with the other functions of this cytokine. This suggests an active participation of platelets in host defense mechanisms. PMID:7628586

  12. Thrombotic thrombocytopenic purpura: MRI demonstration of persistent small cerebral infarcts after clinical recovery

    International Nuclear Information System (INIS)

    Abnormalities in the brain of patients with thrombotic thrombocytopenic purpura (TTP) are infrequent on MRI, often reversible and mainly limited to symptomatic stages of the disease. We report a case in which high-resolution MRI demonstrated multiple persistent small cortical infarcts after clinical remission. High-resolution MRI investigations may detect clinically latent but permanent brain damage, and complement clinical judgement in guiding therapeutic decisions. (orig.)

  13. 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...

  14. 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

  15. 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...

  16. 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...

  17. 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...

  18. 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...

  19. 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

  20. 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

  1. 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.

  2. 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)

  3. [The dynamic detection of cytokins under aplastic anemia and idiopathic thrombocytopenic purpura in the process of impact of hypoxic hypoxia].

    Science.gov (United States)

    Éralieva, M O

    2012-05-01

    The natural hypoxic hypoxia enhances the synthesis processes and decrease the concentration balance in cytokine rets in patients with depression of hematopoiesis. It is established that the patients with aplastic anemia and idiopathic thrombocytopenic purpura, in contrast with patients with acute leucosis, chronic myelolecosis and erythroid myeloma, have quite high values of IL-2, which can be involved in the organization of full-fledgee response to antigen stimuli. The application of hypoxic hypoxia as an additional method in treatment of aplastic anemia and idiopathic thrombocytopenic purpura has no side effects and contraindications. PMID:22834158

  4. 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.

  5. 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...

  6. 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....

  7. 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.

  8. 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.

  9. 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 ...

  10. 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...

  11. 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

  12. Focal ulcerative ileocolitis with terminal thrombocytopenic purpura in juvenile cotton top tamarins (Saguinus oedipus).

    Science.gov (United States)

    Snook, S S; Canfield, D R; Sehgal, P K; King, N W

    1989-03-01

    A newly recognized syndrome characterized by an acute focal ulcerative ileocolitis, anemia and thrombocytopenic purpura in five juvenile cotton-top tamarins is described. The presentation and morphology of this syndrome is distinct from any other reported gastrointestinal disease reported in tamarins. Traditional etiologies such as viruses, ingested toxins, Campylobacter, Salmonella and Yersinia and Clostridium difficile are not considered likely etiologic agents. Nontraditional etiologies such as anaerobes or pathologic strains of Escherichia coli are now being considered. This syndrome is of potential significance to ongoing research into the etiology of idiopathic tamarin colitis. PMID:2496272

  13. 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.

  14. 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

  15. Prognostic Factors of Response to Laparoscopic Splenectomy in Patients with Idiopathic Thrombocytopenic Purpura

    OpenAIRE

    Kwon, Hyuk-Chan; Moon, Chang Hoon; Cho, Young Rak; Kim, Min Chan; Kim, Kyeong Hee; Han, Jin Yeong; Lee, Young Ho; Oh, Sung Yong; Kim, Sung-Hyun; Kim, Jae-Seok; Kim, Hyo-Jin

    2005-01-01

    Laparoscopic splenectomy (LS) has become the treatment of choice for patients with idiopathic thrombocytopenic purpura (ITP) who do not respond to medical treatment. The aim of this study was to identify factors predictive of outcome after LS for ITP. From May 1997 to December 2002, we performed 30 LS on patients with ITP. A positive response was defined as a postoperative platelet count greater than 50,000/µL and no requirement for maintenance therapy. Chi-square testing was performed to det...

  16. A report of three cases with thrombotic thrombocytopenic purpura (TTP) secondary to an occult gastric adenocarcinoma

    International Nuclear Information System (INIS)

    Thrombotic thrombocytopenic purpura (TTP) is a disseminated form of thrombotic microangiopathy with clinical findings consisting of fever, microangiopathic hemolytic anemia (MAHA), thrombocytopenia, fluctuating neurologic impairment and renal dysfunction. However, Microangiopathic hemolytic anemia has been described in association with disseminated malignancies, most commonly adenocarcinoma of the breast or stomach. We present three patients with microangiopathic anemia in whom metastatic cancer was finally diagnosed; however, they died of refractory hemolytic anemia in the end. The occurrence of microangiopathic hemolytic anemia and thrombocytopenia in patients with disseminated malignant in gastric adenocarsinoma is well documented. Therefore, the diagnosis of tumor-associated TTP should be considered in unresponsive TTP patient treated with plasmapheresis. (author)

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

    Directory of Open Access Journals (Sweden)

    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.

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

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    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.

  19. Controversies in the diagnosis and management of childhood acute immune thrombocytopenic purpura.

    Science.gov (United States)

    Segel, George B; Feig, Stephen A

    2009-09-01

    Acute immune thrombocytopenic purpura (ITP) occurs most commonly in young children who present with severe isolated thrombocytopenia and purpura. A marrow examination is not required unless glucocorticoids are used, lest treatment mask incipient acute lymphoblastic leukemia, but controversy exists here. The recommendations for evaluation and management remain controversial, since prospective controlled trials have not been done. There is some consensus based on experience and empiric data. Almost all children with acute ITP will recover completely without therapy. Although the various treatments may increase the platelet count, they do not influence the outcome of the illness, may increase cost, and cause significant side effects. Therefore, careful observation may be the best management option for the patient with ITP, in the absence of severe bleeding. The data available relevant to these issues are discussed. PMID:19165890

  20. 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

  1. Spontaneous bilateral peripapillary, subhyaloid and vitreous hemorrhage with only minor platelet deficit in idiopathic thrombocytopenic purpura

    Directory of Open Access Journals (Sweden)

    Wan-Wei L

    2014-01-01

    Full Text Available Loo Wan-Wei,1,2 Tuan-Jaffar Tengku-Norina,1 Ahmad-Alwi Azma-Azalina,1 Abdul-Ghani Zulkifli,1 Embong Zunaina21Department of Ophthalmology, Hospital Raja Perempuan Zainab II, Kota Bharu, 2Department of Ophthalmology, School of Medical Sciences, Health Campus, Universiti Sains Malaysia, Kubang Kerian, Kelantan, MalaysiaAbstract: A 45-year-old female with underlying idiopathic thrombocytopenic purpura (ITP complained of acute onset of reduced vision and floaters, in both eyes, for 3 weeks. Visual acuity was 6/36 and 6/60 in the right eye and left eye, respectively. Ophthalmoscopy showed bilateral peripapillary, subhyaloid and vitreous hemorrhage. Hematological evaluation revealed moderate anemia (hemoglobin: 93 g/L and mild thrombocytopenia (platelets: 120×109/L. She was co-managed by a hematologist and ophthalmologists; she was treated medically. Follow-up care during the next 6 weeks revealed spontaneous, partially resolving hemorrhage, with improvement of visual acuity. The purpose of this case report is to highlight ophthalmic involvement of ITP in this patient, despite her only-mild thrombocytopenia, and her spontaneous recovery, despite her receiving only medical treatment.Keywords: idiopathic thrombocytopenic purpura, subhyaloid hemorrhage, vitreous hemorrhage

  2. 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

  3. 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

  4. 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

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

    Directory of Open Access Journals (Sweden)

    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

  6. 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

  7. 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.

  8. 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

  9. Subcutaneous anti-D treatment of idiopathic thrombocytopenic purpura in children.

    Science.gov (United States)

    Kjaersgaard, Mimi; Edslev, Pernille Wendtland; Hasle, Henrik

    2009-12-15

    We investigated the effect of subcutaneous anti-D IgG as platelet enhancing therapy in children with idiopathic thrombocytopenic purpura (ITP). Twenty-three children were treated with subcutaneous anti-D 50 microg/kg. The median platelet count increased from 7 x 10(9) to 31 x 10(9)/L on day 3 (P < 0.01). The median decline in hemoglobin was 1.3 g/dl. Two children experienced minor fever and chills within 24 hr of treatment. Pain at the injection site was common but self-limiting with no effect on activity level. These results suggest subcutaneous anti-D IgG 50 microg/kg as an effective and well-tolerated treatment option in childhood ITP. PMID:19722275

  10. Using decision analysis techniques to deal with "unanswerable" questions in idiopathic thrombocytopenic purpura.

    Science.gov (United States)

    Klaassen, Robert

    2003-12-01

    Idiopathic thrombocytopenic purpura (ITP) is a common disorder with rare adverse outcomes. This makes it a particularly difficult area in which to undertake conventional studies. An alternative method for solving clinical questions is decision analysis, which is in essence a computer-assisted synthesis of the literature. Using the example of a newly diagnosed ITP patient, the author attempts to answer the question of whether a bone marrow aspirate (BMA) is required prior to starting steroids. Using decision analysis methodology, the author determines that BMA is not essential prior to starting steroids. More importantly, three variables critical to the decision-making process are determined: the risk of death from the BMA procedure, the altered chance of survival for a patient with acute lymphoblastic leukemia (ALL) inappropriately given steroids, and how sensitive the complete blood count is at determining the risk of ALL. This scenario demonstrates the value of decision analysis and lays the groundwork for future endeavors. PMID:14668643

  11. Refractory chronic immune thrombocytopenic purpura in a child with acute lymphoblastic leukemia.

    Science.gov (United States)

    Horino, Satoshi; Rikiishi, Takeshi; Niizuma, Hidetaka; Abe, Hiroshi; Watanabe, Yuko; Onuma, Masaei; Hoshi, Yoshiyuki; Sasahara, Yoji; Yoshinari, Miyako; Kazama, Takuro; Hayashi, Yutaka; Kumaki, Satoru; Tsuchiya, Shigeru

    2009-11-01

    Immune thrombocytopenic purpura (ITP) has been associated with several hematologic malignancies such as Hodgkin and non-Hodgkin lymphomas and chronic lymphocytic leukemia, but it is rare in children with acute lymphoblastic leukemia (ALL). Here, we report a 7-year-old girl with chronic ITP during early intensive phase of chemotherapy for ALL. She underwent splenectomy because thrombocytopenia had persisted even after treatment with intravenous immunoglobulin (IVIG), steroids, vincristine, rituximab, and anti-D antibody. After splenectomy, her platelet count had recovered, and maintenance therapy could be resumed with a support of IVIG. To our knowledge, this is the first child case of chronic ITP during chemotherapy for ALL and splenectomy was effective in this patient. PMID:19816666

  12. 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

  13. 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

  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. Immune thrombocytopenic purpura induced by intestinal tuberculosis in a liver transplant recipient.

    Science.gov (United States)

    Lugao, Renata dos Santos; Motta, Marina Pamponet; de Azevedo, Matheus Freitas Cardoso; de Lima, Roque Gabriel Rezende; Abrantes, Flávia de Azevedo; Abdala, Edson; Carrilho, Flair José; Mazo, Daniel Ferraz de Campos

    2014-07-01

    A variety of clinical manifestations are associated directly or indirectly with tuberculosis. Among them, haematological abnormalities can be found in both the pulmonary and extrapulmonary forms of the disease. We report a case of immune thrombocytopenic purpura (ITP) associated with intestinal tuberculosis in a liver transplant recipient. The initial management of thrombocytopenia, with steroids and intravenous immunoglobulin, was not successful, and the lack of tuberculosis symptoms hampered a proper diagnostic evaluation. After the diagnosis of intestinal tuberculosis and the initiation of specific treatment, a progressive increase in the platelet count was observed. The mechanism of ITP associated with tuberculosis has not yet been well elucidated, but this condition should be considered in cases of ITP that are unresponsive to steroids and intravenous immunoglobulin, especially in immunocompromised patients and those from endemic areas. PMID:25009408

  16. 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

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

    Directory of Open Access Journals (Sweden)

    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. A Case of Non-Hodgkin's Lymphoma in Patient with Coombs' Negative Hemolytic Anemia and Idiopathic Thrombocytopenic Purpura

    OpenAIRE

    Park, So Yeon; Kim, Soyon; Kim, Eun Sil; Choi, Soon Uk; Hyun, Hee Jae; Ahn, Ju Young; Lee, Ju Hyoung; Ryu, Seo Hee; Park, Jae Hyun; Lee, Gyeong In; Lee, Hyo Jin

    2012-01-01

    Coombs' negative autoimmune hemolytic anemia (AIHA) is a rare disease which shares similar clinical and hematological features with Coombs' positive AIHA, but its exact frequency remains unknown. There have been few reports of idiopathic thrombocytopenic purpura (ITP) and Coombs' negative AIHA associated with other lymphoproliferative disorders (LPDs). Since there is a well known association between LPDs and autoimmune phenomena, it is important to investigate the possibility of an underlying...

  19. Elevated common acute lymphoblastic leukemia antigen expression in pediatric immune thrombocytopenic purpura.

    Science.gov (United States)

    Cornelius, A S; Campbell, D; Schwartz, E; Poncz, M

    1991-01-01

    Bone marrow examination is often performed in thrombocytopenic children to distinguish immune thrombocytopenic purpura (ITP) from acute leukemia. We describe a patient with thrombocytopenia and 50% common acute lymphoblastic leukemia antigen (CALLA) positivity in his marrow who was subsequently shown to have ITP. CALLA (CD10) is a surface antigen found in early B-lymphocytes and is elevated in most cases of childhood acute lymphoblastic leukemia (ALL). This case prompted us to prospectively study the frequency of immature lymphocyte populations in children with ITP. Fourteen patients with acute ITP and five with other conditions were studied. The two groups were comparable with respect to age: ITP mean, 4.3 (range 0.3-15.5) years; control mean, 5.8 (0.6-13.8) years. The ITP group had a significantly higher percentage of CD10 positive bone marrow lymphocytes (p = 0.007). Five of the 10 patients younger than 4 years of age in the ITP group had CD10 levels of greater than 30%, which is in the leukemic range, whereas none of the control patients had a CD10 levels of greater than 17% (p = 0.003). There was good correlation between CD10 positivity and B4 positivity indicating that both of these markers arise from the same population of immature B-lymphocytes. None of the ITP patients who were older than 4 years had a CD10 level of greater than 30%. We conclude that it is common to have an increase in the proportion of immature lymphocytes in the marrow of young children with ITP. The cause of this increase in CD10 positive cells is unknown.(ABSTRACT TRUNCATED AT 250 WORDS) PMID:1827572

  20. Immune thrombocytopenic purpura (ITP) associated with vaccinations: a review of reported cases.

    Science.gov (United States)

    Perricone, Carlo; Ceccarelli, Fulvia; Nesher, Gideon; Borella, Elisabetta; Odeh, Qasim; Conti, Fabrizio; Shoenfeld, Yehuda; Valesini, Guido

    2014-12-01

    Immune thrombocytopenic purpura (ITP) is an autoimmune condition characterized by low platelet count with mucocutaneous and other bleedings. Clinical manifestations may range from spontaneous formation of purpura and petechiae, especially on the extremities, to epistaxis, bleeding at the gums or menorrhagia, any of which occur usually if the platelet count is below 20,000 per μl. A very low count may result in the spontaneous formation of hematomas in the mouth or on other mucous membranes. Fatal complications, including subarachnoid or intracerebral, lower gastrointestinal or other internal bleeding can arise due to an extremely low count. Vaccines may induce ITP by several mechanisms. Vaccine-associated autoimmunity may stem not only from the antigen-mediated responses but also from other constituents of the vaccine, such as yeast proteins, adjuvants, and preservatives diluents. The most likely is through virally induced molecular mimicry. The binding of pathogenic autoantibodies to platelet and megakaryocytes may cause thrombocytopenia by different mechanisms, such as opsonization, direct activation of complement, or apoptotic pathways. The autoantibodies hypothesis is not sufficient to explain all ITP cases: In the anti-platelet antibody-negative cases, a complementary mechanism based on T cell immune-mediated mechanism has been suggested. In particular, T cell subsets seem dysregulated with an increased production of pro-inflammatory cytokines, as IFN-γ and TNF, and chemokines, as CXCL10. Vaccines are one of the most striking discoveries in human history that changed dramatically life expectancy. Nonetheless, the occurrence of adverse events and autoimmune phenomena has been described following vaccination, and ITP may represent one of this. PMID:25427992

  1. Acquired thrombotic thrombocytopenic purpura after vascular prosthesis implantation for impending rupture of an abdominal aortic aneurysm.

    Science.gov (United States)

    Naito, Chiaki; Ogawa, Yoshiyuki; Yanagisawa, Kunio; Ishizaki, Takuma; Mihara, Masahiro; Handa, Hiroshi; Isonishi, Ayami; Hayakawa, Masaki; Matsumoto, Masanori; Nojima, Yoshihisa

    2016-01-01

    Acquired thrombotic thrombocytopenic purpura (TTP) is caused by autoantibodies against ADAMTS13. TTP patients run a rapidly fatal course unless immediate plasma exchange (PEX) is initiated upon diagnosis. Herein, we report a 72-year-old man with TTP, which developed after he underwent artificial blood vessel replacement surgery for an abdominal aneurysm with impending rupture. In the perioperative period, the patient received several platelet transfusions for severe thrombocytopenia (minimum platelet count: 0.6×10(4)/μl). Thereafter, he was admitted to our department for rapidly progressing coma with multiple cerebral infarctions, and was transferred to the ICU. Based on the tentative diagnosis of TTP, we immediately began PEX and steroid pulse therapy. The diagnosis was confirmed thereafter by markedly reduced ADAMTS13 activity (<0.5%) and his being positive for the ADAMTS13 inhibitor. We performed PEX for five consecutive days and administered high-dose prednisolone (PSL). On the second hospital day (HD), his platelet count rose along with improvement of his consciousness level. The ADAMTS13 inhibitor was not detected on the 10th HD. TTP did not relapse and his general condition improved despite tapering of PSL. In this case, by closely monitoring ADAMTS13-related parameters and minimizing the number of plasma exchanges, the patient was able to achieve a remission without the use of boosting inhibitors. PMID:27076251

  2. [Acquired von Willebrand syndrome in a patient with immune thrombocytopenic purpura].

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    Ihara, Akihiro; Suzuki, Nobuaki; Matsushita, Tadashi; Ichinose, Akitada

    2015-07-01

    Acquired von Willebrand syndrome (AVWS) is a rare bleeding disorder similar to inherited von Willebrand disease. We describe a 78-year-old woman with coexistent idiopathic thrombocytopenic purpura (ITP) and AVWS. The patient had once been admitted to our hospital because of cerebral infarction. Her platelet count had been normal at that time. Ten years later, she showed a severe bleeding tendency (platelet count 3.2×10(4)/μl). Analysis of hemostatic parameters showed very low (IgG4) to VWF was detected by enzyme linked immunosorbent assay (ELISA). Factor XIII activity was 42%. Treatment with corticosteroids did not improve the thrombocytopenia, but did correct the bleeding diathesis. Also, VWF: Rco and VIII: C showed normalization. These findings indicated that the patient had ITP associated with AVWS. All reported cases of AVWS associated with systemic lupus erythematosus were cured by appropriate treatment of the underlying autoimmune disease with prednisone or immunosuppression. This bleeding disorder occurs mainly in patients with lymphoproliferative, myeloproliferative, cardiovascular and immunologic disorders, but no patients with ITP have previously been reported. This patient had the rare presentation of AVWS complicated by ITP and factor XIII deficiency. PMID:26256928

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

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    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

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

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    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.

  5. Refractory thrombotic thrombocytopenic purpura associated with primary Sjogren syndrome treated with rituximab: a case report.

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    Toumeh, Anis; Josh, Navpreet; Narwal, Rawan; Assaly, Ragheb

    2014-01-01

    Thrombotic thrombocytopenic purpura (TTP) is an uncommon, serious disease that involves multiple organs and is rapidly fatal if left untreated. TTP is associated with multisystem symptoms, such as thrombocytopenia, microangiopathic hemolytic anemia, renal impairment, central nervous system involvement, and fever. TTP is idiopathic in about 37% of the cases and can be associated with autoimmune diseases in 13% of the cases. Autoimmune disease-associated TTP can be refractory to plasma exchange and requires immunosuppressive therapy. We report a case of a previously healthy 55-year-old African American female who presented with shortness of breath, hemolytic anemia, renal impairment, and thrombocytopenia. The diagnosis of TTP was made, and plasmapheresis was initiated. However, recurrence happened 48 hours after plasmapheresis was stopped. Autoimmune workup for refractory TTP revealed positive antinuclear antibodies, Anti-SSA, and Anti-SSB. Lip biopsy revealed findings consistent with Sjogren syndrome. Treatment with Rituximab was started, and significant clinical and laboratory response was achieved. The patient remained asymptomatic thereafter. A high clinical suspicion of autoimmune diseases is important as TTP tends to be refractory to plasma exchange in these cases, and immunosuppressive therapy is a key. PMID:23011161

  6. 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.

  7. 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.

  8. [Sudden death associated with myocardial damage caused by microthrombi in a patient with thrombotic thrombocytopenic purpura].

    Science.gov (United States)

    Yamamoto, Kiyoko; Hattori, Yukinori; Shimada, Koki; Araki, Yoko; Adachi, Tatsuya; Tsushita, Keitaro

    2015-11-01

    We describe a 35-year-old woman with Down's syndrome who was admitted to a clinic with anorexia and vomiting. Since laboratory findings showed anemia (Hb 7.4 g/dl) and thrombocytopenia (0.5 × 10⁴/μl), she was transferred to our hospital for treatment. Further laboratory examinations revealed schistocytes, LDH elevation, and a negative Coombs' test. Thrombotic thrombocytopenic purpura (TTP) was suspected. Plasma exchange (PEX) and prednisolone administration were thus immediately initiated. Prior to these treatments, ADAMTS13 activity was less than 5% and inhibitors were detected at a level of 0.8 Bethesda U/ml. Although her platelet count had risen to 13.0 × 10⁴/μl by day 6 (post 4 sessions of PEX), it had decreased to 1.8 × 10⁴/μl on day 7. Despite ongoing PEX, thrombocytopenia persisted. On day 21, she suddenly died. Autopsy findings revealed no evidence of myocardial necrosis or coronary artery thrombosis. Extensive microthrombi were, however, detected in precapillary arterioles, capillaries, and post-capillary venules of the heart. Therefore, this patient's sudden death was clinically suspected to have been caused by cardiomyopathy, which had produced cardiogenic shock. PMID:26666721

  9. Genetic variations in complement factors in patients with congenital thrombotic thrombocytopenic purpura with renal insufficiency.

    Science.gov (United States)

    Fan, Xinping; Kremer Hovinga, Johanna A; Shirotani-Ikejima, Hiroko; Eura, Yuka; Hirai, Hidenori; Honda, Shigenori; Kokame, Koichi; Taleghani, Magnus Mansouri; von Krogh, Anne-Sophie; Yoshida, Yoko; Fujimura, Yoshihiro; Lämmle, Bernhard; Miyata, Toshiyuki

    2016-03-01

    The congenital form of thrombotic thrombocytopenic purpura (TTP) is caused by genetic mutations in ADAMTS13. Some, but not all, congenital TTP patients manifest renal insufficiency in addition to microangiopathic hemolysis and thrombocytopenia. We included 32 congenital TTP patients in the present study, which was designed to assess whether congenital TTP patients with renal insufficiency have predisposing mutations in complement regulatory genes, as found in many patients with atypical hemolytic uremic syndrome (aHUS). In 13 patients with severe renal insufficiency, six candidate complement or complement regulatory genes were sequenced and 11 missense mutations were identified. One of these missense mutations, C3:p.K155Q mutation, is a rare mutation located in the macroglobulin-like 2 domain of C3, where other mutations predisposing for aHUS cluster. Several of the common missense mutations identified in our study have been reported to increase disease-risk for aHUS, but were not more common in patients with as compared to those without renal insufficiency. Taken together, our results show that the majority of the congenital TTP patients with renal insufficiency studied do not carry rare genetic mutations in complement or complement regulatory genes. PMID:26830967

  10. 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.

  11. 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

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

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    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

  13. 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

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

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    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

  15. The role of N-acetylcysteine in the treatment of thrombotic thrombocytopenic purpura.

    Science.gov (United States)

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

    2016-05-01

    Thrombotic thrombocytopenic purpura (TTP) is an acute, thrombotic microangiopathy with a high mortality rate if left untreated. Plasma exchange (PEX) is the current standard of care. However, a significant number of patients are refractory to this treatment. N-acetylcysteine (NAC) was recently suggested as a potential therapeutic adjunct for patients with TTP. This study reports a series of three patients with TTP successfully treated with NAC in addition to standard therapy. Detailed chart reviews on these patients were conducted. We discuss clinical features, laboratory findings and management of three patients who presented with microangiopathic hemolytic anemia and thrombocytopenia. Anti-ADAMTS13 antibodies and low levels of ADAMTS13 were detected and confirmed the diagnosis of acquired TTP. Based upon their severe presentation or lack of response to initial treatment with PEX, corticosteroids and other immunosuppressive agents, NAC was added. Under this combined treatment, all three patients hada significant clinical improvement of symptoms with concurrent normalization of platelet count and ADAMTS13 activity level. This report highlights the potential therapeutic utility of NAC in the treatment of TTP. Randomized controlled studies will be required to better characterize the risk-to-benefit ratio of NAC in the treatment of TTP. PMID:26245827

  16. Life-Threatening Autoimmune Hemolytic Anemia and Idhiopatic Thrombocytopenic Purpura. Successful Selective Splenic Artery Embolization

    Science.gov (United States)

    Molica, Matteo; Massaro, Fulvio; Annechini, Giorgia; Baldacci, Erminia; D’Elia, Gianna Maria; Rosati, Riccardo; Trisolini, Silvia Maria; Volpicelli, Paola; Foà, Robin; Capria, Saveria

    2016-01-01

    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. PMID:27158433

  17. 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

  18. 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.

  19. Two cases of Vici syndrome associated with Idiopathic Thrombocytopenic Purpura (ITP) with a review of the literature.

    Science.gov (United States)

    Huenerberg, Katherine; Hudspeth, Michelle; Bergmann, Shayla; Pai, Shashidhar; Singh, Balvir; Duong, Angie

    2016-05-01

    Vici syndrome is a rare congenital disorder first described in 1988. To date, 31 cases have been reported in the literature. The characteristic features of this syndrome include: agenesis of the corpus callosum, albinism, cardiomyopathy, variable immunodeficiency, cataracts, and myopathy. We report two Hispanic sisters with genetically confirmed Vici syndrome who both developed Idiopathic Thrombocytopenic Purpura. To our knowledge, this is an immunologic process that has been previously undescribed within the phenotype of Vici syndrome and should be added to the spectrum of variable immune dysregulation that can be found in these patients. © 2016 Wiley Periodicals, Inc. PMID:26854214

  20. 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

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

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    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

  2. 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

  3. Helicobacter pylori Eradication in Patients with Immune Thrombocytopenic Purpura: A Review and the Role of Biogeography.

    Science.gov (United States)

    Frydman, Galit H; Davis, Nick; Beck, Paul L; Fox, James G

    2015-08-01

    Idiopathic thrombocytopenic purpura (ITP) is typically a diagnosis of exclusion, assigned by clinicians after ruling out other identifiable etiologies. Since a report by Gasbarrini et al. in 1998, an accumulating body of evidence has proposed a pathophysiological link between ITP and chronic Helicobacter pylori (H. pylori) infection. Clinical reports have described a spontaneous resolution of ITP symptoms in about 50% of chronic ITP patients following empirical treatment of H. pylori infection, but response appears to be geography dependent. Studies have also documented that ITP patients in East Asian countries are more likely to express positive antibody titers against H. pylori-specific cytotoxic-associated gene A (CagA), a virulence factor that is associated with an increased risk for gastric diseases including carcinoma. While a definitive mechanism by which H. pylori may induce thrombocytopenia remains elusive, proposed pathways include molecular mimicry of CagA by host autoantibodies against platelet surface glycoproteins, as well as perturbations in the phagocytic activity of monocytes. Traditional treatments of ITP have been largely empirical, involving the use of immunosuppressive agents and immunoglobulin therapy. However, based on the findings of clinical reports emerging over the past 20 years, health organizations around the world increasingly suggest the detection and eradication of H. pylori as a treatment for ITP. Elucidating the exact molecular mechanisms of platelet activation in H. pylori-positive ITP patients, while considering biogeographical differences in response rates, could offer insight into how best to use clinical H. pylori eradication to treat ITP, but will require well-designed studies to confirm the suggested causative relationship between bacterial infection and an autoimmune disease state. PMID:25728540

  4. 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

  5. Phenotypic analysis of bone marrow lymphocytes from children with acute thrombocytopenic purpura.

    Science.gov (United States)

    Guiziry, Dalai E L; El, Gendy Wessam; Farahat, Nahla; Hassab, Hoda

    2005-01-01

    Hematogones are benign immature B cells that commonly populate the bone marrow of children. Their presence has been noted to interfere with the flow-cytometric analysis of acute lymphoblastic leukemia (ALL), because their immunophenotype is similar to B-precursor cell lymphoblasts. Immune-mediated thrombocytopenia is a clinical condition characterized by increased platelet destruction due to sensitization of platelets by autoantibodies. The aim of this study was to determine the incidence and clinical impact of bone marrow hematogones in cases of acute immune thrombocytopenic purpura (ITP) among children. This was done by immunophenotyping of bone marrow lymphocytes of ITP cases and controls and follow up of cases. This study was done on 25 cases of ITP, 12 females and 13 males, their age ranged from 2 to 13 years. A control group was included in the study, 15 cases of apparently healthy children with matching age and sex taken from among bone marrow donors. Cases and controls were subjected to bone marrow lymphocyte immunophenotyping with flow-cytometry to verify the presence of hematogones. A statistically significant increase in the percentage of hematogones was demonstrated in their bone marrows. An increased percentage of CD10+ lymphocytes was demonstrated; with a mean of 18+/-15.2%, CD19+ with a mean of 27+/-16.3% and CD34+ with a mean of 3.7+/-3.2%. No correlation was found between the percentage of hematogones and peripheral platelet count or bone marrow lymphocytic count. In conclusion, there is an increase in the bone marrow hematogones in ITP cases in comparison to normal controls. This could be the sequence of an immunological response to the cause which determined the disease, or the regeneration of the stem cell compartment following transient damage. PMID:16734134

  6. 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

  7. 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.

  8. 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

  9. An infantile case of cytomegalovirus induced idiopathic thrombocytopenic purpura with predominant proliferation of CD10 positive lymphoblast in bone marrow.

    Science.gov (United States)

    Mizutani, K; Azuma, E; Komada, Y; Ito, M; Sakurai, M; Hironaka, T; Hirai, K

    1995-02-01

    An infant with cytomegalovirus infection (CMV) developed idiopathic thrombocytopenic purpura (ITP) at 4 months of age. A bone marrow (BM) aspiration showed a remarkable increase of immature megakaryocytes and prominent proliferation of lymphoblasts. Flow cytometric analysis of the bone marrow cells showed that the predominant cells in the lymphocyte cluster were of B-lineage (CD19) with CD10 (common acute lymphoblastic leukemia antigen) positive. Virus study showed a higher titer of CMV antibody. Cytomegalovirus DNA was detected by the polymerase chain reaction (PCR) method in urine, peripheral cells and marrow cells. Low-grade fever, diarrhea and petechiae were accompanied by mild liver dysfunction. Complete remission was made with intravenous high-dose immunoglobulin (IVIg) without progression to overt acute leukemia. The percentage of CD10+/CD19+ lymphocytes in bone marrow also diminished. We postulated that the proliferation of immature lymphocytes and megakaryocytes in bone marrow was caused by maturation arrest that might result from CMV infection. PMID:7754772

  10. Histochemical and biochemical observations of the spleen in atypical Niemann-Pick disease and in idiopathic thrombocytopenic purpura.

    Science.gov (United States)

    Safanda, J; Fakan, F

    1981-01-01

    In a case of adolescent Niemann-Pick disease (NP) and in a case of idiopathic thrombocytopenic purpura (ITP), the histologic picture of the spleen showed appreciable similarity in localization of sparing cells and in a number of histochemical tests. The sphingomyelin, which was the main organ phospholipid in both conditions, contained substantially elevated content of C24 fatty acids. Detailed analysis of spleen lipids showed great relative increase of lysobisphosphatidic acid and of cholesterol which was in NP mainly in free form but in ITP surprisingly mainly esterified, mostly to oleic and palmitic acid. Possible molecular mechanism of sphingomyelin storage was enzymologically followed in model conditions using separated lipid fractions from NP's spleen. The activity of sphingomyelinase (Cl. perfringens exotoxin) was in comparison to phospholipase C relatively specifically inhibited by lysobisphosphatidic acid. PMID:6168156

  11. Low-dose vincristine in the treatment of corticosteroid-refractory idiopathic thrombocytopenic purpura (ITP) in non-splenectomized patients.

    Science.gov (United States)

    Cervantes, F.; Montserrat, E.; Rozman, C.; Diumenjo, C.; Feliu, E.; Grañena, A.

    1980-01-01

    Eight non-splenectomized patients with corticosteroid-refractory idiopathic thrombocytopenic purpura (ITP) were treated with low-dose vincristine (1 mg/week up to a total dose of 4 mg). Complete remission was achieved in 2 cases and partial remission in 3. Bleeding stopped in one patient who failed to remit. No statistical relationship was found between the response to vincristine and the duration of the disease or the corticosteroid-therapy. Side effects were only observed in one patient. By comparing these results with those reported in the literature, it can be inferred that low-dose vincristine may be useful in the management of corticosteroid-refractory ITP. PMID:7194478

  12. Validity of a procedure to identify patients with chronic idiopathic thrombocytopenic purpura in the Danish National Registry of Patients

    Directory of Open Access Journals (Sweden)

    Katrine Edith Klith Heden

    2009-02-01

    Full Text Available Katrine Edith Klith Heden, Annette Østergaard Jensen, Dora Körmendiné Farkas, Mette NørgaardDepartment of Clinical Epidemiology, Aarhus University Hospital, DenmarkBackground: Administrative data may be useful for epidemiological studies of chronic idiopathic thrombocytopenic purpura (ITP. However, the quality of the recorded diagnoses needs evaluation.Aim: We evaluated the validity in predicting chronic ITP of the International Classifi cation of Diseases (ICD-10 diagnoses of ITP in the Danish National Registry of Patients (NRP.Methods: We used the NRP to identify patients with ITP, according to code D69.3, from January 1, 1996 to December 31, 2007. We defined chronic ITP as lasting longer than 6 months by including only patients with 2 or more hospital ITP diagnoses over longer than 6 months. We confirmed diagnoses by evaluating each candidate chronic ITP patient’s medical chart and estimating the positive predictive value (PPV and 95% confidence interval (CI of the recorded NRP diagnostic code.Results: We identified 513 patients with chronic ITP in the NRP. We were able to retrieve the charts of 439. After evaluation of the charts, 410 patients were deemed to have a valid diagnosis of chronic ITP, yielding a PPV of 0.93 (95% CI: 0.91–0.96.Conclusion: The validity of this procedure to identify chronic ITP patients was high. The NRP is valid for epidemiological studies of patients with chronic ITP.Keywords: validity, idiopathic thrombocytopenic purpura, National Registry of Patients

  13. 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...

  14. Life-threatening postpartum hemolysis, elevated liver functions tests, low platelets syndrome versus thrombocytopenic purpura – Therapeutic plasma exchange is the answer

    OpenAIRE

    Prashant Nasa; J M Dua; Sudha Kansal; Geeta Chadha; Rajesh Chawla; Manav Manchanda

    2011-01-01

    The differential diagnosis of life-threatening microangiopathic disorders in a postpartum female includes severe preeclampsia-eclampsia, hemolysis, elevated liver functions tests, low platelets syndrome and thrombotic thrombocytopenic purpura. There is considerable overlapping in the clinical and laboratory findings between these conditions, and hence an exact diagnosis may not be always possible. However, there is considerable maternal mortality and morbidity associated with these disorders....

  15. 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)

  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. Clinical Features and Treatment Outcomes of Primary Immune Thrombocytopenic Purpura in Hospitalized Children Under 2-Years Old

    Science.gov (United States)

    Farhangi, H; Ghasemi, A; Banihashem, A; Badiei, Z; Jarahi, L; Eslami, G; Langaee, T

    2016-01-01

    Background Immune thrombocytopenic purpura (ITP) is the most prevalent cause of thrombocytopenia in children. Despite the importance of ITP in children under 2-years old, only a few publications are available in the literature.ITP usually presents itself as isolated thrombocytopenia and mucocutaneous bleeding. Materials and Methods This study was conducted on 187 under 2-year-old children diagnosed with ITP and treated at Dr. Sheikh Hospital from 2004 to 2011.In this retrospective study, clinical symptoms, laboratory findings, history of viral infections, vaccination history, and treatment efficacy in children under 2-years old with ITP were investigated.Patients were followed for one year after being discharged from the hospital. Results The risk of the disease developing into chronic form was higher in older children (0.001). ITP in children under 3-months old was significantly associated with vaccination (p=0.007). There was no significant differences between male and female patients in regards to newly diagnosed ITP, persistent, and chronic disease status (p = 0.21). No significant difference in bleeding symptoms was observed between patients under 3-months old and 3 to 24-months old (p=0.18). Conclusion Infantile ITP respond favorably to treatment. The risk of the disease developing into chronic form is higher in 3-to-24-month-old children compared to under-three-month olds.

  18. Congenital thrombotic thrombocytopenic purpura: Upshaw-Schulman syndrome: a cause of neonatal death and review of literature.

    Science.gov (United States)

    Sharma, Deepak; Shastri, Sweta; Pandita, Aakash; Sharma, Pradeep

    2016-06-01

    Thrombotic thrombocytopenic purpura (TTP) is a rare disorder in children characterized by microangiopathic hemolytic anemia (MAHA) and thrombocytopenia. The classic Moschcowitz Pentads of TTP include hemolytic anemia, with fragmentation of erythrocytes, thrombocytopenia, diffuse and non-focal neurologic findings, decrease renal function and fever. We report a newborn who was diagnosed with congenital TTP. The newborn was admitted at age of 40 h, in our hospital, in view of respiratory distress with impending respiratory failure and red colored urine. On examination, the newborn was febrile, tachypneic, had deep icterus, pallor and no hepatosplenomegaly. Family history was significant with one unexplained neonatal death at age of 24 with symptoms of red colored urine. Examination of peripheral smear was diagnostic with the presence of fragmented RBCS, giant but fewer platelets consistent with a diagnosis of MAHA. The diagnosis of TTP was confirmed with low ADAMTS activity and gene analysis showed c 2203 G > T-p.Glu735X (domain TSP1-2) mutation in exon 18 of ADAMTS 13 gene. The newborn had rapid deterioration, with respiratory distress and refractory shock leading to death. Post-mortem bone marrow done showed marrow hyperplasia. PMID:26365135

  19. 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

  20. Clinical significance of detecting soluble glycocalicin and thrombopoietin in the differential diagnosis of idiopathic thrombocytopenic purpura and aplstic anemia

    International Nuclear Information System (INIS)

    Objective: To investigate the clinical significance of detecting soluble platelet glycocalicin (sGC) and thrombopoietin (TPO) in the differential diagnosis of idiopathic thrombocytopenic purpura (ITP) and aplastic anemia (AA). Methods: Plasma sGC and serum TPO in 83 patients with ITP, 47 patients with AA and 50 normal individuals were detected by iminunoradiometric assay (IRMA) and enzymelinked immunosorbent assay (ELISA), respectively. Statistical analysis was performed using Q test and P value of 0.05). But serum TPO level in AA group was significantly higher than that in ITP and normal groups: (857.43 ± 228.43) ng/L vs (90.32 ± 39.43) ng/L and (70.29 ± 25.16) ng/L, and they were considered statistically significant (Q=24.45 and 18.25, both P < 0.01). Conclusion: Detecting plasma sGC and serum TPO might be helpful for differentiating ITP and AA and for understanding the pathophysiology of thrombocytopenia. (authors)

  1. New advances in the treatment of adult chronic immune thrombocytopenic purpura: role of thrombopoietin receptor-stimulating agents

    Directory of Open Access Journals (Sweden)

    Ara Metjian

    2009-12-01

    Full Text Available Ara Metjian1, Charles S Abrams21Department of Medicine, Duke University Medical Center, Durham, NC, USA; 2Department of Medicine, University of Pennsylvania, Philadelphia, PA, USAAbstract: Decades of basic science and clinical research have led to an increased understanding of the pathophysiology of immune thrombocytopenic purpura (ITP, the processes underlying thrombopoiesis, and the treatment of chronic ITP. Now, new agents are available to treat ITP in a nonimmunosuppressive fashion. Lessons learned from the clinical trials of recombinant human thrombopoietin (TPO have led to the development of a novel class of compounds: nonimmunogenic agonists of the thrombopoietin receptor. Representing the first nonimmunosuppressive agents to treat chronic refractory ITP in decades, medications such as romiplostim and eltrombopag were recently approved by the US Food and Drug Administration. These new agents offer physicians a new tool for treating difficult cases of ITP in their medical armamentarium. Additional TPO mimetics are also being developed that show promise in vitro, and await future development.Keywords: thrombocytopenia, immune-mediated thrombocytopenia, eltrombopag, romiplostim, thrombopoietin, thrombopoietin receptor

  2. Life-threatening postpartum hemolysis, elevated liver functions tests, low platelets syndrome versus thrombocytopenic purpura - Therapeutic plasma exchange is the answer

    Directory of Open Access Journals (Sweden)

    Prashant Nasa

    2011-01-01

    Full Text Available The differential diagnosis of life-threatening microangiopathic disorders in a postpartum female includes severe preeclampsia-eclampsia, hemolysis, elevated liver functions tests, low platelets syndrome and thrombotic thrombocytopenic purpura. There is considerable overlapping in the clinical and laboratory findings between these conditions, and hence an exact diagnosis may not be always possible. However, there is considerable maternal mortality and morbidity associated with these disorders. This case underlines the complexity of pregnancy-related microangiopathies regarding their differential diagnosis, multiple organ dysfunction and role of therapeutic plasma exchange in their management.

  3. 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.

  4. 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)

  5. Differential effect of corticosteroids on serum cystatin C in thrombocytopenic purpura and leukemia.

    Science.gov (United States)

    Bardi, Edit; Dobos, Eva; Kappelmayer, János; Kiss, Csongor

    2010-09-01

    The aim of our study was to evaluate the influence of steroid therapy on serum cystatin C (cysC) concentrations in patients with acute lymphoblastic leukemia (ALL) and idiopthiás thrombocytopenias purpura (ITP). We studied 17 patients with ITP (girls: boys =5:12, mean age: 7.6 yrs, range between 1 to 17 years) and 18 patients with ALL (girls: boys =6:12, mean age: 6.3 yrs, range between 2 to 17 years). CysC and white blood cell count (WBC) in both group of patients were determined before and after 300 mg/m(2) cumulative dose of steroid therapy. Corticosteroids increased the level of cysC in both groups of patients, however significant increase was found only in ITP patients between pre- and posttreatment values (0.96 +/-0.27 mg/L vs. 1.16 +/- 0.3 mg/L, p =0,02). Pretreatment cysC concentrations were within the reference range in patients with ITP but not with ALL and were significantly higher in ALL patients, than in ITP patients (1.23 +/- 1,12 mg/L vs. 0.96 +/- 0.27 mg/L, p =0,02). Pretreatment WBC of ALL patients were significantly higher than of ITP patients (22.58 G/L, min. 3.5 G/L, max. 102.1 G/L vs. 7.46 G/L, min. 4.8 G/L, max. 12.3 G/L, p =0.03). We have found significant correlation between pretreatment cysC and WBC values in ALL patients (p = 0.04). Although the concentration of cysC may be slightly and reversibly influenced by corticosteroid treatment, cysC is sensitive to detect early and moderate deterioration of GFR in children with cancer. PMID:20084479

  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. Non-thrombocytopenic purpura in familial Mediterranean fever-comorbidity with Henoch-Schönlein purpura or an additional rare manifestation of familial Mediterranean fever?

    Science.gov (United States)

    Ben-Chetrit, Eldad; Yazici, Hasan

    2016-07-01

    Henoch-Schönlein purpura is a relatively common vasculitis mainly affecting children. It is characterized by purpuric skin rash, abdominal cramping, and haematuria. Skin biopsies taken from Henoch-Schönlein purpura lesions disclose perivascular IgA deposits. FMF is an autoinflammatory disease characterized by recurrent attacks of fever lasting 2-3 days which resolve spontaneously. Typical manifestations of the disease are peritonitis, pleuritis, pericarditis, arthritis and erysipelas-like erythema usually affecting the lower limbs. Over the years many reviews emphasized the clinical impression that Henoch-Schönlein purpura is more common among FMF patients than in healthy control population. In this review we summarize these reports and show that sometimes Henoch-Schönlein purpura associated with FMF differs from typical isolated Henoch-Schönlein purpura, and this is also the case with polyarteritis nodosa and SpA associated with FMF. It is suggested that these clinical manifestations (polyarteritis nodosa, Henoch-Schönlein purpura and SpA) should be considered to be associated with FMF as part of what we call FMF rather than as co-existing additional separate clinical entities. PMID:26464521

  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. Immune Thrombocytopenic Purpura During Maintenance Phase of Acute Lymphoblastic Leukemia: A Rare Coexistence Requiring a High Degree of Suspicion, a Case Report and Review of the Literature.

    Science.gov (United States)

    Bayhan, Turan; Ünal, Şule; Gümrük, Fatma; Çetin, Mualla

    2015-12-01

    Thrombocytopenia may develop in patients with acute lymphoblastic leukemia (ALL) due to myelosuppression of chemotherapy or relapse. Here we report a pediatric patient with ALL whose platelet counts decreased at the 102nd week of maintenance treatment. Thrombocytopenia was refractory to platelet infusions and bone marrow aspiration revealed remission status for ALL along with increased megakaryocytes. The cessation of chemotherapy for 2 weeks caused no increase in thrombocyte counts. The viral serology was unrevealing. A diagnosis of immune thrombocytopenic purpura (ITP) was established. After administration of intravenous immunoglobulin, the thrombocytopenia resolved. When thrombocytopenia occurs in patients with ALL in remission, ITP should be kept in mind after exclusion of the more common etiologies. PMID:25913619

  10. Immune Thrombocytopenic Purpura During Maintenance Phase of Acute Lymphoblastic Leukemia: A Rare Coexistence Requiring a High Degree of Suspicion, a Case Report and Review of the Literature

    Directory of Open Access Journals (Sweden)

    Turan Bayhan

    2015-12-01

    Full Text Available Thrombocytopenia may develop in patients with acute lymphoblastic leukemia (ALL due to myelosuppression of chemotherapy or relapse. Here we report a pediatric patient with ALL whose platelet counts decreased at the 102nd week of maintenance treatment. Thrombocytopenia was refractory to platelet infusions and bone marrow aspiration revealed remission status for ALL along with increased megakaryocytes. The cessation of chemotherapy for 2 weeks caused no increase in thrombocyte counts. The viral serology was unrevealing. A diagnosis of immune thrombocytopenic purpura (ITP was established. After administration of intravenous immunoglobulin, the thrombocytopenia resolved. When thrombocytopenia occurs in patients with ALL in remission, ITP should be kept in mind after exclusion of the more common etiologies.

  11. Thrombotic Thrombocytopenic Purpura

    Science.gov (United States)

    ... and Marrow Stem Cell Transplant Excessive Blood Clotting Hemolytic Anemia Send a link to NHLBI to someone by ... the body can replace them. This leads to hemolytic anemia (HEE-moh-lit-ick uh-NEE-me-uh)— ...

  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. 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

  14. Micromegakaryocytes in a patient with partial deletion of the long arm of chromosome 11 [del(11)(q24.2qter)] and chronic thrombocytopenic purpura

    Energy Technology Data Exchange (ETDEWEB)

    Gangarossa, S.; Mattina, T.; Romano, V.; Milana, G.; Mollica, F.; Schiliro, G. [Universita di Catania (Italy)

    1996-03-15

    Thrombocytopenia or pancytopenia is frequently reported in patients with partial 11q deletion but there are no reports on bone marrow morphology of these patients. We report on a patient with partial deletion of the long arm of chromosome 11 [del(11)(q24.2qter)] and its classical clinical manifestations including chronic thrombocytopenic purpura in whom micromegakaryocytes were found in the bone marrow aspirate. This is the first report of the presence of micromegakaryocytes in the bone marrow of a patient with 11q deletion. Accurate examination of the bone marrow of other patients with the 11q deletion may clarify whether the observation of micromegakaryocytes is common in these patients. Micromegakaryocytes may indicate a defect of development. Two genes for two DNA binding proteins that are likely to be involved in hematopoiesis map in the 11q region: Ets-1, that maps to 11q24, close to D11S912, and the nuclear-factor-related-kB gene that maps to 11q24-q25. It is possible that these genes, when present in only one copy, result in thrombocytopenia or pancytopenia as observed in this patient. 23 refs., 2 figs., 1 tab.

  15. Micromegakaryocytes in a patient with partial deletion of the long arm of chromosome 11 [del(11)(q24.2qter)] and chronic thrombocytopenic purpura.

    Science.gov (United States)

    Gangarossa, S; Mattina, T; Romano, V; Milana, G; Mollica, F; Schilirò, G

    1996-03-15

    Thrombocytopenia or pancytopenia is frequently reported in patients with partial 11q deletion but there are no reports on bone marrow morphology of these patients. We report on a patient with partial deletion of the long arm of chromosome 11 [del(11)(q24.2qter)] and its classical clinical manifestations including chronic thrombocytopenic purpura in whom micromegakaryocytes were found in the bone marrow aspirate. This is the first report of the presence of micromegakaryocytes in the bone marrow of a patient with 11q deletion. Accurate examination of the bone marrow of other patients with the 11q deletion may clarify whether the observation of micromegakaryocytes is common in these patients. Micromegakaryocytes may indicate a defect of development. Two genes for two DNA binding proteins that are likely to be involved in hematopoiesis map in the 11q region: Ets-1, that maps to 11q24, close to D11S912, and the nuclear-factor-related-kB gene that maps to 11q24-q25. It is possible that these genes, when present in only one copy, result in thrombocytopenia or pancytopenia as observed in this patient. PMID:8882392

  16. 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

  17. 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

  18. 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...

  19. 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.

  20. 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

  1. 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

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

    Directory of Open Access Journals (Sweden)

    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

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

    Directory of Open Access Journals (Sweden)

    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.

  5. Púrpura trombocitopénico inmune asociado a infección por virus hepatitis C en paciente hemofílico: Caso clínico Immune thrombocytopenic purpura associated to hepatitis C virus infection: Report of one case

    Directory of Open Access Journals (Sweden)

    LILIAN PILLEUX C

    2010-09-01

    Full Text Available Hepatitis C Virus (HCV is a cause of secondary chronic immune thrombocytopenic purpura (ITP. We report a 43 year old man with mild Hemophilia A, who received blood transfusions during childhood, that consulted for epistaxis and ecchymoses. The laboratory showed a platelet count of 23.000/mm³, positive HCV serology and elevated transaminases. Steroids administered in pulses followed by oral doses resulted in a partial response requiring a second pulse and association of azathioprine. A steroidal diabetes appeared. Given his refractoriness, splenectomy and liver biopsy were performed. He continued on azathioprine maintaining a platelet count near 50.000/mm³ but continued with bleeding episodes. Liver biopsy showed a chronic active hepatitis.

  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. Clinical significance of HLA-DR+, CD19+, CD10+ immature B-cell phenotype and CD34+ cell detection in bone marrow lymphocytes from children affected with immune thrombocytopenic purpura.

    Science.gov (United States)

    Callea, V; Comis, M; Iaria, G; Sculli, G; Morabito, F; Lombardo, V T

    1997-01-01

    In children with immune thrombocytopenic purpura (ITP), bone marrow lymphocytes can express the common acute lymphoblastic leukemia antigen (CALLA) pattern with no evidence of leukemia or lymphoma. Bone marrow lymphocytes from 23 children and 20 adults affected with ITP were studied to determine the incidence and the clinical impact of lymphocytes with the immature B-cell phenotype and CD34+ cell expression. In this investigation we identified a group consisting of 52% of the children who showed the immature B phenotype, while the remaining 48%, similarly to adult ITP displayed an increase of T-cell antigens. CD34 was positive in 53% of children, but it was present in only half of the patients with the immature B phenotype and it was always absent in adults. IgH genes disclosed a germline configuration in all six patients in the immature B phenotype group. No difference was found in the two groups of children in terms of age, presentation of the disease or final outcome. Finally, no patient in either children's group has developed an acute lymphoproliferative disorder. PMID:9299867

  8. 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

    Directory of Open Access Journals (Sweden)

    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.

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

    Directory of Open Access Journals (Sweden)

    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.

  10. 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

    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.%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。

  11. 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

    Institute of Scientific and Technical Information of China (English)

    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.

  12. 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

  13. 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.

  14. 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

  15. 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.

  16. 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

  17. 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

  18. 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...

  19. 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

  20. Platelet alloimmunization after transfusion

    DEFF Research Database (Denmark)

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

    1997-01-01

    (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......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......-antibody-specific immobilization of platelet antigen assay. HLA antibodies were detected by the standard lymphocyte cytotoxicity techniques. RESULTS: We found platelet-specific anti-HPA-5b (anti-Bra) in 2 cases (1.7%). One antibody was the result of de novo immunization. We detected lymphocytotoxic HLA antibodies in 21 patients...

  1. 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

  2. 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

  3. 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。

  4. 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 a...... 14-year-old boy has acquired TTP due to an infection with Campylobacter jejuni and Samonella szentes. Plasma exchange plays an essential role in the treatment of TTP....

  5. 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.

  6. 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

  7. 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.

  8. 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

  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. 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...

  11. 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.

  12. Idiopathic thrombocytopenic purpura following successful treatment of acute lymphoblastic leukemia.

    Science.gov (United States)

    Tannir, N M; Kantarjian, H

    2001-03-01

    Thrombocytopenia is common in patients with acute lymphocytic leukemia (ALL) at diagnosis. It is a universal side effect of dose-intensive regimens employed in the treatment of adult ALL. In patients with ALL who achieve remission, thrombocytopenia frequently indicates relapse. We report three adult patients successfully treated for ALL who developed thrombocytopenia and were found to have immune-mediated thrombocytopenia (ITP). Possible pathophysiologic mechanisms underlying the association of ALL and ITP are discussed. PMID:11342378

  13. 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...

  14. 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。

  15. 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...

  16. 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

  17. Exercise-induced purpura.

    Science.gov (United States)

    Ramelet, Albert-Adrien

    2004-01-01

    Exercise-induced purpura (EIP) occurs on the lower legs after unusual or major muscular activity, as in marathon runners or as after long walks, especially in the mountains in hot weather. In leisure walkers, patients are otherwise healthy females. There is no relation with chronic venous disorder. Erythematous, urticarial or purpuric plaques arise on the lower leg, usually sparing the skin compressed by socks. Symptoms include itch, pain and a burning sensation. Histopathology demonstrates leukocytoclastic vasculitis. The lesions fade after some days, with frequent relapses at further muscular exercises and may be prevented in some cases by compression, intake of venoactive drugs and local application of steroids. EIP is not uncommon, even if very few descriptions have yet been published. It appears to be consecutive to venous stasis induced by an acute failure of the muscle pump of the calf and thermoregulation decompensation, after a prolonged and unusual exercise, such as running or walking in hot weather. PMID:15178910

  18. 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.

  19. Alloimmunization among transfusion-dependent thalassemia patients

    Directory of Open Access Journals (Sweden)

    Sadeghian Mohammad

    2009-01-01

    Full Text Available 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 thalassemia patients in the northeast of Iran, who received regular blood transfusion, were included in this study. Screening of antibodies was performed on fresh serum of all patients and then antibodies were identified in patients′ serum that had positive antibody screening test using a panel of recognized blood group antigens. Results: We identified 12 alloantibodies in 9 patients (2.87% that all were against Rhesus (Rh blood group antigens (D, C, E. Three patients developed 2 antibodies, and others had one antibody. The most common alloantibodies were Anti-D (88.88% and followed by Anti-C and Anti-E. Higher frequency of alloimmunization was observed in female, Rh negative and splenectomized patients. Conclusion: This study showed that evaluation of the packed cells for Rh (C, E from the start of transfusion can be helpful in decreasing the rate of alloantibody synthesis.

  20. 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

  1. 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.

  2. 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.

  3. 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

  4. 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

  5. Risk factors for alloimmunization by patients with sickle cell disease

    Directory of Open Access Journals (Sweden)

    M. Murao

    2005-05-01

    Full Text Available Blood transfusion in patients with sickle cell disease (SCD is limited by the development of alloantibodies to erythrocytes. In the present study, the frequency and risk factors for alloimmunization were determined. Transfusion records and medical charts of 828 SCD patients who had been transfused and followed at the Belo Horizonte Blood Center, Belo Horizonte, MG, Brazil, were retrospectively reviewed. Alloimmunization frequency was 9.9% (95% CI: 7.9 to 11.9% and 125 alloantibodies were detected, 79% of which belonged to the Rhesus and Kell systems. Female patients developed alloimmunization more frequently (P = 0.03. The median age of the alloimmunized group was 23.3 years, compared to 14.6 years for the non-alloimmunized group (P < 0.0001. Multivariate analyses were applied to the data for 608 hemoglobin (Hb SS or SC patients whose number of transfusions was recorded accurately. Number of transfusions (P = 0.00006, older age (P = 0.056 and Hb SC (P = 0.02 showed independent statistical associations with alloimmunization. Hb SC patients older than 14 years faced a 2.8-fold higher (95% CI: 1.3 to 6.0 risk of alloimmunization than Hb SS patients. Female Hb SC patients had the highest risk of developing alloantibodies. In patients younger than 14 years, only the number of transfusions was significant. We conclude that an increased risk of alloimmunization was associated with older patients with Hb SC, specially females, even after adjustments were made for the number of transfusions received, the most significant variable.

  6. 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...

  7. Prevalence of Alloimmunization against RBC Antigens in Thalassemia Major Patients

    OpenAIRE

    Amin Mirzaeian; Gholamhossein Tamaddon; Majid Naderi; Marziyeh Hosseinpour; Narges Sargolzaie

    2013-01-01

    Background: Regular blood transfusions to treat the patients with thalassemia major generate antibodies acting against red blood cells antigens. This immune response is called alloimmunity. This study was conducted with the purpose of determining the prevalence of alloantibodies and autoantibody, identifying the type of causative antigen, and recognizing the factors affecting alloimmunization among the patients with thalassemia major receiving blood. Materials and Methods: In this cross-secti...

  8. A rare manifestation of neonatal alloimmune thrombocytopaenia

    Science.gov (United States)

    Jerónimo, Monica; Azenha, Cátia; Mesquita, Joana; Pereira, Dolores Faria

    2014-01-01

    Neonatal alloimmune thrombocytopaenia (NAIT) results from a fetomaternal incompatibility with maternal sensitisation against a fetal human platelet antigen (HPA) and antibodies transfer to the fetal circulation, leading to platelet destruction. The clinical presentation is variable and isolated intraocular haemorrhage is rare. We present the case of a male newborn, with intrauterine growth restriction, born at 29 weeks due to pre-eclampsia. He presented proptosis of the left eye, hyphaema and elevated intraocular pressure, with no other signs of haemorrhage. Severe thrombocytopaenia was found (27×109/L). Perinatal infection and maternal thrombocytopaenia were excluded. Positive anti-HPA-1a and antihuman leucocyte antigen class I alloantibodies were found in the mother. Platelet crossmatch between the father's platelets and mother's plasma was positive. Platelet transfusions and intravenous immunoglobulin were given with favourable response. This case highlights an unusual presentation of NAIT, which should be suspected in the presence of severe thrombocytopaenia in the first 24–72 h of life. PMID:24891486

  9. 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

  10. 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.

  11. 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.

  12. Lesões microscopicas do baço na purpura trombocitopenica idiopatica

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    Manuel Riveros

    1947-09-01

    Full Text Available Histopathological changes strikingly similar were found in the spleen of four cases (young female subjects of idiopathic thrombocytopenic purpura hemorrhagica in which splenectomy was performed. The chief changes reported are enlargement of the marginal zone of the malpighian corpuscles, proliferation and mobilization of the reticulo-endothelial cells, myeloid metaplasia, local (tissue eosinophilia, and stoppage of the circulation or stasis of platelets from which results a filling of the spelenic sinuses by such elements. The latter phenomenon will possibly present some bearing with thrombocytopenia which is such a characteristic feature in this disease and will perhaps account for the rapid increase in blood platelets which usually follows splenectomy and or the finding of increased megakaryocytes in the bone marrow.

  13. 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...

  14. 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...

  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...

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

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    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.

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

    International Nuclear Information System (INIS)

    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

  18. 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

  19. 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

  20. 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

  1. 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...

  2. 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

  3. 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.

  4. HENOCH-SCHONLEIN PURPURA IN CHILDHOOD

    OpenAIRE

    Hristina M Stamenković; Tatjana Stanković; Zlatko Đurić

    2015-01-01

    Henoch-Schonlein purpura is the most common vasculitis in children. The process affects small blood vessels of the skin, joints, gastrointestinal tract, kidneys and the central nervous system. The clinical manifestations in children with Henoch-Schonlein purpura were analyzed. The count of white blood cell, trombocytopenia, CRP, LDH, CPK, titer of antistreptolysin antibody (ASO) were analyzed. Urinalysis and urine culture tests were performed. The values of complements (C3 and C4) and immunog...

  5. Detection of alloimmunization to ensure safer transfusion practice

    OpenAIRE

    2013-01-01

    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 an...

  6. [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

  7. Prevalence of Alloimmunization against RBC Antigens in Thalassemia Major Patients

    Directory of Open Access Journals (Sweden)

    Amin Mirzaeian

    2013-07-01

    Full Text Available Background: Regular blood transfusions to treat the patients with thalassemia major generate antibodies acting against red blood cells antigens. This immune response is called alloimmunity. This study was conducted with the purpose of determining the prevalence of alloantibodies and autoantibody, identifying the type of causative antigen, and recognizing the factors affecting alloimmunization among the patients with thalassemia major receiving blood. Materials and Methods: In this cross-sectional study, 385 patients with thalassemia major participated. After recording their demographic information, serum specimens taken from the patients were screened using pooled cells obtained from Biorad Company. The positive cases were examined to identify antibodies using panel cells obtained from Iranian Blood Transfusion Organization. In this study, SPSS 16 was employed for performing statistical analysis.Results: Of the 385 patients, 69 subjects (17.9% comprising 221 men and 164 women had alloantibody. In 57 cases, the antibody type was exactly identified. In 21 patients (5.5% the existence of autoantibody was determined. The mean ages of the participants were within 14.3±7.5 and 13.3±7.9 years old for male and female groups, respectively. 28 patients had splenectomy and age at the onset of blood transfusion ranged from a month after birth to nine years.Conclusion: In these patients, the most significant blood group systems acted by alloantibodies were Rh and Kell. Since the results of this study show 17.9% incidence of alloimmunization in these patients, it is recommended to carry out injected blood compatibility test (cross-match after antibody screening.

  8. Scrub typhus presenting as Purpura fulminans

    Directory of Open Access Journals (Sweden)

    Suhasini Tirumala

    2014-01-01

    Full Text Available Seriously ill patients presenting with purpura fulminans, sepsis and multi-organ failure often require extensive diagnostic workup for proper diagnosis and management. Host of common infections prevalent in the tropics, e.g. malaria, dengue; other septicemic infections e.g. meningococcemia, typhoid, leptospirosis, toxic shock syndrome, scarlet fever, viral exanthems like measles, infectious mononucleosis, collagen vascular diseases (Kawasaki disease, other vasculitis diseases, and adverse drug reactions are often kept in mind, and the index of suspicion for rickettsial illness is quite low. We present a case of Indian tick typhus presenting with purpura fulminans (retiform purpura all over the body, sepsis and multiorgan failure without lymphadenopathy and eschar, successfully treated with doxycycline and discharged home. Hence, a high index clinical suspicion and prompt administration of a simple therapy has led to successful recovery of the patient.

  9. Role of heat shock protein 70 in innate alloimmunity

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    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

  10. Simultaneous occurrence of fetal and neonatal alloimmune thrombocytopenia and neonatal neutropenia due to maternal neutrophilic autoantibodies

    DEFF Research Database (Denmark)

    Taaning, Ellen; 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...

  11. 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...

  12. 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.

  13. 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...

  14. 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

  15. 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

  16. 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

  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. Henoch Schönlein purpura associated with pulmonary adenocarcinoma

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    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.

  19. 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

  20. 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...

  1. Massive soft tissue infections: necrotizing fasciitis and purpura fulminans.

    Science.gov (United States)

    Edlich, Richard F; Winters, Kathryne L; Woodard, Charles R; Britt, L D; Long, William B

    2005-01-01

    Necrotizing fasciitis and purpura fulminans are two destructive infections that involve both skin and soft tissue. Necrotizing fasciitis is characterized by widespread necrosis of subcutaneous tissue and the fascia. Historically, group A beta-hemolytic streptococcus has been identified as a major cause of this infection. However, this monomicrobial infection is usually associated with some underlying cause, such as diabetes mellitus. During the last two decades, scientists have found that the pathogenesis of necrotizing fasciitis is polymicrobial. The diagnosis of necrotizing fasciitis must be made as soon as possible by examining the skin inflammatory changes. Magnetic resonance imaging is strongly recommended to detect the presence of air within the tissues. Percutaneous aspiration of the soft tissue infection followed by prompt Gram staining should be conducted with the "finger-test" and rapid-frozen section biopsy examination. Intravenous antibiotic therapy is one of the cornerstones of managing this life-threatening skin infection. Surgery is the primary treatment for necrotizing fasciitis, with early surgical fasciotomy and debridement. Following debridement, skin coverage by either Integra Dermal Regeneration Template or AlloDerm should be undertaken. Hyperbaric oxygen therapy complemented by intravenous polyspecific immunoglobulin are useful adjunctive therapies. Purpura fulminans is a rare syndrome of intravascular thrombosis and hemorrhagic infarction of the skin; it is rapidly progressive and accompanied by vascular collapse. There are three types of purpura fulminans: neonatal purpura fulminans, idiopathic or chronic purpura fulminans, and acute infectious purpura fulminans. Clinical presentation of purpura fulminans involves a premonitory illness followed by the rapid development of a septic syndrome with fever, shock, and disseminated intravascular coagulation. The diagnosis and treatment of these conditions is best accomplished in a regional burn

  2. 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.

  3. 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

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

    Directory of Open Access Journals (Sweden)

    Ashu Dogra

    2015-01-01

    Full Text Available 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 receiving regular blood transfusions, registered at SMGS Blood Bank, Jammu. Relevant clinical and laboratory data was collected with reference to age at the start of transfusions, total number of transfusions received and splenectomy status. Antibodies screening, antibody identification, and cross matching was done on allpatient samples included in the study, during the period between November 2009 and October 2010. Results: In this study, a total of six alloantibodies six patients (8.5% and one autoantibody (1.42% was detected. All identified alloantibodies belonged to Rh system (i.e. anti-E, in 3 patients (50%, anti D, in one patient (16.66% and Kell system (anti-K, in two patients (33.34%. Higher frequency of alloimmunization was found, with increase in number of transfusions and in those who received transfusions after 1 year of age. Alloimmunization was not significantly associated with gender and splenectomy status (P-value > 0.05. Conclusion: Red cell alloantibodies developed in 8.5% of thalassemic patients and 1.42% had autoantibodies. The most common alloantibodies identified were anti Rh system antibodies (anti-E and anti-D present in 50% and 16.66% of patients respectively. Alloimmunization is not an uncommon problem faced by blood banks and finding compatible units for regularly transfused thalassemic patients may become very difficult. In order to reduce alloimmunization, a policy for performing extended red cell phenotyping of these patients is

  5. 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

  6. 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

  7. 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.

  8. [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

  9. [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

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

    OpenAIRE

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

    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 bet...

  11. 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

  12. 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...

  13. 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...

  14. Pattern and prevelence of alloimmunization in multiply transfused patients with sickle cell disease in Nigeria

    OpenAIRE

    Kangiwa, Umar; Ibegbulam, Obike; Ocheni, Sunday; Madu, Anazoeze; Mohammed, Ndakosu

    2015-01-01

    Background and study objectives Blood transfusion is central in the prevention and treatment of certain chronic complications of sickle cell disease. It is indispensible in correcting anaemias as well as in the practice of exchange blood transfusion. These gains are largely limited by formation of allo-antibodies. Several studies demonstrated varying frequencies of allo-immunization in various patient groups. The effect of the racial differences between the donor and recipient pool, which has...

  15. 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

  16. 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...

  17. A case of Hydrops fetalis due to Kell alloimmunization: A perinatal approach to a rare case

    Directory of Open Access Journals (Sweden)

    Arzu Akdag

    2012-03-01

    Full Text Available Introduction: While the routine administration of Rhesus immunoglobulin has significantly reduced the incidence of this type of alloimmunization, maternal alloimmunization to other red cell antigens continues to contribute to perinatal morbidity and mortality. Although the Kell antigen is found on the red cells of only 9% of the population, attention has increasingly been focused on Kell antibodies. Case Report: We present a case of fetal hydrops who was sonographically detected at 30th week of pregnancy. Antenatal tests for evaluation of fetal condition clearly showed the critical level of hemolytic disease but the baby was delivered prematurely due to fetal distress. The combination of anemia, reticulocytopenia, hydrops fetalis, and positive indirect Coombs test suggested Kell isoimmunization. The baby was treated by exchange transfusion with the Kell-negative packed red cell succesfully, and was discharged on postnatal 30th day. Conclusion: Here we describe a case of hydrops fetalis caused by Kell alloimmunization that was determined in postnatal period, and thus we plan to discuss the perinatal approach to the Kell immunization.

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

    Directory of Open Access Journals (Sweden)

    Tamima Al-Dughaishi

    2016-01-01

    Full Text Available 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 June 2013. Pregnant women were tested for ABO blood type and were screened for RhD antigen and antibodies at their first antenatal clinic visit. In women who tested positive for the RhD antibodies, an antibody titer was performed to evaluate the severity of their case. Results: Data was available on 1,251 pregnant women who were managed and delivered at Sultan Qaboos University Hospital. The prevalence of RhD negative pregnant women was 7.3%. Blood group O was the most common followed by A, B, and AB. The rate of RhD negative alloimmunization was 10%, and anti-D was the most common antibody detected. There were no stillbirths or neonatal deaths. Postnatal transfusion was necessary for only one baby. Conclusions: The prevalence of RhD negativity was comparable to other Asian countries. Previous RhD alloimmunization and history of miscarriages were the most common maternal medical history.

  19. Risk estimation of HNA-3 incompatibility and alloimmunization in Thai populations.

    Science.gov (United States)

    Nathalang, Oytip; Intharanut, Kamphon; Siriphanthong, Kanokpol; Nathalang, Siriporn; Leetrakool, Nipapan

    2015-01-01

    Severe transfusion-related acute lung injury (TRALI) is often due to antibodies in blood components directed against human neutrophil antigen (HNA)-3a. This study aimed to report the genotype frequencies of the HNA-3 system and to estimate the potential risk of HNA-3 incompatibility and alloimmunization in two Thai populations. Eight hundred DNA samples obtained from 500 unrelated healthy blood donors at the National Blood Centre, Thai Red Cross Society, Bangkok and 300 samples from the Blood Bank, Faculty of Medicine, Chiang Mai University, Chiang Mai, Thailand were included. HNA-3 genotyping was performed using an in-house polymerase chain reaction with sequence-specific primer (PCR-SSP) technique. The observed frequencies of the HNA-3a/3a, HNA-3a/3b, and HNA-3b/3b genotypes were 0.528, 0.380, and 0.092 in central Thais and 0.600, 0.350, and 0.050 in northern Thais, respectively. The frequencies were used to estimate HNA-3 incompatibility and risk of HNA-3a alloimmunization. The HNA-3 incompatibility in central Thais (33.28%) was higher than northern Thais (28.75%), corresponding to a significantly higher probability of HNA-3a alloimmunization (Ptransfusion. PMID:25608003

  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. Purpura fulminans due to acquired protein C deficiency

    Directory of Open Access Journals (Sweden)

    Devdeep Mukherjee

    2015-01-01

    Full Text Available Purpura fulminans (PF may be the presenting symptom in a patient with protein C (PC deficiency. It is a hematological emergency and presents with extensive areas of hemorrhagic necrosis of the skin. PC deficiency is usually genetically inherited. However, we report a 1 year and 4 months boy, who presented with acquired PC deficiency possibly of postinfectious etiology and developed PF.

  2. 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

  3. 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

  4. [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

  5. 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.

  6. Adult purpura fulminans associated with non-steroidal anti-inflammatory drug use

    Directory of Open Access Journals (Sweden)

    N Kosaraju

    2011-01-01

    Full Text Available Purpura fulminans is an acute illness characterized by rapidly progressive dermal vascular thrombosis, leading to hemorrhagic necrosis of the skin. Here, we describe the case of a healthy woman who developed acute disseminated intravascular coagulation (DIC with purpura fulminans after intramuscular administration of a single dose of ketorolac. Review of literature showed only one case description of non-steroidal anti-inflammatory drug (diclofenac-related purpura fulminans with DIC.

  7. Adult purpura fulminans associated with non-steroidal anti-inflammatory drug use

    OpenAIRE

    N Kosaraju; V Korrapati; Thomas, A; B R James

    2011-01-01

    Purpura fulminans is an acute illness characterized by rapidly progressive dermal vascular thrombosis, leading to hemorrhagic necrosis of the skin. Here, we describe the case of a healthy woman who developed acute disseminated intravascular coagulation (DIC) with purpura fulminans after intramuscular administration of a single dose of ketorolac. Review of literature showed only one case description of non-steroidal anti-inflammatory drug (diclofenac)-related purpura fulminans with DIC.

  8. Clinical and Laboratory Findings in Henoch-Schoenlein Purpura

    Directory of Open Access Journals (Sweden)

    A Hashemzadeh

    2006-06-01

    Full Text Available Background: Henoch-Schoenlein purpura (HSP is the most common vasculitis in children. It is characterized by purpura, arthritis, gasterointestinal involvment and glumerulonephritis. There is a male to female predominance. It is estimated that some infections such as BHGAS can predispose to HSP. Methods: In a retrospective study we evaluated clinical and laboratory findings in patients with HSP diagnosis admitted to Ghaem and Imam Raza hospitals in Mashad, Findings: It is known that skin, joint and gastrointestinal involvement is the most common presentation of disease. In our patients, kidney disorders were not detected. Hyperleukocytosis, positive CRP, elevated ESR and ASO titers were frequently observed laboratory signs in these patients Conclusion: In this study HSP had a good prognosis and we didn’t any kidney complication in our subjects.

  9. Non-invasive ultrasonographic diagnostics of Rh(D) alloimmunized pregnancies

    International Nuclear Information System (INIS)

    A non-invasive ultrasonographic assessment of systolic ejection peak in the middle cerebral artery is very efficient in detection of anomalies in alloimmunized pregnancies and is a diagnostic method of choice prior to cordocentesis. This is applied in combination with detection of titre of Rh(D) antibodies. In our study, two groups of patients were tested and results were compared. The first sample included 52 newborns in the period 1998-2007 (initial period of intrauteral intravascular transfusion, while the second one included 16 newborns from the period 1998-2008 (second period, when better prenatal control was achieved). Levels of titter of antibodies in salty environment, in both the control and the analyzed group, indicated moderate sensitization in a small group of patients. However, in albumin and in particular Coombs environment, the level of sensitization was significant in all patients (p>0.05; DF = 67). Our investigation suggests that with ultrasound as a method of choice and with prenatal screening it is possible to achieve better diagnosis and prognosis in the case of Rh(D) conditions of alloimmunized pregnancies. (author)

  10. 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.

  11. 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

  12. 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

  13. 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...

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

    Directory of Open Access Journals (Sweden)

    Jophy Varghese

    2013-01-01

    Full Text Available 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 between January 2008 and January 2009, were screened for irregular antibodies using a commercial 3-cell antibody screening panel. Antibody identification was performed on samples found positive using a commercial 11 cell-panel. Results: Screening was performed on 5347 women, 339 (6.34% of whom were Rh negative. Allosensitization was found in 79 women (1.48%; confidence interval 1.17 -1.84. In 29 of these 79 (37% women the allo-antibodies could not be identified. In the remaining 50 women, 54 antibodies were characterized. A total of 40 clinically significant antibody specificities were identified among 36 women, of whom four were Rh(D positive. Allosensitization with clinically significant antibodies was found in 9.43 per cent (confidence interval 6.55-13.06 Rh(D negative and in 0.08 per cent (confidence interval .02-0.2 Rh(D positive women. Anti D was the most frequent antibody found in 8.85 per cent Rh(D negative women. The remaining clinically significant antibodies identified included anti-C, c, E, Jk a , Jk b , M and S. In Rh(D negative women, anti-D and antibodies of the Rh system contributed 83.3 and 94.4 per cent of clinically significant antibodies. However, in Rh(D positive women, non-Rh antibodies comprised three out of four clinically significant antibodies. Interpretation & conclusions: The presence of alloimmunization in our study corroborated with data reported from India. The most frequent antibody was anti-D. However, a significant fraction was

  15. 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

  16. 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).

  17. 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

  18. 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

    haematology analysers and commonly used methods for testing platelet function. The test principles, advantages and disadvantages of each test are described. We also evaluate the current literature regarding the clinical utility of the test for prediction of bleeding in thrombocytopenia in haematological and...... 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. To...... fully uncover the clinical utility of platelet-related tests, information about the prevalence of platelet function defects in thrombocytopenic conditions is required. Finally, knowledge of the performance in thrombocytopenic samples from patients is essential....

  19. 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...

  20. 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.

  1. Anti-TJa alloimmunization (anti-PP1Pk): two consecutive pregnancies of an anti-TJa-carrying patient.

    Science.gov (United States)

    Haentjens-Verbeke, K; Dufour, P h; Vinatier, D; Tordjeman, N; Monnier, J C; Manessier, L

    1996-01-01

    Anti-TJa alloimmunization is an extremely rare condition which can cause miscarriages during the first and second terms of pregnancy. At 20-year-old female patient, an anti-TJa carrier, was given plasma transfusions in the course of her first two pregnancies. The cordocentesis, which was the main follow-up test during the first pregnancy, could not be carried out during the second pregnancy due to the presence of a huge subchorionic hematoma. Both pregnancies were characterized by fetal growth retardation, which was particularly considerable in one. Due to chronic fetal distress, both infants were delivered prematurely by cesarean section. The immunohematological data concerning anti-TJa alloimmunization and the different palliative treatments available to date are reviewed. PMID:8838768

  2. 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.

  3. 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;

  4. 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

  5. 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

  6. 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

  7. 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

  8. Acute Scrotal Swelling in Henoch-Schonlein Purpura: Case Report and Review of the Literature.

    Science.gov (United States)

    Modi, Sunny; Mohan, Muhunthan; Jennings, Andrew

    2016-05-01

    Henoch-Schönlein purpura (HSP) is a systemic vasculitis characterized classically by purpura, arthritis and abdominal pain. Epididymitis/orchitis is rarely seen as a complication of HSP. Testicular or scrotal involvement has been reported in children with Henoch-Schonlein purpura and must be distinguished from testicular torsion. We report a case of a 5 year old boy diagnosed with Henoch-Schönlein purpura with acute scrotal swelling. He was managed successfully with conservative approach. The history, clinical examination findings and scrotal ultrasound evaluation should suffice to make the correct diagnosis and avoid surgery. Steroid treatment and/or antibiotics appeared to be effective for this condition. PMID:27169017

  9. 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.

  10. 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.

  11. Atypical Henoch-Schonlein purpura? Consider polyarteritis nodosa!

    Science.gov (United States)

    Braungart, Sarah; Campbell, Alison; Besarovic, Sanja

    2014-01-01

    We describe the case of a previously healthy 12-year-old boy admitted to a tertiary paediatric centre with the clinical diagnosis of Henoch-Schonlein purpura (HSP). Symptoms on admission included a generalised rash, colicky abdominal pain, hypertension, proteinuria and fresh rectal bleeding. Abdominal pain and distension worsened and serial ultrasounds suggested bowel ischaemia. He underwent repeat laparotomy and bowel resection, with slow improvement after the second laparotomy. The severity of systemic involvement (gastrointestinal, cardiac, renal and skin) made the initial diagnosis of HSP questionable. Immunohistochemistry of skin biopsies was negative for HSP. Histopathology of the bowel specimen revealed features of necrotising small and medium vessel vasculitis in keeping with polyarteritis nodosa. PMID:24717855

  12. Itching Papules in a child; Scabies or Henoch Schonlein Purpura?

    Directory of Open Access Journals (Sweden)

    Zahra Soheili Rad

    2015-12-01

    Full Text Available Introduction: Henoch schonlein purpura (HSP is a self-limited systemic disease with multiorgan involvement. While cutaneous involvement may wary, presenting as itching papules is not a common manifestation. In this report we discuss a case of HSP which was presented solely with itching papules; mistaken for scabies. Case presentation: A 7 year old boy presented with itchy palpable papules without any gastrointestinal or urinary problem which was primary taught to be an ectoparasitic infestation. During the hospitalization the patient developed abdominal pain as well as hematuria and the diagnosis became more evident. The skin biopsy showed perivascular inflammatory cells infiltration in epidermis, perivascular fibrinoid necrosis and leukocytoclastic vasculitis. The patient received corticosteroids and discharged healthy.Conclusion: While HSP may have different cutaneous manifestation, presenting as only itchy papules may complicate the diagnosis and physicians should always keep in mind that HSP might be presented as other common infestations unresponsive to treatment.

  13. 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

  14. 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

  15. 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.

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

    DEFF Research Database (Denmark)

    Laursen, B; Morling, N; Rosenkvist, J; Sørensen, H; Thyme, S

    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 meas...

  17. Henoch-Schönlein purpura in an older man presenting as rectal bleeding and IgA mesangioproliferative glomerulonephritis: a case report

    OpenAIRE

    Howarth Charles B; Jirajariyavej Teeranun; Cheungpasitporn Wisit; Rosen Raquel M

    2011-01-01

    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 Pol...

  18. No red cell alloimmunization or change of clinical outcome after using fresh frozen cancellous allograft bone for acetabular reconstruction in revision hip arthroplasty: a follow up study

    Directory of Open Access Journals (Sweden)

    Mittag Falk

    2012-09-01

    Full Text Available Abstract Background Possible immunization to blood group or other antigens and subsequent inhibition of remodeling or incorporation after use of untreated human bone allograft was described previously. This study presents the immunological, clinical and radiological results of 30 patients with acetabular revisions using fresh frozen non-irradiated bone allograft. Methods AB0-incompatible (donor-recipient bone transplantation was performed in 22 cases, Rh(D incompatible transplantation in 6 cases. The mean follow up of 23 months included measuring Harris hip score and radiological examination with evaluation of remodeling of the bone graft, implant migration and heterotopic ossification. In addition, all patients were screened for alloimmunization to Rh blood group antigens. Results Compared to the whole study group, there were no differences in clinical or radiological measurements for the groups with AB0- or Rh(D-incompatible bone transplantation. The mean Harris Hip Score was 80.6. X-rays confirmed total remodeling of all allografts with no acetabular loosening. At follow up, blood tests revealed no alloimmunization to Rh blood group donor antigens. Conclusions The use of fresh frozen non-irradiated bone allograft in acetabular revision is a reliable supplement to reconstruction. The risk of alloimmunization to donor-blood group antigens after AB0- or Rh-incompatible allograft transplantation with a negative long-term influence on bone-remodeling or the clinical outcome is negligible.

  19. 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. 

  20. 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.

  1. 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.

  2. 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

  3. 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

  4. 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.

  5. 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.

  6. 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

  7. 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.

  8. 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...

  9. 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...

  10. 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.

  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. Effects of increased von Willebrand factor levels on primary hemostasis in thrombocytopenic patients with liver cirrhosis.

    Science.gov (United States)

    Wannhoff, Andreas; Müller, Oliver J; Friedrich, Kilian; Rupp, Christian; Klöters-Plachky, Petra; Leopold, Yvonne; Brune, Maik; Senner, Mirja; Weiss, Karl-Heinz; Stremmel, Wolfgang; Schemmer, Peter; Katus, Hugo A; Gotthardt, Daniel N

    2014-01-01

    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 future

  13. Prevention of MHC-alloimmunization by UV-B irradiation in a murine model: effects of UV dose and number of transfused cells

    International Nuclear Information System (INIS)

    The optimal dose of UV-B radiation for prevention of in vivo alloimmunization (AI) against major histocompatibility complex (MHC) antigens was investigated in a murine transfusion model. Two groups with five C57BL/6 mice (H-2b) each were transfused at weekly intervals with 1 x 105 or 1 x 106 DBA/2 (H-2d) leucocytes. Both suspensions induced anti-H-2d antibodies in all mice after the second transfusion. The minimal UV-B dose required for abolition of alloreactivity in the mixed leucocyte reaction (MLR) was 0.6 J/cm2. This dose completely prevented the onset of MHC-AI in all five mice transfused with six suspensions containing 1 x 105 leucocytes. In contrast, suspensions with 1 x 106 leucocytes and exposed to 0.6 J/cm2 induced immunization in 4/5 mice. Further increase of the dose to 1.8 or 5.4 J/cm2 did not prevent the onset of MHC-AI. We conclude that the number of leucocytes per transfusion determines the efficacy of UV irradiation for the prevention of MHC-AI. For UV irradiation of human platelet concentrates (PCs) we propose to reduce the number of leucocytes by centrifugation prior to UV exposure. UV-B irradiation of PCs with high numbers of leucocytes may not be effective for prevention of alloimmunization. (Author)

  14. 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

  15. 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]  

  16. 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.

  17. 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.

  18. 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

  19. Treatment of severe henoch-schonlein purpura nephritis with mycophenolate mofetil

    Directory of Open Access Journals (Sweden)

    Ahmad Ali Nikibakhsh

    2014-01-01

    Full Text Available Henoch-Schonlein purpura (HSP is the most common childhood vasculitis. Renal involvement in HSP is one of the major causes of chronic renal failure in children. It is important to start effective and relatively safe medication to prevent end-stage renal disease (ESRD. Mycophenolate mofetil (MMF appears to be a promising therapeutic agent in many autoimmune diseases such as lupus nephritis and vasculitis. Herein, we describe the treatment with MMF of three patients with HSP nephritis. In two cases with rapidly progressive glomerulonephritis without response to steroid, after treatment with MMF, significant improvement in kidney function and proteinuria were observed. In another patient with HSP nephritic-nephrotic syn-drome who showed resistance to steroid, MMF offered a favorable effect. MMF seems to be a promising therapeutic agent in the treatment of the severe HSP nephritis.

  20. 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.

  1. Disseminate intradermal bacterial colonization presenting as palpable purpura in lymphoblastic leukemia.

    Science.gov (United States)

    Shelley, W B; Zolin, W D

    1983-05-01

    A patient with acute lymphoblastic leukemia suddenly developed a disseminated monomorphic eruption of purpuric papules. The papules were initially thought to be lesions of vasculitis, leukemia cutis, or septic emboli. Histologic study, however, revealed large focal colonies of gram-positive cocci within the dermis. It is postulated that a bacteremia of antibiotic-resistant cocci led to dissemination of these organisms into a virtually defenseless skin. The patient's pancytopenia and consequent immune paralysis are viewed as accounting for this focal bacterial colonization and for the remarkable absence of clinical and histologic inflammatory response. Such hematogenous noninflammatory bacterial colonization of the skin must be added to the differential diagnosis of palpable purpura. PMID:6575017

  2. 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

  3. Thrombocytosis following splenectomy and aortic valve replacement for idiopathic thrombocytopaenic purpura with bicuspid aortic valve

    Science.gov (United States)

    Katiyar, Sarika; Ganjsinghani, Payal Kamlesh; Jain, Rajnish Kumar

    2015-01-01

    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 PMID:26379295

  4. 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.

  5. 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...

  6. 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...

  7. [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

  8. 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

  9. Lifetime cost of meningococcal disease in France: Scenarios of severe meningitis and septicemia with purpura fulminans.

    Science.gov (United States)

    Bénard, Stève; Wright, Claire; Voisine, Jimmy; Olivier, Catherine W; Gaudelus, Joël

    2016-01-01

    Invasive meningococcal disease (IMD) is life-threatening and can result in severe sequelae. In France, no data have been published on the costs of severe IMD cases. Two realistic scenarios were developed with national experts (clinicians and social workers): a 6-year-old child with purpura fulminans with amputation of both legs below the knee (case A) and a 3-year-old with meningitis and severe neurological sequelae (case B). Additional scenarios included other typical sequelae of IMD such as chronic kidney disease (CKD), profound deafness and epilepsy. Data on healthcare, disability, educational and other resource use were obtained from experts and families of patients with similar sequelae. Unit costs (2013) were mainly obtained from the literature and the National Health Insurance (NHI). Time horizon was based on life expectancies of patients (77 and 55 years, respectively). A 4% discount rate decreasing to 2% after 30 years was applied. Costs are presented from the perspective of the NHI, publicly funded organizations and patients' families or their private health insurances. purpura fulminans with amputations is associated with a lifelong discounted cost of €768,875. Adding CKD doubles the amount (€1,480,545). Meningitis with severe neuro-cognitive sequelae results in a lifelong discounted cost of €1,924,475. Adding profound deafness and epilepsy slightly increases the total cost (€2,267,251). The first year is the most expensive in both scenarios (€166,890 and €160,647 respectively). The main cost drivers for each scenario are prostheses and child/adult stays in healthcare facilities, respectively. Overall, patients' families or his private insurance had to pay around 13% of total cost (101,833€ and 258,817€, respectively). This study fills a gap in the body of knowledge on IMD sequelae care and lifetime costs in France. The potentially high economic burden of IMD, in addition to its physical, psychological and social burden, reinforces the

  10. 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.

  11. 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

  12. Henoch-Schönlein purpura complicated by acalculous cholecystitis and intussusception, and following recurrence with appendicitis.

    Science.gov (United States)

    Özkaya, Ahmet Kağan; Güler, Ekrem; Çetinkaya, Ahmet; Karakaya, Ali Erdal; Göksügür, Yalçın; Katı, Ömer; Güler, Ahmet Gökhan; Davutoğlu, Mehmet

    2016-05-01

    Henoch-Schönlein purpura (HSP) is the most common childhood systemic vasculitis. Gastro-intestinal involvement occurs in two-thirds of patients. The characteristic skin lesions generally precede abdominal symptoms or present concurrently. A 7-year-old boy presented with intussusception and acalculous cholecystitis and had a cholecystectomy. Two weeks later he was re-admitted with features typical of HSP which responded to corticosteroids. Eleven months later he presented with abdominal pain and recurrence of HSP and, at laparotomy, there was acute appendicitis. This is the first case of a child presenting with HSP complicated by acalculous cholecystitis. PMID:27077617

  13. 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:

  14. Cocaine/levamisole-induced systemic vasculitis with retiform purpura and pauci-immune glomerulonephritis

    Directory of Open Access Journals (Sweden)

    F.V. Veronese

    2016-01-01

    Full Text Available Levamisole has been increasingly used as an adulterant of cocaine in recent years, emerging as a public health challenge worldwide. Levamisole-associated toxicity manifests clinically as a systemic vasculitis, consisting of cutaneous, hematological, and renal lesions, among others. Purpura retiform, cutaneous necrosis, intravascular thrombosis, neutropenia, and less commonly crescentic nephritis have been described in association with anti-neutrophil cytoplasmic antibodies (ANCAs and other autoantibodies. Here we report the case of a 49-year-old male who was a chronic cocaine user, and who presented spontaneous weight loss, arthralgia, and 3 weeks before admission purpuric skin lesions in the earlobes and in the anterior thighs. His laboratory tests on admission showed serum creatinine of 4.56 mg/dL, white blood count 3,800/μL, hemoglobin 7.3 g/dL, urinalysis with 51 white blood cells/μL and 960 red blood cells/μL, and urine protein-to-creatinine ratio 1.20. Serum ANCA testing was positive (>1:320, as well as serum anti-myeloperoxidase and anti-proteinase 3 antibodies. Urine toxicology screen was positive for cocaine and levamisole, with 62.8% of cocaine, 32.2% of levamisole, and 5% of an unidentified substance. Skin and renal biopsies were diagnostic for leukocytoclastic vasculitis and pauci-immune crescentic glomerulonephritis, respectively. The patient showed a good clinical response to cocaine abstinence, and use of corticosteroids and intravenous cyclophosphamide. Last serum creatinine was 1.97 mg/dL, white blood cell count 7,420/μL, and hemoglobin level 10.8 g/dL. In levamisole-induced systemic vasculitis, the early institution of cocaine abstinence, concomitant with the use of immunosuppressive drugs in severe cases, may prevent permanent end organ damage and associate with better clinical outcomes.

  15. 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

  16. Trombocitopenia fetal/neonatal aloinmune: Revisión a propósito de un caso Foetal/neonatal alloimmune thrombocytopenia: A review and case report

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    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

  17. Differential clot stabilising effects of rFVIIa and rFXIII-A2 in whole blood from thrombocytopenic patients and healthy volunteers

    DEFF Research Database (Denmark)

    Johansson, Per Ingemar; Jacobsen, Niels; Viuff, D.; Olsen, E.H.; Rojkjaer, R.; Andersen, S.; Petersen, Claus Leth; Kjalke, M.

    2008-01-01

    The haemostatic effect of recombinant activated factor VII (rFVIIa;NovoSeven) in thrombocytopenic patients has been a matter of controversy. Haemostasis by rFVIIa occurs via FVIIa-mediated thrombin generation in a platelet-dependent manner and may therefore be suboptimal in patients without....../ml). rFVIIa only modestly improved anti-fibrinolysis,whereas rFXIII-A2 (0-20 microg/ml) enhanced anti-fibrinolysis without effect on clotting time. TEG analysis showed rFVIIa shortened the clotting time, and enhanced clot development, maximal mechanical strength and resistance to fibrinolysis, whereas, r...

  18. Henoch-Schönlein purpura in an older man presenting as rectal bleeding and IgA mesangioproliferative glomerulonephritis: a case report

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    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

  19. A Case of Thrombotic Thrombocytopenia Purpura Associated with Systemic Lupus Erythematosus: Diagnostic Utility of ADAMTS-13 Activity

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    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.

  20. 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...

  1. 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

  2. Vincristine Injection

    Science.gov (United States)

    ... also sometimes used to treat thrombotic thrombocytopenic purpura (TPP; a blood disorder that causes blood clots to ... your medications or monitor you carefully for side effects.tell your doctor what herbal products you are ...

  3. 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 ...

  4. 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, ...

  5. 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

  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. 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)

    Jacob Couturier

    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.

  8. 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.

  9. 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

  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. Post-transfusion purpura in an African-American man due to human platelet antigen-5b alloantibody: a case report

    Directory of Open Access Journals (Sweden)

    Lynce Filipa

    2012-12-01

    Full Text Available Abstract Introduction Post-transfusion purpura is a rare immunohematological disorder characterized by severe thrombocytopenia following transfusion of blood components and induced by an alloantibody against a donor platelet antigen. It occurs primarily in women sensitized by pregnancy and is most commonly caused by anti-human platelet antigen-1a antibodies. Here, we describe what we believe to be the first documented case of an African-American man who developed post-transfusion purpura due to an anti-human platelet antigen-5b alloantibody after receiving multiple blood products. Case presentation A 68-year-old African-American man initially admitted with atrial flutter was started on anticoagulation treatment, which was complicated by severe hematemesis. On days 4 and 5 of hospitalization, he received six units of packed red blood cells, and on days 4, 13 and 14 he received plasma. His platelet count began to drop on day 25 and on day 32 reached a nadir of 7 × 109/L. His platelet count increased after receiving intravenous immune globulin. An antibody with reactivity to human platelet antigen-5b was detected by a solid-phase enzyme-linked immunoassay. Our patient was homozygous for human platelet antigen-5a. Conclusion This case emphasizes the importance of including post-transfusion purpura in the differential diagnosis for both men and women with acute onset of thrombocytopenia following transfusion of blood products. The prompt recognition of this entity is crucial for initiation of the appropriate management.

  13. 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.

  14. 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...

  15. 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

  16. Similar disturbances in B cell activity and regulatory T cell function in Henoch-Schonlein purpura and systemic lupus erythematosus

    International Nuclear Information System (INIS)

    The immunoglobulin synthesizing activities of peripheral mononuclear cells (MNC) from five patients with Henoch-Schonlein purpura (HSP) and eight patients with active systemic lupus erythematosus (SLE) were compared. Cumulative amounts of IgM, IgG, and IgA synthesized and secreted by unstimulated and PWM-stimulated patient cells over a 12-day period were determied in a solid-phase radioimmunoassay. In unstimulated control cultures mean rates of IgM, IgG, and IgA synthesis were less than 250 ng/ml. The synthetic activities of patient MNC were markedly increased. In HSP cultures IgA was the major immunoglobulin class produced (2810 x/divide 1.33 ng/ml) followed by IgG (1754 x/divide 1.32 ng/ml) and IgM (404 x/divide 1.16 ng/ml). In SLE cultures IgA and IgG syntheses were equally elevated (4427 x/divide 1.20 and 4438 x/divide 1.49 ng/ml, respectively) whereas IgM synthesis averaged 967 x/divide 1.66 ng/ml. PWM stimulation of pateient MNC caused a sharp decline in the synthesis of all three immunoglobulin classes. After T cell depletion B cell-enriched fractions from HSP and SLE patients maintained high levels of IgA and IgG synthesis that were inhibited by PWM and by normal allogeneic but not autologous T cells. In PWM-stimulted co-cultures, patient T cells nonspecifically suppressed the synthetic activities of autologous and control B cells. in contrast patient B cells achieved normal levels of immunoglobulin synthesis when cultured with control T cells plus PWM. In longitudinal studies patient B and T cell disturbances persisted despite clinical improvement

  17. 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; Dolezalova, Pavla; Brogan, Paul; Cabral, David A; Cuttica, Ruben; Khubchandani, Raju; Lovell, Daniel J; O'Neil, Kathleen M; Quartier, Pierre; Ravelli, Angelo; Iusan, Silvia M; Filocamo, Giovanni; Magalhães, Claudia Saad; Unsal, Erbil; Oliveira, Sheila; Bracaglia, Claudia; Bagga, Arvind; Stanevicha, Valda; Manzoni, Silvia Magni; Pratsidou, Polyxeni; Lepore, Loredana; Espada, Graciela; Kone-Paut, Isabella; Paut, Isabelle Kone; Zulian, Francesco; Barone, Patrizia; Bircan, Zelal; Maldonado, Maria del Rocio; Russo, Ricardo; Vilca, Iris; Tullus, Kjell; Cimaz, Rolando; Horneff, Gerd; Anton, Jordi; Garay, Stella; Nielsen, Susan Mary; Barbano, Giancarlo; Martini, Alberto

    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....

  18. 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

  19. 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.

  20. 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

  1. 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.

  2. 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.

  3. 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

  4. 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 ...

  5. 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.

  6. 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...

  7. 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 ...

  8. Serum Thrombopoietin and cMpl Expression in Thrombocytopenia of Different Etiologies

    OpenAIRE

    Fabrizio Vianello; Silvia Vettore; Fabiana Tezza; Luca De Toni; Raffaella Scandellari; Luisa Sambado; Martina Treleani; Fabrizio Fabris

    2014-01-01

    The relationship between thrombopoietin (TPO) and its receptor cMpl in thrombocytopenic conditions has not been entirely clarified. To elucidate this interplay may expand the spectrum of indications of TPO mimetics. In this study we have explored the relationship between TPO and cMpl in platelets and megakaryocytes of 43 patients with thrombocytopenia due to idiopathic thrombocytopenic purpura (ITP), bone marrow hypoplasia, myelodysplastic syndromes (MDS), and familial thrombocytopenia. Data ...

  9. 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...

  10. A Case of Microangiopathic Hemolytic Anemia after Myxoma Excision and Mitral Valve Repair Presenting as Hemolytic Uremic Syndrome

    Science.gov (United States)

    Park, Young Joo; Kim, Sang Pil; Shin, Ho-Jin

    2016-01-01

    Microangiopathic hemolytic anemia occurs in a diverse group of disorders, including thrombotic thrombocytopenic purpura, hemolytic uremic syndrome, and prosthetic cardiac valves. Hemolytic anemia also occurs as a rare complication after mitral valve repair. In this report, we describe a case of microangiopathic hemolytic anemia following myxoma excision and mitral valve repair, which was presented as hemolytic uremic syndrome. PMID:27081450

  11. 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

  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. 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

  14. 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.

  15. 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...

  16. 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

  17. 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)

  18. 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.

  19. 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

  20. 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

  1. 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

  2. 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...... exposes N-acetylgalactosamine-containing neoepitopes recognized by naturally occurring IgG or IgA1 antibodies resulting in formation of immune complexes. IgA1 contains up to six O-glycosylation sites per heavy chain; it is not known whether the glycosylation defect occurs randomly or preferentially 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...

  3. 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

  4. 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

    Directory of Open Access Journals (Sweden)

    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

  5. 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

  6. Intracranial Hemorrhage as a Source of Headache in a Patient with Postpartum

    Directory of Open Access Journals (Sweden)

    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.

  7. Immune thrombocytopenia. Use of a Coombs antiglobulin test to detect IgG and C3 on platelets

    International Nuclear Information System (INIS)

    We applied a radiolabeled Coombs antiglobulin test to the diagnosis and management of immune thrombocytopenia in adults and children. This assay substantiated that the majority of patients with idiopathic thrombocytopenic purpura have increased levels of IgG on their platelets. Platelets from a patient with the post-transfusion-purpura syndrome also carried increased IgG, indicating a role for IgG antibody or IgG-containing immune complexes in the destruction of host platelets in this disease. The radiolabeled Coombs test provides a general means to help diagnose, manage, and study immune platelet disorders

  8. 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

  9. 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

  10. 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

  11. 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...

  12. 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

    Directory of Open Access Journals (Sweden)

    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

  13. 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

    Directory of Open Access Journals (Sweden)

    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

  14. 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 ...

  15. 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 ...

  16. 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...

  17. 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 ...

  18. 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 (...

  19. 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...

  20. A case of congenital TTP presenting with microganiopathy in adulthood

    OpenAIRE

    Gallivan, Chris D; Conrad, David M; Kew, Andrea K

    2014-01-01

    Background Congenital thrombotic thrombocytopenic purpura (TTP), also known as Upshaw-Schulman Syndrome is a rare inherited deficiency of ADAMTS13. Unlike the more common acquired TTP which is characterized by an acquired inhibitor of ADAMTS13, patients with congenital TTP have an absolute deficiency of ADAMTS13 without an inhibitor. Congenital TTP generally presents in infancy with repeat episodes of acute hemolysis and evidence of microangiopathy, these episodes are usually triggered by ill...

  1. The Presence of Precursors of Benign Pre-B Lymphoblasts (Hematogones in the Bone Marrow of a Paediatric Patient with Cytomegalovirus Infection

    Directory of Open Access Journals (Sweden)

    Moreno-Madrid F

    2008-01-01

    Full Text Available Hematogones are normal B-lymphoid precursors that multiply in the bone marrow of small children and of adults with ferropenic anaemia, neuroblastoma or idiopathic thrombocytopenic purpura. They are not normally found in peripheral blood, and the immunophenotype is virtually indistinguishable from that of B lymphoblasts. We discuss the case of a 3-month infant with an active cytomegalovirus infection, with hepatitis and pancytopenia associated with 13% hematogones in the bone marrow.

  2. Is it safe to avoid bone marrow examination in suspected itp?

    Science.gov (United States)

    Naithani, Rahul; Kumar, Rajat; Mahapatra, M; Agrawal, Neerja; Pati, H P; Choudhry, V P

    2007-01-01

    Two children with suspected ITP are described. One child was treated outside with corticosteroids and was diagnosed acute lymphoblastic leukemia. Another child was fresh and diagnosed as acute myeloid leukemia on bone marrow aspirate examination. Both the children had no physical or peripheral smear finding suggestive of leukemia. We suggest that a bone marrow examination is required in developing countries for evaluation of thrombocytopenia before labeling it an immune thrombocytopenic purpura. PMID:17454791

  3. 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

  4. 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

  5. Henoch-Schönlein Purpura

    Science.gov (United States)

    ... campuses in Maryland and Arizona Research Resources Protocols, repositories, mouse models, plasmids, and more Technology Advancement & Transfer ... through its clearinghouses and education programs to increase knowledge and understanding about health and disease among patients, ...

  6. Palpable Purpura with an Unexpected Outcome

    Directory of Open Access Journals (Sweden)

    Anne Friesgaard Christensen

    2013-01-01

    Full Text Available Scurvy is a potentially lethal condition caused by inadequate vitamin C intake. Hypovitaminosis of vitamin C causes vessel fragility and follicular hyperkeratosis that can lead to palpable purpuric skin lesions. In this case report, we aim to remind readers that scurvy still occurs in Western countries and that the clinical presentation may resemble vasculitis.

  7. Palpable Purpura with an Unexpected Outcome

    OpenAIRE

    Anne Friesgaard Christensen; Ole Clemmensen; Peter Junker

    2013-01-01

    Scurvy is a potentially lethal condition caused by inadequate vitamin C intake. Hypovitaminosis of vitamin C causes vessel fragility and follicular hyperkeratosis that can lead to palpable purpuric skin lesions. In this case report, we aim to remind readers that scurvy still occurs in Western countries and that the clinical presentation may resemble vasculitis.

  8. 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 ...

  9. 复方甘草酸苷注射液治疗过敏性紫癜临床疗效及安全性观察%Clinical effect and safety observation of compound glycyrrhizin injection in the treatment of Anaphylactoid purpura

    Institute of Scientific and Technical Information of China (English)

    刘磊; 张晓彬

    2014-01-01

    Objective To observe the clinical efficacy and security of compound glycyrrhizin injection in the treatment of Anaphylactoid purpura. Methods 60 patients with Anaphylactoid purpura were randomly divided into two groups using contrast method,Control group was given routine treatment, antihistamines, vitamin C, rutin and symptomatic treatment,the kinds of joint, gastrointestinal tract,kidney type,mixed were added in a needle of prednisolone 1-2mg/(kg·d) by intravenous drip, and the dosage was reducted after symptoms improved;while the treatment group was given the compound glycyrrhizin injection(meineng),intravenous infusion of 10mL/d for 12 years old.in addition to the treatment in the control group,The clinical efficacy of conjunction use of 12 days was observed.The symptoms and skin recovery were observed. Results The total effective rate of treatment group was 93.3%,which was significantly better than that in the control group (73.3%) , the difference was statistically significant(x2=4.320,P<0.05).The cure rate and effective rate in treatment group were significantly higher than that in the control group. Adverse reactions can be tolerated and go away after drug withdrawal. Conclusion compound glycyrrhizin injection was safe and effective in the treatment of Anaphylactoid purpura,had higher clinical value,and was worthy of clinical promotion.%目的:观察复方甘草酸苷治疗过敏性紫癜的临床疗效和安全性。方法采用对比法将60例过敏性紫癜患者随机分为两组:对照组常规治疗,抗组胺药、维生素C、芦丁及对症治疗,关节型、胃肠道型、肾型、混合型患者加用甲泼尼龙针1~2mg/(kg·d)静脉滴注,好转后减量;治疗组在以上对照组常规治疗基础上加用复方甘草酸苷(美能)注射液,≤12岁10mL/d,>12岁20mL/d,静脉滴注,疗程均12d,观察患者症状及皮损恢复情况。结果治疗组有效率为93.3%,对照组有效率为73.3%,两组比

  10. Application of Combined Bacillus Subtilis and Enterococcus Faecium Granules with Multivitamines-Live in Treatment of Henoch Purpura in Children%枯草杆菌二联活菌颗粒在儿童腹型过敏性紫癜中的应用

    Institute of Scientific and Technical Information of China (English)

    左满凤; 艾柳; 舒琼璋; 董晶

    2012-01-01

    Objective:To observe the adjunctive effect of the viable organism praeparatum Combined Bacillus Subtilis and Enterococcus Faecium Granules with Multivitamines-Live(Medilae-Vita) in the treatment of henoch purpura in children. Methods: Together 139 children with henoch purpura as research subjects were divided into observation group and control group by random number table method. In the control group, children were treated with cimetidine, vitamin C, calcium, glucocorticosteroid; and antibiotics therapy was given to those who had combined infection. The observation group were given Medilac-Vita in addition to the same treatment as the control group. Aged 4~6 years, 1 g each time; aged - 12 years, 1. 5 g each time; all three times a day, 7 days as a course of treatment. The symptom changes of digestive tube ( stomachache, emesia, hemafecia), levels of salivary secretary IgA ( sIgA) and clinical effect after treatment in I week of the two groups were observed. Results: The extinction time of digestive tube symptoms in observation group was less than that of the control group (P0.05). The total effective rate in observation group was 95.71% , while that of the control group was 81.82% , there was significant difference in the two groups (x2 = 4. 978, P<0. 05). Conclusions: Viable organism praeparatum Medilac-Vila can enhance the immunity of mucous membrane. In the treatment of henoch purpura in children, it can relieve digestive tube symptoms quickly, prevent alimentary tract hemorrhage, and shorten the course of treatment%目的:观察枯草杆菌二联活菌颗粒在儿童腹型过敏性紫癜(HSP)治疗中的辅助作用.方法:将139例腹型HSP住院患儿中完成研究的125例纳入研究对象,采用随机数字表法随机分为观察组70例和对照组55例.对照组给予西咪替丁、维生素C、钙剂及糖皮质激素等综合治疗,合并感染者给予抗生素治疗,观察组在对照组治疗基础上加服枯草杆菌二联活菌颗粒,年龄4

  11. Serum thrombopoietin and cMpl expression in thrombocytopenia of different etiologies

    Directory of Open Access Journals (Sweden)

    Fabrizio Vianello

    2014-03-01

    Full Text Available The relationship between thrombopoietin (TPO and its receptor cMpl in thrombocytopenic conditions has not been entirely clarified. To elucidate this interplay may expand the spectrum of indications of TPO mimetics. In this study we have explored the relationship between TPO and cMpl in platelets and megakaryocytes of 43 patients with thrombocytopenia due to idiopathic thrombocytopenic purpura (ITP, bone marrow hypoplasia, myelodysplastic syndromes (MDS, and familial thrombocytopenia. Data were compared to cMpl and TPO in patients with a normal platelet count and in patients with thrombocytosis due to essential thrombocythemia (ET. All but familial patients showed higher TPO compared to controls. All thrombocytopenic states were invariably associated with increased expression of platelet cMPL compared to healthy controls. ET patients showed normal TPO and a trend toward a reduced cMpl expression. Immunofluorescence of bone marrow sections from patients with ITP and MDS failed to show a peculiar pattern compared to controls. Multiple mechanisms regulate TPO and cMpl in thrombocytopenic conditions.

  12. A Case of Microangiopathic Antiphospholipid-Associated Syndromes during Pregnancy: Review of the Literature

    Directory of Open Access Journals (Sweden)

    Nobuhiro Suzumori

    2012-01-01

    Full Text Available Microangiopathic antiphospholipid-associated syndromes (MAPSs are reported as encompassing several conditions mainly affecting the microvasculature of selected organs: the liver in HELLP syndrome (hemolysis, elevated liver enzymes, and low platelet; kidney, brain, and skin in TTP (thrombotic thrombocytopenic purpura. It is predominant in patients with catastrophic antiphospholipid syndrome (APS. A recent report suggests that APS is not only a thrombotic disease but also associated with microangiopathic features, and it can explain the greater prevalence of HELLP syndrome in these patients. We here report a case of MAPS during pregnancy associated with systemic lupus erythematosus (SLE in early second trimester.

  13. Pathophysiology of tumour-induced microangiopathic haemolytic anaemia.

    Science.gov (United States)

    Chalasani, Pavani; Segar, Jennifer M; Marron, Marilyn; Stopeck, Alison

    2016-01-01

    Cancer-associated microangiopathic haemolytic anaemia (CA-MAHA) is a syndrome characterised by Coombs-negative haemolytic anaemia and thrombocytopenia. It is primarily seen in advanced solid tumours and is distinct from thrombotic thrombocytopenic purpura/haemolytic uraemic syndrome. Diagnosis is often delayed and patients have a high mortality. We present the case of CA-MAHA in a patient with metastatic breast cancer treated successfully with early initiation of chemotherapy. In addition, we report longitudinal laboratory evaluation of circulating tumour cells and microparticles and suggest a hypothesis for the mechanism behind CA-MAHA. PMID:26744538

  14. A first case of congenital TTP on the African continent due to a new homozygous mutation in the catalytic domain of ADAMTS13.

    Science.gov (United States)

    Meyer, Sara C; Jeddi, Ramzi; Meddeb, Balkis; Gouider, Emna; Lämmle, Bernhard; Kremer Hovinga, Johanna A

    2008-08-01

    Hereditary thrombotic thrombocytopenic purpura (TTP) is a rare disorder characterized by occlusive microvascular thrombosis, consumptive thrombocytopenia, and microangiopathic hemolytic anemia. Homozygous or compound heterozygous mutations in the ADAMTS13 gene result in a congenital severe ADAMTS13 deficiency and subsequent accumulation of ultra-large von Willebrand factor multimers, which tend to form platelet thrombi in the microcirculation. We report a first case of congenital TTP on the African continent with a new, homozygous mutation in the metalloprotease domain of ADAMTS13. An initially oligo-symptomatic presentation was followed by acute exacerbation with ischemic stroke and acute renal failure highlighting the severity of this syndrome. PMID:18443791

  15. Thrombocytopenia-associated multiple organ failure or severe haemolysis, elevated liver enzymes, low platelet count in a postpartum case

    Directory of Open Access Journals (Sweden)

    Manish Jagia

    2013-01-01

    Full Text Available Thrombocytopenia-associated multiple organ failure (TAMOF is a thrombotic microangiopathic syndrome that includes thrombotic thrombocytopenic purpura, secondary thrombotic microangiopathy, and disseminated intravascular coagulation. We report a case of postpartum female who presented with TAMOF or severe Haemolysis, elevated liver enzymes, low platelet count (HELLP which was managed with plasma exchange. This case report is to make clinicians aware that TAMOF, severe HELLP, and other differential diagnosis in a postpartum case have a thin differentiating line and plasma exchange can be considered as one of the management options.

  16. Parvovirus B19 infection presenting as pre-B-cell acute lymphoblastic leukemia: a transient and progressive course in two children.

    Science.gov (United States)

    Yetgin, Sevgi; Cetin, Mualla; Aslan, Deniz; Ozyurek, Emel; Oyürek, Emel; Anlar, Banu; Uçkan, Duygu

    2004-10-01

    Parvovirus B19 is the causative agent of various forms of hematologic diseases such as aplastic crisis in patients with hemolytic anemia, aplastic anemia, hypoplastic anemia, and idiopathic thrombocytopenic purpura. In addition, parvovirus B19 infection may precede or be associated with acute lymphoblastic leukemia (ALL). The authors present two cases of parvovirus B19 infection and bone marrow infiltration with pre-B-cell lymphoblasts; one patients was diagnosed as having ALL, and the other patient, with neurologic findings, showed total resolution of the blastic morphology and phenotype. PMID:15454845

  17. Management of Thrombotic Microangiopathic Hemolytic Anemias with Therapeutic Plasma Exchange: When It Works and When It Does Not.

    Science.gov (United States)

    Mehmood, Tahir; Taylor, Michelle; Winters, Jeffrey L

    2016-06-01

    Thrombotic microangiopathies are a heterogeneous group of inherited and acquired disorders sharing a common clinical presentation of microangiopathic hemolytic anemia, thrombocytopenia, and organ damage. These disorders have been treated with plasma exchange (TPE) based on randomized controlled trials, which found this therapy to be effective in thrombotic thrombocytopenic purpura (TTP). For the remaining disorders, low- to very low-quality evidence exists for the use of TPE. When TPE is applied, the treatment regimen used for TTP is usually applied. There is a need for further evaluation of the role of TPE in the treatment of thrombotic microangiopathies other than TTP. PMID:27113004

  18. Suppression of spleen pathological function by roentgenoendovascular occlussion in some blood systemic diseases

    International Nuclear Information System (INIS)

    It is shown that REO of splenic artery in thrombocytopenic purpura, hypoplastic and autoimmune hemolytic anemia manifests the same clinical effect as splenectomy. This treatment procedure may substitute splenectomy in a definite group of patients with above-mentioned blood systemic diseases or precede it as a preparation and prognosis stage. Stage-by-stage total embolization of splenic artery is a necessary technical method permitting to attain the necessary medical effect without risk of developing acute spleen infarction. At present REO is used in some blood diseases in patients with high risk of operation, particularly with active hemorrhagic syndrome of adrenal insufficiency, concomitant inflammatory processes

  19. Proportion of drug-related serious rare blood dyscrasias: estimates from the Berlin Case-Control Surveillance Study.

    Science.gov (United States)

    Andersohn, Frank; Bronder, Elisabeth; Klimpel, Andreas; Garbe, Edeltraut

    2004-11-01

    Drugs are an important cause of serious rare blood dyscrasias. To estimate the proportion of drug-related cases, we used data from the ongoing Berlin Case-Control Surveillance Study. The analysis included a total of 171 cases. The number of cases in which a drug etiology was assessed as at least "possible" was n = 29 (97%) for acute agranulocytosis, n = 4 (0.21%) for aplastic anemia, n = 8 (26%) for immune hemolytic anemia, n = 20 (25%) for immune thrombocytopenia, and n = 2 (20%) for thrombotic thrombocytopenic purpura/hemolytic uremic syndrome. Our analysis suggests that a substantial fraction of blood dyscrasias may be attributable to drug therapy. PMID:15495238

  20. An Unusual Case of Rapidly Progressive Hyperbilirubinemia

    Directory of Open Access Journals (Sweden)

    Kimberly M. Thornton

    2013-01-01

    Full Text Available We present an unusual case of hyperbilirubinemia with rapid early progression leading to bilirubin encephalopathy in a term neonate. Despite early recognition and intervention, the total serum bilirubin reached a maximum level of 39 mg/dL at 32 hours of life. Prior to an emergent exchange transfusion, the patient’s diagnostic evaluation was significant for Coombs-negative microangiopathic hemolytic anemia and thrombocytopenia. Further testing revealed a deficiency of ADAMTS13 protein, or von Willebrand factor-cleaving protease, a finding diagnostic of congenital thrombotic thrombocytopenic purpura, or Upshaw-Schulman syndrome. This rare disease is often misdiagnosed, especially in the newborn period.

  1. Thrombocytopenia and Cornelia de Lange syndrome: Still an enigma?

    Science.gov (United States)

    Cavalleri, Valeria; Bettini, Laura R; Barboni, Chiara; Cereda, Anna; Mariani, Milena; Spinelli, Marco; Gervasini, Cristina; Russo, Silvia; Biondi, Andrea; Jankovic, Momcilo; Selicorni, Angelo

    2016-01-01

    Cornelia de Lange Syndrome (CdLS) is a rare genetic disorder caused by mutations in the cohesion complex and its regulators. The syndrome is characterized by multiple organ system abnormalities, pre- and post-natal growth retardation and typical facial features. Thrombocytopenia is a reduction in platelet count to <150 × 10(9)  L. It can be caused by congenital or acquired decreased production, increased destruction, or sequestration of platelets. In recent years, several papers reported thrombocytopenia and immune thrombocytopenia in patients affected by CdLS. In 2011, Lambert et al. estimated the risk of idiopathic thrombocytopenia purpura in CdLS patients to be 31-633 times greater than in the general population. We describe the incidence of thrombocytopenia in 127 Italian CdLS patients, identifying patients with transient or persistent thrombocytopenia, but a lower incidence of true idiopathic thrombocytopenic purpura (ITP). PMID:26437745

  2. 浆细胞样树突状细胞在过敏性紫癜患儿外周血和肾组织中表达的意义%Expression of plasmacytoid dendritic cells in peripheral blood and renal tissues in children with Henoch-Sch(o)nlein purpura

    Institute of Scientific and Technical Information of China (English)

    王娟; 郭桂梅; 夏敏; 郑林; 郝胜; 黄文彦; 何威逊

    2014-01-01

    目的 研究浆细胞样树突状细胞(pDC)在过敏性紫癜(HSP)患儿外周血和肾脏组织中的表达和分布情况,探讨pDC在紫癜性肾炎发病中的作用.方法 40例HSP患儿,其中急性期28例,缓解期12例,分离患儿外周血单个核细胞,流式细胞术检测pDC的表达;设立正常对照组(15例).同时抽提外周血总RNA并反转录为cDNA,以实时荧光定量PCR法检测患者组和正常对照组趋化因子干扰素γ诱导蛋白10 (CXCL10)、CC类趋化因子配体5(CCL5)及相应受体CXC亚家族趋化因子受体3(CXCR3)、趋化因子受体5(CCR5)mRNA的定量表达水平(以2-△△Ct值表示).应用免疫组织化学技术,观察8例行肾活检患者肾脏组织中pDC的分布;设立正常对照(3例).结果 HSP急性期患者外周血pDC表达的百分数为0.051±0.039,低于缓解期的0.181 ±0.082和正常对照组的0.166±0.079(P<0.000 1).HSP患者外周血高表达CXCL10、CCL5,而低表达CXCR3、CCR5.正常对照肾脏组织未见pDC浸润,而紫癜性肾炎患者pDC呈局灶节段分布于肾小球系膜区.结论 pDC在HSP患儿中表达异常,紫癜性肾炎的发生可能与外周血中pDCs在趋化因子作用下向肾组织的迁移,引起局部炎性反应有关.%Objective To investigate the expression and distribution of plasmacytoid dendritic cells(pDC) in peripheral blood and renal tissues in children with Henoch-SchSnlein purpura(HSP),and explore the role of pDCs in the pathogenesis of Henoch-Schtnlein purpura nephritis(HSPN).Methods Among the 40 children with HSP,28 cases were in the active phase(renal biopsy performed in 8 cases of them) and the other 12 in remission phase.Peripheral blood mononuclear cells were isolated,and the expression of pDC was detected by flow cytometry.The normal control group was established (n =15).Total RNA of peripheral blood was extracted and transcripted into cDNA.Sybr green dye based real-time quantitative PCR method was used to compare the expression

  3. Study on the Incidence of Hospitalized Henoch-Sch(o)nlein Purpura Cases in 6 Cities in China,2007-2009%中国六个市2007~2009年过敏性紫癜住院病例发病情况分析

    Institute of Scientific and Technical Information of China (English)

    李克莉; 方兴; 王华庆; 罗会明; 梁晓峰; 刘大卫; 武文娣; 许涤沙; 涂秋凤; 郑敏; 丁峥嵘; 唐婷婷; 韩悦

    2011-01-01

    Objective To analyze the incidence and epidemiological characteristics of Henoch-Sch()nlein Purpura(HSP)in selected districts in China, and to assess safety of vaccination. Methods The basic information on hospitalized HSP cases occurred in 2007-2009 were collected from hospitals at county level in 6 cities from Yunnan, Jiangxi, and Liaoning provinces, by multi-stage stratified cluster sampling method. The descriptive methodology is used for this study. Results Daring 2007-2009,7743 hospitalized HSP eases occurred with an average incidence rate of 8.82 per 100, 000 people.Among them, 5677 children were <15 years old, incidence rate was 33.86 per 100000, and 1639 (28.87%)with a definitely risk factors before onset. In 6 cities, average incidence rates among 3 years old children were 4.08, 6.28, 15.28 per 100, 000 people respectively, and 14.48, 24.59, 59.42 per 100,000 in children aged < 15 years. Incidence pick was in spring in 2 cities of Yunnan, and in autum and winter in 4 cities in Jiangxi and Liaoning provinces. The incidence in group of aged 5-9 years were the highest in the investigated areas in 3 provinces. Conclusions During 2007-2009,the incidence rates of hospitalized HSP were relatively stable for whole population and < 15 years old children in the investigated areas in 3 provinces. However, there were apparently differences in incidence and seasonal characteristic from different provinces. These findings suggested that the time for mass campaign vaccination should be conducted according the local seasonal characteristic of HSP. Before vaccination, all recipients or their guardians should be asked carefully by vaccination providers to avoid risk factors, such as infection and allergy history.%目的 分析中国六个市过敏性紫癜(Henoch-sch(o)nlein Purpura,HSP)的发生率及流行病学特征,为开展预防接种的安全性评价提供参考依据.方法 采用分层多阶段整群抽样的方法,选择江西省南昌、宜春、景德

  4. Role of HLA antigens in Rh (D) alloimmunized pregnant women from Mumbai, Maharashtra, India.

    Science.gov (United States)

    Kumar, U Shankar; Ghosh, K; Gupte, S S; Gupte, S C; Mohanty, D

    2002-03-01

    Immunogenetic studies in various diseases provide potential genetic markers. We have studied the incidence of HLA A, B, C, DR and DQ loci antigen in Rh (D) antigen isoimmunized mothers compared to those nonimmunized isoimmunized Rh negative mothers. Seventy six mothers who were immunized to Rh (D) antigen due to pregnancy (responders) and fifty four mothers who did not develop Rh (D) isoimmunization despite positive pregnancies (nonresponders) were selected for the study. Standard methods of serological HLA typing, ABO and Rh (D) groups, and screening for Rh D antibodies were used. 392 unrelated individuals from the population were compared as controls. In addition 45 unrelated individuals from the same population were typed for HLA DRB and DQB gene using PCR-SSP kits. The genotype frequencies of HLA A2, A3, A28, B13, B17, B35, B52, B60, Cw2, Cw6, DR4, and DQ3 were significantly increased, while the frequencies of the HLA A11, A29, A31, B7, B37, B51, Cw1 and DR9 were decreased in the responder women when compared to the non-responder women. HLA A30 (19) split antigen was not identified in immunized women while HLA A23 (9) split antigen was not identified in non immunized women. HLA A3, B17, Cw2 and DR4 showed a significant relative risk among the immunized responder women. When compared with Rh immunized women (responders) reported from USA, England and Hungary the phenotype frequencies of HLA A11, A24, A28, B5, B17, B40, DR2 and DR5 were increased while HLA A23, B8, B18, and DR6 were decreased in the Indian Rh immunized women. Two locus haplotype frequency analysis observed among the responders women revealed that among the significant haplotypes expressed A2-B5, B7-Cw1, DR2-DQ1 were highly significant haplotypes in positive linkage, while A1-B5, and A1-B7 were in significant negative linkage disequilibrium. The haplotype frequencies were HLA A3, B17, Cw2 and DR4 increases the relative risk factor by 2.6 times among Indian Rh isoimmunized women. Further, it is evident that there are significant differences in the observed HLA antigen frequencies and two locus haplotypes in Rh isoimmunized women when compared to women from USA, UK and Hungary due to extreme HLA polymorphism in different populations of the world. PMID:11937684

  5. Anti-G antibody in alloimmunized pregnant women: Report of two cases.

    Science.gov (United States)

    Makroo, Raj Nath; Kaul, Anita; Bhatia, Aakanksha; Agrawal, Soma; Singh, Chanchal; Karna, Prashant

    2015-01-01

    Anti-G has been reported as a possible cause of hemolytic disease of the fetus and newborn (HDFN), either independently or in association with anti-D, anti-C or both. The antibody mimics the pattern of anti-C and anti-D reactivity in the identification panel and is often present along with either or both of these antibodies. The differentiation of anti-D, -C and-G in routine pretransfusion workup is particularly essential in antenatal cases. We report two antenatal cases where anti-G was identified on advanced immunohematological workup, in addition to other alloantibodies. PMID:26420948

  6. Anti-G antibody in alloimmunized pregnant women: Report of two cases

    OpenAIRE

    Raj Nath Makroo; Anita Kaul; Aakanksha Bhatia; Soma Agrawal; Chanchal Singh; Prashant Karna

    2015-01-01

    Anti-G has been reported as a possible cause of hemolytic disease of the fetus and newborn (HDFN), either independently or in association with anti-D, anti-C or both. The antibody mimics the pattern of anti-C and anti-D reactivity in the identification panel and is often present along with either or both of these antibodies. The differentiation of anti-D, -C and-G in routine pretransfusion workup is particularly essential in antenatal cases. We report two antenatal cases where anti-G was iden...

  7. Anti-G antibody in alloimmunized pregnant women: Report of two cases

    Directory of Open Access Journals (Sweden)

    Raj Nath Makroo

    2015-01-01

    Full Text Available Anti-G has been reported as a possible cause of hemolytic disease of the fetus and newborn (HDFN, either independently or in association with anti-D, anti-C or both. The antibody mimics the pattern of anti-C and anti-D reactivity in the identification panel and is often present along with either or both of these antibodies. The differentiation of anti-D, -C and-G in routine pretransfusion workup is particularly essential in antenatal cases. We report two antenatal cases where anti-G was identified on advanced immunohematological workup, in addition to other alloantibodies.

  8. Abacavir induced T cell reactivity from drug naive individuals shares features of allo-immune responses.

    Directory of Open Access Journals (Sweden)

    Jacqueline Adam

    Full Text Available Abacavir hypersensitivity is a severe hypersensitivity reaction which occurs exclusively in carriers of the HLA-B*57∶01 allele. In vitro culture of PBMC with abacavir results in the outgrowth of abacavir-reacting CD8+ T cells, which release IFNγ and are cytotoxic. How this immune response is induced and what is recognized by these T cells is still a matter of debate. We analyzed the conditions required to develop an abacavir-dependent T cell response in vitro. The abacavir reactivity was independent of co-stimulatory signals, as neither DC maturation nor release of inflammatory cytokines were observed upon abacavir exposure. Abacavir induced T cells arose in the absence of professional APC and stemmed from naïve and memory compartments. These features are reminiscent of allo-reactivity. Screening for allo-reactivity revealed that about 5% of generated T cell clones (n = 136 from three donors were allo-reactive exclusively to the related HLA-B*58∶01. The addition of peptides which can bind to the HLA-B*57∶01-abacavir complex and to HLA-B*58∶01 during the induction phase increased the proportion of HLA-B*58∶01 allo-reactive T cell clones from 5% to 42%. In conclusion, abacavir can alter the HLA-B*57∶01-peptide complex in a way that mimics an allo-allele ('altered self-allele' and create the potential for robust T cell responses.

  9. Post-transfusion and maternal red blood cell alloimmunization in Uganda

    NARCIS (Netherlands)

    Natukunda, Bernard

    2013-01-01

    Over the last two decades, there has been substantial progress in the area of blood safety in Uganda. In contrast, little attention has been paid to transfusion safety in Uganda and there are gaps in laboratory and clinical transfusion practices within hospitals. Assessment of the current practice a

  10. Role of HLA antigens in Rh (D) alloimmunized pregnant women from Mumbai, Maharashtra, India

    Indian Academy of Sciences (India)

    U Shankar Kumar; K Ghosh; S S Gupte; S C Gupte; D Mohanty

    2002-03-01

    Immunogenetic studies in various diseases provide potential genetic markers. We have studied the incidence of HLA A, B, C, DR and DQ loci antigen in Rh (D) antigen isoimmunized mothers compared to those nonimmunized isoimmunized Rh negative mothers. Seventy six mothers who were immunized to Rh (D) antigen due to pregnancy (responders) and fifty four mothers who did not develop Rh (D) isoimmunization despite positive pregnancies (nonresponders) were selected for the study. Standard methods of serological HLA typing, ABO and Rh (D) groups, and screening for Rh D antibodies were used. 392 unrelated individuals from the population were compared as controls. In addition 45 unrelated individuals from the same population were typed for HLA DRB and DQB gene using PCR-SSP kits. The genotype frequencies of HLA A2, A3, A28, B13, B17, B35, B52, B60, Cw2, Cw6, DR4, and DQ3 were significantly increased, while the frequencies of the HLA A11, A29, A31, B7, B37, B51, Cw1 and DR9 were decreased in the responder women when compared to the non-responder women. HLA A30 (19) split antigen was not identified in immunized women while HLA A23 (9) split antigen was not identified in non immunized women. HLA A3, B17, Cw2 and DR4 showed a significant relative risk among the immunized responder women. When compared with Rh immunized women (responders) reported from USA, England and Hungary the phenotype frequencies of HLA A11, A24, A28, B5, B17, B40, DR2 and DR5 were increased while HLA A23, B8, B18, and DR6 were decreased in the Indian Rh immunized women. Two locus haplotype frequency analysis observed among the responders women revealed that among the significant haplotypes expressed A2–B5, B7–Cw1, DR2–DQ1 were highly significant haplotypes in positive linkage, while A1–B5, and A1–B7 were in significant negative linkage disequilibrium. The haplotype frequencies were ≤ one when these common hapoltypes were compared with control population. Thus in the present study it is evident that the inheritance of HLA A3, B17, Cw2 and DR4 increases the relative risk factor by 2.6 times among Indian Rh isoimmunized women. Further, it is evident that there are significant differences in the observed HLA antigen frequencies and two locus haplotypes in Rh isoimmunized women when compared to women from USA, UK and Hungary due to extreme HLA polymorphism in different populations of the world.

  11. Allergic Conjunctivitis Exacerbates Corneal Allograft Rejection by Activating Th1 and Th2 Alloimmune Responses

    OpenAIRE

    Niederkorn, Jerry Y.; Chen, Peter W.; Mellon, Jessamee; Stevens, Christina; Mayhew, Elizabeth

    2010-01-01

    Allergic conjunctivitis (AC) and airway hyperreactivity exacerbate corneal allograft rejection. Because AC and airway hyperreactivity are allergic diseases of mucosal tissues, we determined whether an allergic disease of a nonmucosal tissue would affect corneal allograft rejection and whether Th2 cells alone accounted for accelerated graft rejection in allergic mice. Hosts sensitized cutaneously with short ragweed pollen developed cutaneous immediate hypersensitivity but rejected corneal allo...

  12. Cure of alopecia areata after eradication of Helicobacter pylori : A new association?

    Institute of Scientific and Technical Information of China (English)

    Germán Campuzano-Maya

    2011-01-01

    Alopecia areata is a disease of the hair follicles, with strong evidence supporting autoimmune etiology. Alopecia areata is frequently associated with immune-mediated diseases with skin manifestations such as psoriasis and lichen planus, or without skin manifestations such as autoimmune thyroiditis and idiopathic thrombocytopenic purpura. Helicobacter pylori (H. pylori ) infection is present in around 50% of the world's population and has been associated with a variety of immune-mediated extra-digestive disorders including autoimmune thyroiditis, idiopathic thrombocytopenic purpura, and psoriasis. A case of a 43-year old man with an 8-mo history of alopecia areata of the scalp and beard is presented. The patient was being treated by a dermatologist and had psychiatric support, without any improvement. He had a history of dyspepsia and the urea breath test confirmed H. pylori infection. The patient went into remission from alopecia areata after H. pylori eradication. If such an association is confirmed by epidemiological studies designed for this purpose, new therapeutic options could be available for these patients, especially in areas where infection with H. pylori is highly prevalent.

  13. Hepato-splenic lymphoma: a rare entity mimicking acute hepatitis: A case report

    Institute of Scientific and Technical Information of China (English)

    Federico Perfetto; Roberto Tarquini; Franceco Mancuso; Simonetta di Lollo; Silvia Tozzini; Giampiero Belesi; Giacomo Laffi

    2003-01-01

    We reported a case of non-Hodgkin's lymphoma where liver involvement was the predominant clinical manifestation. A 27-year old man presented wiht markedly elevated serum aspartate aminotrasferase, alanine aminotransferase and lactate dehydrogenase, reduced prothrombin activity, thrombocytopenic purpura and hepato-splenomegaly without adenopathy. Viral, toxic,autoimmune and metabolic liver diseases were excluded.Bone marrow biopsy showed an intracapillary infiltration of T-lymphocytes with no evidence of lipid storage disease.Because of a progressive spleen enlargement, splenectomy was performed. Histological examination showed lymphomatous intrasinuses invasion of the spleen.Immunohistochemical investigation revealed the T phenotype of the neoplastic cells: CD45+, CD45RO+,CD3+, CD4-, CD8-, TIA1-. About 50 % of the lymphoid cells expressed CD56 antigen. The diagnosis of hepatosplenic T cell lymphoma was done. The patient was treated with chemotherapy, which induced a complete remission. Eighteen months later, he had a first relapse with increased aspartate aminotransferase, alanine aminotransferase, lactate dehydrogenase,thrombocytopenic purpura and blast in the peripheral blood.In spite of autologous bone marrow transplantation, he died twenty months after the diagnosis. Even in the absence of a mass lesion or lymphoadenopathy, hepatosplenic Tcell lymphoma should be considered in the differential diagnosis of a patient whose clinical course is atypical for acute hepatic dysfunction.

  14. Epitope specificity and isotype of monoclonal anti-D antibodies dictate their ability to inhibit phagocytosis of opsonized platelets.

    Science.gov (United States)

    Kjaersgaard, Mimi; Aslam, Rukhsana; Kim, Michael; Speck, Edwin R; Freedman, John; Stewart, Donald I H; Wiersma, Erik J; Semple, John W

    2007-08-15

    Rh immune globulin (WinRho SDF; Cangene, Mississauga, ON, Canada) is an effective treatment for autoimmune thrombocytopenic purpura; however, maintaining a sustained supply for its use in autoimmune thrombocytopenic purpura and its primary indication, hemolytic disease of the newborn, makes the development of alternative reagents desirable. We compared Rh immune globulin and 6 human monoclonal anti-D antibodies (MoAnti-D) with differing isotypes and specificities for their ability to opsonize erythrocytes and inhibit platelet phagocytosis in an in vitro assay. Results demonstrated that opsonization of erythrocytes with Rh immune globulin significantly (P < .001) reduced phagocytosis of fluorescently labeled opsonized platelets in an Fc-dependent manner. Of the MoAnti-D that shared specificity but differed in isotype, only IgG3 antibodies could significantly (P < .001) inhibit platelet phagocytosis. In contrast, 2 MoAnti-D shared isotypes and differed in specificity; however, only one could significantly (P < .001) inhibit platelet phagocytosis. The results suggest that MoAnti-D epitope specificity and isotypes are critical requirements for optimal inhibition of opsonized platelet phagocytosis. PMID:17456719

  15. Antigen and substrate withdrawal in the management of autoimmune thrombotic disorders.

    Science.gov (United States)

    Cines, Douglas B; McCrae, Keith R; Zheng, X Long; Sachais, Bruce S; Luning Prak, Eline T; Siegel, Don L

    2012-11-15

    Prevailing approaches to manage autoimmune thrombotic disorders, such as heparin-induced thrombocytopenia, antiphospholipid syndrome and thrombotic thrombocytopenic purpura, include immunosuppression and systemic anticoagulation, though neither provides optimal outcome for many patients. A different approach is suggested by the concurrence of autoantibodies and their antigenic targets in the absence of clinical disease, such as platelet factor 4 in heparin-induced thrombocytopenia and β(2)-glycoprotein-I (β(2)GPI) in antiphospholipid syndrome. The presence of autoantibodies in the absence of disease suggests that conformational changes or other alterations in endogenous protein autoantigens are required for recognition by pathogenic autoantibodies. In thrombotic thrombocytopenic purpura, the clinical impact of ADAMTS13 deficiency caused by autoantibodies likely depends on the balance between residual antigen, that is, enzyme activity, and demand imposed by local genesis of ultralarge multimers of von Willebrand factor. A corollary of these concepts is that disrupting platelet factor 4 and β(2)GPI conformation (or ultralarge multimer of von Willebrand factor oligomerization or function) might provide a disease-targeted approach to prevent thrombosis without systemic anticoagulation or immunosuppression. Validation of this approach requires a deeper understanding of how seemingly normal host proteins become antigenic or undergo changes that increase antibody avidity, and how they can be altered to retain adaptive functions while shedding epitopes prone to elicit harmful autoimmunity. PMID:22966172

  16. 槐杞黄对紫癜性肾炎患儿Th17/Treg细胞免疫失衡的调节作用%Balance of regulation of Th17/Treg in children with Henoch-Sch(o)nlein purpura nephritis using Huaiqihuang

    Institute of Scientific and Technical Information of China (English)

    周杜鹃; 吴小川; 王复娟; 易著文; 曹艳; 黄丹琳; 何庆南; 党西强

    2013-01-01

    Objective To observe the levels of Th17 and associated cytokine IL-17 and Treg in peripheral blood in children with Henoch-Sch(o)nlein purpura nephritis (HSPN),and to explore the balance of regulation of Th17/Treg and the function of improvement of clinical symptoms in children with HSPN by using Huaiqihuang.Methods The objects were divided into 3 groups:healthy control group (n =11),Henoch-Schonlein purpura (HSP) group (n =10) and HSPN group(n =30).Healthy children were measured for the level of IL-17 by enzyme-linked immunosorbent assay(ELISA) and the circulating frequencies of Th17 and CD4 + CD25 + Treg cells by flow cytometry (FCM).HSP group and HSPN group were measured for the levels of IL-17,Th17 cells and Treg cells when illness had acute attack and had not used any drugs.Furthermore,HSPN group was measured for 24-hour urine proteins and urinary sediment red blood cells(RBCs).Patients in HSPN group with the pathological grading Ⅱ were divided into conventional therapy group(n =10) and Huaiqihuang group(n =10) randomly.Patients in HSPN group with the pathological grading Ⅲa were divided into Prednisone group(n =5) and Prednisone + Huaiqihuang group(n =5) randomly.Conventional therapy group was treated with oral Fosinopril and Dipyridamole.Huaiqihuang group was treated with conventional therapy and oral Huaiqihuang particles.Prednisone group was treated with conventional therapy and oral Prednisone.Prednisone +Huaiqihuang group was treated with conventional therapy,Prednisone and Huaiqihuang particles.After 2 months of treatments,the levels of IL-17,Th17 cells,Treg cells,24-hour urine proteins and urinary sediment RBCs were measured.Results The circulating frequencies of Th17 cells and IL-17 in HSP group and HSPN group were higher than those in healthy control group(all P < 0.05),while the circulating frequencies of Treg cells were lower than those in healthy control group(all P <0.05),but there was no significant difference between HSP group and

  17. Comprehensive three year progress report, August 1, 1975-October 31, 1978

    Energy Technology Data Exchange (ETDEWEB)

    Baldini, M G

    1978-01-01

    The major lines of investigation pursued during the past three years are listed here. A brief report and discussion on each subject of investigation is also given. Preservation and transfusion of viable human platelets studied were: improved preservation of platelets by freezing; and arrest and prevention of hemorrhage in alloimmunized thrombocytopenic patients. Basic studies of platelet function included: in vivo elution of /sup 51/Cr from labeled platelets induced by antibody; platelet age and adhesion to collagen; effect of serotonin on cyclic nucleotides of human platelets; response of cyclic nucleotides to stimulation in stored human platelets; and effect of ..cap alpha..-tocopherol on lipid peroxidation and platelet aggregation. Studies of cell-to-cell interaction using platelets as a model system took the form of: platelet interaction with tumor cells; and the effect of ..cap alpha..-tocopherol on platelet membrane fluidity. (PCS)

  18. 匹多莫德对儿童过敏性紫癜 Th17/Treg 细胞免疫的影响%Effects of pidotimod on Treg/Th17 cell immunity in children with Henoch-Schonlein purpura

    Institute of Scientific and Technical Information of China (English)

    刘彩霞; 罗卉丽; 姚远; 李兴朝

    2015-01-01

    Objective To observe and analyze the effects of pidotimod on the immunity of Treg/Th17 cells in the children with Henoch-Schonlein purpura (HSP).Methods 60 cases of HSP were selected and randomly divided into the pidotimod treatment group and the conventional treatment group,30 cases in each group.30 healthy children were selected as the control group.The con-ventional treatment group was given the routine therapy,while the pidotimod treatment group was added with oral pidotimod dis-persible tablets on the basis of the routine therapy.The ratios of Treg cell subsets and Th17 cell subsets,the plasma expression lev-els of interleukin-17 (IL-17),tumor necrosis factor-α(TNF-α),interferon-γ(IFN-γ)and interferon inducible protein-10 (IP-10) among 3 groups and the time for rash relief,remission rate of hematuria and proteinuria,recurrence rate within 1 month in HSP children were observed and compared.Results The ratios of Treg cell subsets and Th17 cell subsets after treatment had no statisti-cal difference between the pidotimod treatment group and the control group(P >0.05),while which had the statistical difference be-tween the conventional treatment group and the control group(P 0.05),but the IL-17 and IFN-γexpression levels in the conventional treatment group were still significantly higher than those in the control group.the remission rate of simple hematuria in the pidotimod treatment group was significantly higher than that in the conventional treatment group, while the recurrence rate within 1 month after treatment was significantly lower than that in the conventional treatment group.Con-clusion The application of pidotimod therapy in the treatment of children with HSP can significantly correct the imbalance of Treg cell subsets and Th17 cell subset,decrease the expression levels of the cytokines such as IL-17 and IFN-γ,suppress the autoimmune reaction so as to achieve the effects of significantly alleviating the impairment of renal function and reducing the

  19. Marked Improvement in Refractory TTP Directly after H. pylori Eradication Therapy.

    Science.gov (United States)

    Gringauz, Irina; Carmel-Neiderman, Narin Nard; Mangel, Tobin; Portnoy, Orith; Segal, Gad; Goren, Idan

    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 medical therapy; however the patient underwent complete resolution of her TTP following H. pylori eradication. We conclude that acquired TTP may be triggered by H. pylori infection and that treating the underlying infection may play a role in improving TTP's outcome in some patients, especially when disease is refractory to medical therapy. PMID:27127663

  20. Deterioration of autoimmune condition associated with repeated injection of dextranomer/hyaluronic acid copolymer: A case report

    Directory of Open Access Journals (Sweden)

    Kazuto Suda

    2016-01-01

    Full Text Available A 6-year-old girl underwent ureterocystoneostomy (UCN because of left flank pain due to delayed onset of ureteral stenosis one and a half years after endoscopic dextranomer/hyaluronic acid copolymer (Deflux injection for the treatment of vesicoureteral reflux (VUR. Histopathological examination indicated chronic inflammation with abundant eosinophils characteristic of a reaction to Deflux. Several autoimmune diseases developed during the treatment for ureteral stenosis. First, 2 weeks prior to the onset of left flank pain, she was diagnosed as having systemic lupus erythematosus. Finally, she died of pulmonary hemorrhage due to thrombotic thrombocytopenic purpura 4 months after UCN. The fatal outcome in this case was suspected to be caused by autoimmune syndrome induced by adjuvants, which in this case was the hyaluronic acid polymer injected into the ureteric orifice for the treatment of VUR.

  1. Autoimmune diseases in Adult Life after Childhood Cancer in Scandinavia (ALiCCS)

    DEFF Research Database (Denmark)

    Holmqvist, Anna Sällfors; Olsen, Jørgen H.; Mellemkjaer, Lene;

    2015-01-01

    all autoimmune diseases combined, corresponding to an AER of 67 per 100 000 person-years. The SHRRs were significantly increased for autoimmune haemolytic anaemia (16.3), Addison's disease (13.9), polyarteritis nodosa (5.8), chronic rheumatic heart disease (4.5), localised scleroderma (3......OBJECTIVES: The pattern of autoimmune diseases in childhood cancer survivors has not been investigated previously. We estimated the risk for an autoimmune disease after childhood cancer in a large, population-based setting with outcome measures from comprehensive, nationwide health registries.......6), idiopathic thrombocytopenic purpura (3.4), Hashimoto's thyroiditis (3.1), pernicious anaemia (2.7), sarcoidosis (2.2), Sjögren's syndrome (2.0) and insulin-dependent diabetes mellitus (1.6). The SHRRs for any autoimmune disease were significantly increased after leukaemia (SHRR 1.6), Hodgkin's lymphoma (1...

  2. [Shiga toxin and tetanus toxin as a potential biologic weapon].

    Science.gov (United States)

    Toczyska, Izabela; Płusa, Tadeusz

    2015-09-01

    Toxins produced by the bacteria are of particular interest as potential cargo combat possible for use in a terrorist attack or war. Shiga toxin is usually produced by shiga toxigenic strains of Escherichia coli (STEC - shigatoxigenic Escherichia coli). To infection occurs mostly after eating contaminated beef. Clinical syndromes associated with Shiga toxin diarrhea, hemorrhagic colitis, hemolytic uremic syndrome (HUS - hemolytic uremic syndrome) or thrombotic thrombocytopenic purpura. Treatment is symptomatic. In HUS, in which mortality during an epidemic reaches 20%, extending the kidney injury dialysis may be necessary. Exposure to tetanus toxin produced by Clostridium tetani, resulting in the most generalized tetanus, characterized by increased muscle tension and painful contractions of individual muscle groups. In the treatment beyond symptomatic behavior (among others spasticity medications, anticonvulsants, muscle relaxants) is used tetanus antitoxin and antibiotics (metronidazole choice). A common complication is acute respiratory failure - then it is necessary to implement mechanical ventilation. PMID:26449578

  3. Microangiopathic Hemolytic Anemia in 57-year-old woman with Borderline Serous Tumor of the Ovary:Real-Time Management of Common Pathways of Hemostatic Failure

    Directory of Open Access Journals (Sweden)

    Gloria Joan Morris

    2012-05-01

    Full Text Available We present a case of a 57-year-old woman who underwent surgery for the removal of an ovarian mass but subsequently experienced microangioathic hemolytic anemia post-operatively, associated with fevers, renal insufficiency, hypertension, and hemolysis. While her clinical situations was initially suspicious for thrombotic thrombocytopenic purpura (TTP, further sorting of clinical information led to other explanations of these findings, including a systemic inflammatory response. Multiple triggers of the coagulation system which can lead to a common pathway of hemostatic failure were considered, and specific criteria seen in disseminated intravascular coagulation (DIC, TTP, heparin-induced thrombocytopenia (HIT, catastrophic antiphospholipid anitbody syndrom (APS, all of which can seem to overlap when a physician is faced with distinguishing the diagnosis clinically. We propose a chronologic and strategic approach for the clinician to consider when approaching this diagnostic dilemma.

  4. [Diagnostic and therapeutic guidelines of thrombotic microangiopathies of the Spanish Apheresis Group].

    Science.gov (United States)

    Contreras, Enric; de la Rubia, Javier; Del Río-Garma, Julio; Díaz-Ricart, Maribel; García-Gala, José María; Lozano, Miguel

    2015-04-01

    Thrombotic microangiopathies (TMA) are disorders defined by the presence of a microangiopathic hemolytic anemia (with the characteristic hallmark of schistocytes in the peripheral blood smear), thrombocytopenia and organ malfunction of variable intensity. Thrombotic thrombocytopenic purpura and hemolytic uremic syndrome are the most important forms of TMA and, without the adequate treatment, they are associated with high morbimortality. In recent years, significant advances in the knowledge of the pathophysiology of TMA have occurred. Those advances have allowed us to move from a syndromic diagnosis with a similar treatment to all entities to the search of etiologic diagnosis which would lead to a specific treatment, finally leading to a better outcome of the patient. This document pretends to summarize the current status of knowledge of the pathophysiology of TMA and the therapeutic options available, and to offer a diagnostic and therapeutic practical tool to the professionals caring for the patients. PMID:25433791

  5. Single Incision Laparoscopic Splenectomy: Our First Experiences

    Directory of Open Access Journals (Sweden)

    Umut Barbaros

    2011-06-01

    Full Text Available Objective: Most laparoscopic surgeons have attempted to reduce incisional morbidity and improve cosmetic outcomes by using less and smaller trocars. Single incision laparoscopic splenectomy is a new laparoscopic procedure. Herein we would like to present our experiences.Material and Methods: Between January 2009 and June 2009, data of the 7 patients who underwent single incision laparoscopic splenectomy were evaluated retrospectively.Results: There were 7 patients (5 females and 2 males with a mean age of 29.9 years. The most common splenectomy indication was idiopathic thrombocytopenic purpura. Single incision laparoscopic splenectomy was performed successfully in 6 patients. In one patient the operation was converted to an open procedure.Conclusion: With surgeons experienced in minimally invasive surgery, single incision laparoscopic splenectomy could be performed successfully. However, in order to demonstrate the differneces between standard laparoscopic splenectomy and SILS splenetomy, prospective randomized comparative studies are required.

  6. Case report: passively acquired anti-D in a D+ pregnant patient.

    Science.gov (United States)

    Holub, M P; Kitchen, K; Mensinger, E

    1999-01-01

    A sample was submitted for serologic evaluation from a pregnant patient with immune thrombocytopenic purpura (ITP) for possible transfusion in the future because of a decreased platelet count. Anti-D and -E were identified in the patient's serum using several antibody identification techniques, and anti-D was recovered in an acid eluate prepared from the patient's red cells. It was discovered that WinRho had been administered to treat the ITP. This product has been licensed for treatment of nonsplenectomized D+ children and adults with ITP to increase the platelet count. Administration of anti-D to D+ individuals for treatment of ITP can cause a red cell anemia. PMID:15373521

  7. A more practical therapy for low platelets.

    Science.gov (United States)

    Torres, G

    1996-01-01

    The Food and Drug Administration (FDA) approved WinRho as a new treatment for ITP or thrombocytopenic purpura (low blood platelet count) in people with HIV. Current treatment options for ITP include AZT, corticosteroids, immunoglobulin therapy, and splenectomy. Clinical evaluation rates AZT as the best of the options. WinRho treatment will not work on Rh-negative patients or patients with no spleen. The largest WinRho study to date, involving 267 patients, shows a mean platelet increase of 76,000 cells with no adverse effect on CD4 counts or evidence of an acceleration of HIV disease progression. HIV-positive patients had a poorer platelet increase response than HIV-negative patients. PMID:11363379

  8. Safety profile of WinRho anti-D.

    Science.gov (United States)

    Hong, F; Ruiz, R; Price, H; Griffiths, A; Malinoski, F; Woloski, M

    1998-01-01

    WinRho anti-D is manufactured with multiple processes to minimize the risk of transmitting blood-borne diseases such as viruses. These safety features include donor selection, plasma testing, solvent-detergent viral inactivation, and nanofiltration. To date, there has not been any case of viral transmission in association with use of WinRho anti-D. Adverse drug reactions are infrequent and generally mild; the most common are headache, fever, and chills. Some degree of hemolysis is inevitable due to the mechanism of action of WinRho anti-D, but this is predictable and transient. A few cases of intravascular hemolysis have been reported; hypersensitivity reactions are very rare. WinRho anti-D has been shown in both clinical trials and postmarketing surveillance to be safe and effective in the treatment of idiopathic thrombocytopenic purpura (ITP) and in the prevention of Rh isoimmunization. PMID:9523744

  9. New treatment for low platelets.

    Science.gov (United States)

    Grodeck, B

    1995-01-01

    The Food and Drug Administration (FDA) recently approved WinRho SD, a new treatment for immune thrombocytopenic purpura (ITP). A clinical trial in children with acute ITP found that WinRho SD is as effective as IVIG or prednisone, but less expensive and dramatically easier to administer. WinRho SD is the first polyclonal antibody product shown to increase platelets in ITP patients. Platelets usually rise within one to two days and peak within seven to fourteen days after initial therapy. On average, platelet levels are maintained for approximately thirty days. Each year, 50,000 people nationally suffer from ITP as a complication of HIV infection, while another 18,000 manifest the disease as a primary condition. PMID:11362579

  10. [Basedow disease associated with Evans syndrome].

    Science.gov (United States)

    Kuroda, Hiroyuki; Kida, Masaya; Watanabe, Hideki; Matsunaga, Takuya; Niitsu, Yoshiro; Matsumoto, Masanori

    2005-10-01

    A 60-year-old woman was admitted to a hospital complaining of dizziness and general fatigue in October, 2004. Because of heart failure and severe anemia, she was referred to our hospital. Based on a positive direct Coombs test and an elevated level of platelet-associated IgG (PAIgG), the patient was diagnosed as having autoimmune hemolytic anemia (AIHA) associated with idiopathic thrombocytopenic purpura (ITP), i.e., Evans syndrome. Basedow disease was also diagnosed due to hyperthyroidism with an elevation of anti-thyroid stimulating hormone (TSH) receptor antibodies. Both the Evans syndrome and Basedow disease were considerably ameliorated with plasma exchange, corticosteroid and thiamazole therapy. Although Basedow disease is known to be associated with hematological disorders such as AIHA or ITP, the combination of Basedow disease and Evans syndrome is rare. We report here a case of Basedow disease associated with Evans syndrome. PMID:16440774

  11. [High-dose intravenous immunoglobulin treatment].

    Science.gov (United States)

    Taneichi, Hiromichi; Miyawaki, Toshio

    2011-03-01

    Intravenous immunoglobulin treatment was introduced as replacement therapy for patients with congenital agammaglobulinemia. For the last three decades, high-dose intravenous immunoglobulin (HD-IVIg) has been used for autoimmune diseases and systemic inflammatory diseases, such as idiopathic thrombocytopenic purpura, Kawasaki disease, myasthenia gravis and Guillain-Barré/syndrome. Although the immunomodulatory mechanisms of HD-IVIg remains unclear. Its use in many other diseases have been expected. Acute encephalitis/encephalopathy is a complex neurological syndrome associated with significant morbidity and mortality. The pathogenicity of brain dysfunction is still unknown. This review provides an overview and discussion of mechanisms that may be responsible for HD-IVIg effects in acute encephalitis/encephalopathy. PMID:21400848

  12. Management and outcomes for patients with TTP: analysis of 100 cases at a single institution.

    Science.gov (United States)

    Chaturvedi, Shruti; Carcioppolo, Desiree; Zhang, Li; McCrae, Keith R

    2013-07-01

    The advent of plasma exchange has led to a dramatic improvement in the survival of patients with thrombotic thrombocytopenic purpura (TTP), though approximately 10% of patients still die and a third suffer relapses. Clinical features that identify poor risk patients have not been clearly identified. We reviewed 100 patients who were treated for a first episode of TTP at the Cleveland Clinic between 2000 and 2012 to identify factors predictive of poor outcomes. On multivariate analysis, increasing age, especially age > 60 (RR: 7.08, 95% CI: 2.15-23.39, P = 0.002), severe neurological symptoms at presentation (RR: 18.37, 95% CI: I4.19-80.13, P  5% was an independent predictor of adverse renal outcomes (need for dialysis and progression to chronic kidney disease). These variables may be useful for risk stratification and identification of patients who could potentially benefit from early institution of adjunctive therapy. PMID:23605996

  13. Vincristine-induced unilateral ptosis in a child.

    Science.gov (United States)

    Gursel, Orhan; Sari, Erkan; Altun, Demet; Atay, A Avni; Akin, Ridvan

    2009-12-01

    Vincristine is a vinca alkaloid used in combination with other agents in the treatment of solid tumors, lymphoma, and leukemia, as well as for idiopathic thrombocytopenic purpura and autoimmune hemolytic anemia. A dose-limiting complication of vinca alkaloids is neurotoxicity. Vincristine is the oldest and also the most neurotoxic agent in this group. Described here is the case of a 4-year-old girl with unilateral palpebral ptosis. She has been diagnosed with precursor B-cell acute lymphoblastic leukemia. Ptosis was noted on the 45th day of therapy, and the last vincristine was administered on the 28th day of protocol 1. Vincristine-induced unilateral palpebral ptosis is a novel finding. Experience with this case suggests conservative treatment, with periodic examination, especially if ptosis is mild. PMID:19931172

  14. Application of polymerase chain reaction to detect rearrangement of immunoglobulin heavy chain genes in lymphoproliferative disease.

    Science.gov (United States)

    Khalil, S H; Siegrist, K; Akhtar, M

    1997-07-01

    As part of our routine work-up in the diagnosis of lymphoproliferative disease, we used a rapid polymerase chain reaction (PCR) assay to amplify the DNA fragments of the framework 3 (FR3) region of the immunoglobulin heavy (IgH) chain genes. The assay does not involve hybridization, nested priming, or sequencing of the amplified PCR product. It was performed on 66 specimens of B-cell lymphoproliferative disease, including acute lymphoblastic leukemia, chronic lymphocytic leukemia, multiple myeloma, hairy cell leukemia and follicular lymphoma. Twenty-six specimens of negative controls, including acute myeloid leukemia, chronic myeloid leukemia in myeloid transformation and idiopathic thrombocytopenic purpura, were also analyzed. The assay was performed with 77% sensitivity and 100% specificity. The standard IgH chain gene rearrangement by Southern blot analysis is reserved for the remaining negative cases if clinically indicated. PMID:17353588

  15. Identification of human megakaryocytes with rhodanile blue.

    Science.gov (United States)

    Kass, L

    1985-04-01

    Using the oxazine dye rhodanile blue, large typical megakaryocytes and small megakaryocytes (micromegakaryocytes) from the bone marrows of normal persons, and from patients with a variety of preleukemic disorders, acute lymphoblastic and nonlymphoblastic leukemia, chronic granulocytic leukemia, and idiopathic thrombocytopenic purpura as an example of nonmalignant but abnormal megakaryocytopoiesis, showed intense pink staining of the cytoplasm. This pink metachromasia was not obliterated by prior digestion with either diastase or ribonuclease, but was markedly diminished or obliterated by preincubation with hyaluronidase, suggesting that the stain may detect a high content of acid mucopolysaccharides in megakaryocytes. Since the stain is simple, direct, and reproducible, it may represent a useful addition to the cytochemistry of megakaryocytes and complement the more complex immunologic techniques available currently. PMID:2580502

  16. A RARE CASE OF GRISCELLI’S SYNDROME WITH REVIEW OF LITERATURE

    Directory of Open Access Journals (Sweden)

    Narayan Reddy

    2014-06-01

    Full Text Available INTRODUCTION: Griscelli syndrome is a rare autosomal recessive disorder characterized by partial albinism and immunodeficiency. The characteristic features include silver hair due to clumped melanosomes in hair shaft. Other features include hepatospleenomegaly, hepatitis, pancytopenia and immune abnormalities. CASE REPORT: A seven years old male child presented with partial albinic features and history of recurrent respiratory tract infection. The child had fever since 2months along with hepatospleenomegaly, pancytopenia and thrombocytopenic purpura. These rare clinical manifestations were diagnosed as GS which was confirmed by bone marrow biopsy and light microscopy of hair shaft. The mean patient age of survival is 5years. As early diagnosis can improve the outcome in such cases, a finding of partial albinism should alert the diagnosis of Griscelli’s syndrome. CONCLUSION: We report a rare case of GS with characteristic manifestation surviving beyond the mean age of reported survival.

  17. Eosinophilic gastroenteritis associated with systemic lupus erythematosus.

    Science.gov (United States)

    Barbie, David A; Mangi, Abeel A; Lauwers, Gregory Y

    2004-01-01

    Eosinophilic gastroenteritis is an uncommon disease with an obscure etiology, although associations with allergy, the idiopathic hypereosinophilic syndrome, and connective tissue disease have been reported. We present the case of a 37-year-old woman with a history of idiopathic thrombocytopenic purpura who presented with refractory nausea, vomiting, and abdominal pain. Imaging studies were significant for bowel wall thickening and ascites, while laboratory studies revealed a positive antinuclear antibody (ANA), a positive anti-double stranded (DS) DNA antibody, low complement, and proteinuria. Exploratory laparotomy with gastric and small bowel biopsies established the diagnosis of eosinophilic gastroenteritis. In addition, the patient met clinical criteria for the diagnosis of systemic lupus erythematosus. Previous studies have described eosinophilic gastroenteritis in patients with scleroderma, polymyositis, or dermatomyositis. This is the first report to our knowledge of an individual with eosinophilic gastroenteritis and systemic lupus erythematosus. PMID:15492606

  18. Autosomal Dominant Hereditary Macrothrombocytopenia in an Iranian Family

    Directory of Open Access Journals (Sweden)

    M Isadiar

    2006-06-01

    Full Text Available Objective: Thrombocytopenia is the most common hemostatic disease of the newborn. Inherited giant platelet syndromes are a heterogeneous group of rare bleeding disorders. In this paper we describe here a female neonate with autosomal dominant hereditary macrothrombocytopenia. Case report: A female neonate was referred to our center due to mucosal hemorrhage (nasal and gastrointestinal bleeding. Her mother’s platelet count was normal. However her father, paternal uncle and two paternal aunts also had severe thrombocytopenia and all of them underwent splenectomy for idiopathic thrombocytopenic purpura (ITP. Considering all clinical and laboratory findings, autosomal dominant hereditary macrothrombocytopenia was the best diagnosis. Conclusion: It is important to differentiate between congenital and acquired thrombocytopenia to avoid unneeded and potentially harmful therapy. Treatment is not usually necessary, however some patients with hereditary thrombocytopenia may benefit from bone marrow transplantation.

  19. Single-incision laparoscopic splenectomy with innovative gastric traction suture

    Directory of Open Access Journals (Sweden)

    Srikanth G

    2011-01-01

    Full Text Available Laparoscopic splenectomy is now the gold standard for patients with idiopathic thrombocytopenic purpura (ITP undergoing splenectomy. There are a few reports in literature on single-incision laparoscopic (SIL splenectomy. Herein, we describe a patient undergoing SIL splenectomy for ITP without the use of a disposable port device. We report a 20-year-old female patient with steroid-refractory ITP having a platelet count of 14,000/cmm who underwent a SIL splenectomy. Dissection was facilitated by the use of a single articulating grasper and a gastric traction suture and splenic vessels were secured at the hilum with an endo-GIA stapler. She made an uneventful postoperative recovery and was discharged on the second postoperative day. She is doing well with no visible scar at 8-month follow-up.

  20. Pulmonary Limited MPO-ANCA Microscopic Polyangiitis and Idiopathic Lung Fibrosis in a Patient with a Diagnosis of IgA Nephropathy

    Directory of Open Access Journals (Sweden)

    Alwin Tilanus

    2015-01-01

    Full Text Available We present a case of a male patient with chronic renal insufficiency, due to crescentic glomerulonephritis with IgA deposits, who successively developed (idiopathic thrombocytopenic purpura (ITP and MPO-ANCA microscopic polyangiitis (MPA with pulmonary fibrosis. The patient presented with cough, weight loss, and dyspnea on exertion. CT imaging and pulmonary function tests were compatible with interstitial pneumonitis with pulmonary fibrosis. Laboratory results showed high MPO-ANCA titers; the urinary sediment was bland. The patient was treated successfully with cyclophosphamide and methyl-prednisolone. This unique case illustrates the diagnostic and therapeutic challenges of an unusual presentation of microscopic polyangiitis presenting first as isolated kidney disease with recurrence in the form of pneumonitis without renal involvement, in association with renal IgA deposits and ITP as coexisting autoimmune conditions.

  1. [Lupus nephropathy in childhood and familial lupus. Genetic study of a family].

    Science.gov (United States)

    Gómez Campdera, F J; Yebra, M; Vicario, J L; Rodríguez, M; Rengel, M; Manzano, L; Martín Villa, J M; Gutiérrez, C

    1989-04-15

    We report a case of a 14 1/2-year-old boy who was diagnosed of systemic lupus erythematosus in the background of an acute nephritic syndrome, 3 1/2 years after being diagnosed of idiopathic thrombocytopenic purpura. The familial history suggested the presence of other cases of SLE, which were proven with relevant clinical and laboratory studies. A genetic study for disease markers was carried out and a correlation was found with haplotypes HLA A25, B18, BW6, DRX, and DQW; C2 deficiency was ruled out. We conclude that it is of paramount importance to rule out the existence of familial SLE in front of infantile SLE, particularly in boys, and we emphasize the necessity of keeping on further searching for genetic markers of the disease. PMID:2755225

  2. Marked Improvement in Refractory TTP Directly after H. pylori Eradication Therapy

    Science.gov (United States)

    Gringauz, Irina; Carmel-Neiderman, Narin Nard; Mangel, Tobin; Portnoy, Orith; Segal, Gad; Goren, Idan

    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 medical therapy; however the patient underwent complete resolution of her TTP following H. pylori eradication. We conclude that acquired TTP may be triggered by H. pylori infection and that treating the underlying infection may play a role in improving TTP's outcome in some patients, especially when disease is refractory to medical therapy. PMID:27127663

  3. Evidence of platelet activation in multiple sclerosis

    Directory of Open Access Journals (Sweden)

    Alexander J Steven

    2008-06-01

    Full Text Available Abstract Objective A fatality in one multiple sclerosis (MS patient due to acute idiopathic thrombocytopenic purpura (ITP and a near fatality in another stimulated our interest in platelet function abnormalities in MS. Previously, we presented evidence of platelet activation in a small cohort of treatment-naive MS patients. Methods In this report, 92 normal controls and 33 stable, untreated MS patients were studied. Platelet counts, measures of platelet activation [plasma platelet microparticles (PMP, P-selectin expression (CD62p, circulating platelet microaggragtes (PAg], as well as platelet-associated IgG/IgM, were carried out. In addition, plasma protein S activity was measured. Results Compared to controls, PMP were significantly elevated in MS (p Conclusion Platelets are significantly activated in MS patients. The mechanisms underlying this activation and its significance to MS are unknown. Additional study of platelet activation and function in MS patients is warranted.

  4. False Positive B-Cells Crossmatch after Prior Rituximab Exposure of the Kidney Donor

    Directory of Open Access Journals (Sweden)

    Judith Desoutter

    2016-01-01

    Full Text Available Crossmatching is essential prior to kidney transplantation to confirm compatibility between the donor and the recipient, particularly to prevent acute antibody-mediated rejection. An unexpected positive crossmatch may be obtained in recipients with an autoimmune disease or preexisting antibodies not detected by single-antigen bead array due to complement interference or who have been previously treated by desensitization protocols such as rituximab, antithymocyte globulin, or intravenous immunoglobulins. We report donor and recipient investigations that revealed unexpected positive B-cells crossmatch, probably due to donor cells, as the donor had received rituximab therapy shortly before organ harvesting, in a context of severe idiopathic thrombocytopenic purpura. We consequently detected unexpected Class II IgG complement-dependent cytotoxicity for all sera tested. Other laboratory investigations failed to elucidate the reasons for this recipient-related positivity.

  5. The ADAMTS(L) family and human genetic disorders.

    Science.gov (United States)

    Le Goff, Carine; Cormier-Daire, Valérie

    2011-10-15

    ADAMTS designates a family of 19 secreted enzymes, whose the first member ADAMTS1 was described in 1997. The ADAMTS family has a role in extracellular matrix degradation and turn over and has previously been involved in various human biological processes, including connective tissue structure, cancer, coagulation, arthritis, angiogenesis and cell migration. More recently, the ADAMTS(L) family has been described, sharing the same ancillary domain but distinct by the absence of any enzyme activity. Mutations in ADAMTS13, ADAMTS2, ADAMTS10, ADAMTS17, ADAMTSL2 and ADAMTSL4 have been identified in distinct human genetic disorders ranging from thrombotic thrombocytopenic purpura to acromelic dysplasia. The aim of our review was to emphasize the role of this family in the extracellular matrix based on human phenotypes so far identified in relation with ADAMTS(L) mutations. PMID:21880666

  6. Screening for neonatal hyperbilirubinaemia and ABO alloimmunization at the time of testing for phenylketonuria and congenital hypothyreosis.

    Science.gov (United States)

    Meberg, A; Johansen, K B

    1998-12-01

    In a population-based study including 2463 infants, serum bilirubin measurements were added to the neonatal screening programme for phenylketonuria and congenital hypothyreosis. This screening programme detected 11/17 (65%) of infants with serum bilirubin levels >350 micromol 1(-1), of whom 7 (3 per 1000) were readmitted from home (6 treated with phototherapy). A total of 139 infants (5.6%) received phototherapy. Maternal blood type O occurred significantly more often in term infants treated (30/54; 55.6%) compared with preterm infants treated (32/85; 37.6%) and with blood type O occurrence in the total population of mothers (906/2426; 37.3%) (p constellations mother O/infant A or B showed a sensitivity of 64%, specificity 65%, positive predictive value 12% and a negative predictive value of 96% for the requirement of phototherapy for the whole material. Exchange transfusion was not required in any of the infants. No infant developed bilirubin encephalopathy (kemicterus). Adding bilirubin to a neonatal screening programme detects some cases with unexpectedly high bilirubin levels in need of intervention. Routine ABO blood typing of pregnant women, ABO cord blood typing and Coombs' test in infants of mothers with blood type O cannot be recommended because of low positive predictive value for the requirement of intervention (phototherapy) by these tests. PMID:9894828

  7. Constructing a population-based research database from routine maternal screening records: a resource for studying alloimmunization in pregnant women.

    Directory of Open Access Journals (Sweden)

    Brian K Lee

    Full Text Available BACKGROUND: Although screening for maternal red blood cell antibodies during pregnancy is a standard procedure, the prevalence and clinical consequences of non-anti-D immunization are poorly understood. The objective was to create a national database of maternal antibody screening results that can be linked with population health registers to create a research resource for investigating these issues. STUDY DESIGN AND METHODS: Each birth in the Swedish Medical Birth Register was uniquely identified and linked to the text stored in routine maternal antibody screening records in the time window from 9 months prior to 2 weeks after the delivery date. These text records were subjected to a computerized search for specific antibodies using regular expressions. To illustrate the research potential of the resulting database, selected antibody prevalence rates are presented as tables and figures, and the complete data (from more than 60 specific antibodies presented as online moving graphical displays. RESULTS: More than one million (1,191,761 births with valid screening information from 1982-2002 constitute the study population. Computerized coverage of screening increased steadily over time and varied by region as electronic records were adopted. To ensure data quality, we restricted analysis to birth records in areas and years with a sustained coverage of at least 80%, representing 920,903 births from 572,626 mothers in 17 of the 24 counties in Sweden. During the study period, non-anti-D and anti-D antibodies occurred in 76.8/10,000 and 14.1/10,000 pregnancies respectively, with marked differences between specific antibodies over time. CONCLUSION: This work demonstrates the feasibility of creating a nationally representative research database from the routine maternal antibody screening records from an extended calendar period. By linkage with population registers of maternal and child health, such data are a valuable resource for addressing important clinical questions, such as the etiological significance of non-anti-D antibodies.

  8. Maternal alloimmunization as a risk factor of haemolytic disease of the foetus and newborn in Owerri metropolis, Nigeria

    Directory of Open Access Journals (Sweden)

    A.A.O. Chinwe

    2013-10-01

    Full Text Available Blood group incompatibility between mother and her foetus which usually arises as a result of heterospecific pregnancy causes haemolytic disease of the foetus and newborn (HDN. Five hundred (500 pregnant women attending antenatal clinic at Federal Medical Centre (FMC, Owerri were recruited for this research work. They were aged between 17 and 40 years. All subjects gave informed consent to participate in the study. They were screened for ABO blood groups RhD status and immune alloantibodies. Anti-A and anti-B from group O subjects compared with anti-A from group B women and anti-B from group A women among the trimester. The rate of RhD negativity (5.4% was significantly lower than the RhD positively (94.6% (P<0.005. Haemolysin tests were positive in 110 (22.0% cases and negative in 390 (78.0% cases. Group O women produced more alpha and / or beta haemolysins than either A or B women (P<0.005. Anti-A titres were higher in the trimesters than anti-B. Indirect antiglobulin test (IAT was positive in 12(80.0% cases among ABO group and positive in 3(20.0% cases among RhD group. There was a higher prevalence of ABO HDN than RhD HDN in the ratio of 3:1 respectively. This study illustrates the presence of immune alloantibodies in the sera of allominiunized pregnant women and the rise in the antibody titres has helped to identify pregnancies at risk of foetal and neonatal HDN. Anti-D immunoprophylaxis has made HDN caused by sensitization to the D-antigen a preventable disease and prenatal deaths from allominization by immunoprophylaxis has been primarily responsible for the dramatic reduction in the diseases, although changes in family size and the quality of prenatal care have also contributed.

  9. [Severe hemolytic disease of the newborn as a result of late and undiagnosed alloimmunization--case report].

    Science.gov (United States)

    Drozdowska-Szymczak, Agnieszka; Czaplińska, Natalia; Borek-Dziecioł, Beata; Kociszewska-Najman, Bozena; Bartkowiak, Robert; Wielgoś, Mirosław

    2014-03-01

    We report a case of a hemolytic disease in a newborn from the first pregnancy due to anti-D antibodies. The maternal blood group was A Rhesus negative. She had an antibody screening test twice during the pregnancy (in the second trimester) and it was negative. The pregnancy was uneventful, without any invasive procedures and bleeding. The infant was born at 39 weeks of gestation in good overall condition. After the delivery the blood group of the neonate was indicated - A Rhesus positive, BOC positive. Anti-D antibodies were detected in maternal blood. Neonatal blood tests revealed severe anemia (hemoglobin level: 6.0g/dl, hematocrit: 22.2%, erythrocytes: 2.01T/L). During the first day of neonatal life, the newborn received two transfusions of red blood cells. Bilirubin level and rate of rise were not recommendation enough for exchange transfusion. The newborn was treated with continuous phototherapy since the delivery The perinatal period was complicated with intrauterine infection and respiratory failure. Hematopoietic vitamins and iron supplementation was initiated in the second week of neonatal life due to persistent anemia. The child remained under medical care of a hematologic clinic and received human recombinant erythropoietin treatment. PMID:24783436

  10. Maternal alloimmunization as a risk factor of haemolytic disease of the foetus and newborn in Owerri metropolis, Nigeria

    OpenAIRE

    A.A.O. Chinwe; J. Nnodim; N. Constance; O. Hope

    2013-01-01

    Blood group incompatibility between mother and her foetus which usually arises as a result of heterospecific pregnancy causes haemolytic disease of the foetus and newborn (HDN). Five hundred (500) pregnant women attending antenatal clinic at Federal Medical Centre (FMC), Owerri were recruited for this research work. They were aged between 17 and 40 years. All subjects gave informed consent to participate in the study. They were screened for ABO blood groups RhD status and immune alloantibodie...

  11. Cumulative experience with a simplified solid-phase radioimmunoassay for the detection of bound antiplatelet IgG, serum auto-, allo-, and drug-dependent antibodies

    International Nuclear Information System (INIS)

    A simplified, sensitive, solid-phase radioimmunoassay employing 125I-staphylococcal protein A has been developed that is capable of detecting bound antiplatelet IgG as well as serum auto-, allo-, and drug-dependent antiplatelet antibodies. The simplified assay employs a ratio of test over control platelet counts per minute (cpm) for detection of positive results. All reagents are commercially available. The assay can be performed with as little as 106 washed platelets (10 μl of whole blood) that have been stored for as long as 8 wk at 40C in microtiter plates. The assay time, employing stored platelets, is 4 hr. Bound platelet IgG is positive in 93% of 46 thrombocytopenic patients with autoimmune disease and correlates inversely with their platelet count, r = -0.65, p 1:64); 7 of 12 multiply transfused patients who were refractory to platelet transfusion (2 had titers of >1:256 and 1:32); 5 of 19 patients with autoimmune thrombocytopenic purpura (2 had titers of 1:64 and 1:32); and 10 of 14 patients with clinical histories of drug-dependent antiplatelet antibody (2 had titers of 1:1280 for quinidine and 1:384 for phenazopyridine)

  12. Dose escalation study of an anti-thrombocytopenic agent in patients with chemotherapy induced thrombocytopenia

    International Nuclear Information System (INIS)

    Preclinical studies demonstrated that small chain RNA fragments accelerate the recovery of platelets numbers in animals exposed to high doses of chemotherapeutic drugs. There is anecdotal data supporting the same application in humans. The Phase I clinical trial described here was designed to investigate the relationship between the administration of small chain RNA fragments and the recovery in platelets following Chemotherapy-Induced Thrombocytopenia (CIT). Cancer patients with solid tumors that experienced post chemotherapy thrombocytopenia with a nadir of < = 80,000 platelets/ml were eligible for this clinical trial. There were no exclusions based on ECOG status, tumor type, tumor burden or chemotherapeutic agents. Patients received a unique preparation of RNA derived from either E. coli or yeast. Ten patients per group received 20, 40, or 60 mg as a starting dose. Subjects self-administered RNA fragments sublingually on an every other day schedule while undergoing chemotherapy. The dose was escalated in 20 mg increments to a maximum dose of 80 mg if the nadir was < 80,000 platelets/ml at the start of the next cycle. Subjects were treated for three cycles of chemotherapy with the maximum effective dose of RNA fragments. Subjects continued on planned chemotherapy as indicated by tumor burden without RNA fragment support after the third cycle. Subjects kept a diary indicating RNA fragment and magnesium administration, and any experienced side effects. Patients receiving E. coli RNA fragments demonstrated a more rapid recovery in platelet count and higher nadir platelet count. None of the patients receiving the E. coli RNA fragments required a chemotherapy dose reduction due to thrombocytopenia. The optimal dose for minimizing CIT was 80 mg. Conversely, subjects receiving yeast RNA fragments with dose escalation to 80 mg required a chemotherapy dose reduction per American Society of Clinical Oncology guidelines for grade 3 and 4 thrombocytopenia. Patients receiving myelosuppressive chemotherapy experienced an improvement in the platelet nadir and shorter recovery time when receiving concurrent E coli RNA fragments, when compared to patients who received yeast RNA fragments. These data indicate that 60 and 80 mg doses of E. coli RNA accelerated platelet recovery. Further clinical investigations are planned to quantify the clinical benefits of the E. coli RNA at the 80 mg dose in patients with chemotherapy induced thrombocytopenia. Clinical Trials.gov Identifier: NCT01163110

  13. Оцінка ефективності фармакологічної корекції ендотеліальної дисфункції у дітей з пурпурою Шенлейн-Геноха в залежності від виду лікування = Evaluation of pharmacological correction of endothelial dysfunction in children with Schonlein-Henoch purpura depending on the type treatment

    OpenAIRE

    Dudnyk, V. M.; Korol, T.G.

    2015-01-01

    Dudnyk V. M., Korol T.G. Оцінка ефективності фармакологічної корекції ендотеліальної дисфункції у дітей з пурпурою Шенлейн-Геноха в залежності від виду лікування = Evaluation of pharmacological correction of endothelial dysfunction in children with Schonlein-Henoch purpura depending on the type treatment. Journal of Education, Health and Sport. 2015;5(3):189-198. ISSN 2391-8306. DOI: 10.5281/zenodo.16359 http://ojs.ukw.edu.pl/index.php/johs/article/view/2015%3B5%283%29%3A189-198 https...

  14. CRISPR/Cas9-mediated conversion of human platelet alloantigen allotypes.

    Science.gov (United States)

    Zhang, Nanyan; Zhi, Huiying; Curtis, Brian R; Rao, Sridhar; Jobaliya, Chintan; Poncz, Mortimer; French, Deborah L; Newman, Peter J

    2016-02-11

    Human platelet alloantigens (HPAs) reside on functionally important platelet membrane glycoproteins and are caused by single nucleotide polymorphisms in the genes that encode them. Antibodies that form against HPAs are responsible for several clinically important alloimmune bleeding disorders, including fetal and neonatal alloimmune thrombocytopenia and posttransfusion purpura. The HPA-1a/HPA-1b alloantigen system, also known as the Pl(A1)/Pl(A2) polymorphism, is the most frequently implicated HPA among whites, and a single Leu33Pro amino acid polymorphism within the integrin β3 subunit is responsible for generating the HPA-1a/HPA-1b alloantigenic epitopes. HPA-1b/b platelets, like those bearing other low-frequency platelet-specific alloantigens, are relatively rare in the population and difficult to obtain for purposes of transfusion therapy and diagnostic testing. We used CRISPR/Cas9 (clustered regularly interspaced short palindromic repeats/CRISPR associated protein 9) gene-editing technology to transform Leu33 (+) megakaryocytelike DAMI cells and induced pluripotent stem cells (iPSCs) to the Pro33 allotype. CD41(+) megakaryocyte progenitors derived from these cells expressed the HPA-1b (Pl(A2)) alloantigenic epitope, as reported by diagnostic NciI restriction enzyme digestion, DNA sequencing, and western blot analysis using HPA-1b-specific human maternal alloantisera. Application of CRISPR/Cas9 technology to genetically edit this and other clinically-important HPAs holds great potential for production of designer platelets for diagnostic, investigative, and, ultimately, therapeutic use. PMID:26634302

  15. Perplexing purpura in two females: Rare case of autoerythrocyte sensitization syndrome

    Directory of Open Access Journals (Sweden)

    Ram R Tainwala

    2013-01-01

    Full Text Available Autoerythrocyte sensitization syndrome is a psychologically induced painful bruising condition. Two female, 19 and 30-year-old presented with recurrent episodes of painful ecchymotic bruising over accessible areas of body. In the younger female, episodes were since 3 years and were precipitated by stress and trivial trauma. The elder female presented with similar lesions since 3 months which were spontaneous in presentation. There were no obvious psychiatric manifestations in either. Clinically, ecchymotic changes in various stages of development were seen. Routine hemogram and coagulation profile were normal. Histopathology showed extravasated erythrocytes, perivascular neutrophils and fibrinoid deposition. Intradermal injection of autologous whole blood produced a painful ecchymotic reaction after 2 h similar to the presenting lesions. Psychiatric evaluation revealed mild mixed depression - anxiety disorder in the younger female while the latter revealed no abnormalities. The diagnosis of autoerythrocyte sensitization syndrome was made based on clinical history and findings, positive autoerythrocyte sensitization test, psychiatric evaluation and absence of any other clinical or laboratory pathology.

  16. Perplexing purpura in two females: Rare case of autoerythrocyte sensitization syndrome.

    Science.gov (United States)

    Tainwala, Ram R; Phiske, Meghna; Raghuwanshi, Abhijith; Mathapati, Sukesh; Manjare, Ashwini K; Jerajani, Hemangi R

    2013-10-01

    Autoerythrocyte sensitization syndrome is a psychologically induced painful bruising condition. Two female, 19 and 30-year-old presented with recurrent episodes of painful ecchymotic bruising over accessible areas of body. In the younger female, episodes were since 3 years and were precipitated by stress and trivial trauma. The elder female presented with similar lesions since 3 months which were spontaneous in presentation. There were no obvious psychiatric manifestations in either. Clinically, ecchymotic changes in various stages of development were seen. Routine hemogram and coagulation profile were normal. Histopathology showed extravasated erythrocytes, perivascular neutrophils and fibrinoid deposition. Intradermal injection of autologous whole blood produced a painful ecchymotic reaction after 2 h similar to the presenting lesions. Psychiatric evaluation revealed mild mixed depression - anxiety disorder in the younger female while the latter revealed no abnormalities. The diagnosis of autoerythrocyte sensitization syndrome was made based on clinical history and findings, positive autoerythrocyte sensitization test, psychiatric evaluation and absence of any other clinical or laboratory pathology. PMID:24350012

  17. 小儿血液系统疾病人细小病毒B 19感染的研究%The investigation of HPV B19 infection on children with blood system diseases

    Institute of Scientific and Technical Information of China (English)

    许东亮; 张国成; 聂晓晶; 孙新; 吴海霞; 徐晓莉

    2014-01-01

    目的:探讨我国西北地区血液系统疾病患儿人细小病毒B19(H PV B19)感染情况及两者的相关性。方法:对特发性血小板减少性紫癜、再生障碍性贫血和白血病患儿的外周血分别采用PCR方法检测HPV B19-DNA和ELISA方法检测HPV B19-IgG。结果:特发性血小板减少性紫癜患儿HPV B19-IgG阳性率为43.08%(28/65),白血病患儿 HPV B19-IgG阳性率为41.07%(46/112),病例组较对照组比较有极显著性差异(P<0.01);再生障碍性贫血患儿 HPV B19-IgG阳性率为21.43%(6/28),病例组较对照组比较有显著性差异( P<0.05)。65例特发性血小板减少性紫癜患儿HPV B19-DNA阳性率为40.00%(26/65);28例再生障碍性贫血患儿 HPV B19-DNA阳性率为21.43%(6/28);112例白血病患儿 HPV B19-DNA 阳性率为27.68%(31/112);对照组 HPV B19-DNA检测均为阴性。结论:HPV B19感染与我国西北地区部分血液系统疾病患儿发病相关。%Objective:To investigate the relationship between human parvovirus B 19 (HPV B19 ) infection and children who had blood system disease .Methods :PCR and ELISA methods were used to detecte HPV B19-DNA and HPV B19-IgG in child who had idiopathic thrombocytopenic purpura ,aplastic anemia or leukemia .Results :The positive rate of HPV B19-IgG was 43 .08% in patient with idiopathic thrombocytopenic purpura ,the positive rate of HPV B19-IgG was 41 .07% in patient with leukemia ,and the positive rate of HPV B19-IgG was 21 .43% in patient with aplastic anemia .There were statistical differences between the experiment groups and the control group .The positive rate of HPV B19-DNA was 40 .00% in patient with idiopathic thrombocytopenic purpura ,the positive rate of HPV B19-DNA was 27 .68% in patient with leukemia ,and the positive rate of HPV B19-DNA was 21 .43% in patient with aplastic anemia .Conclusion :HPV B19 infection have relationship with some children

  18. Antibody-mediated immune suppression of erythrocyte alloimmunization can occur independently from red cell clearance or epitope masking in a murine model.

    Science.gov (United States)

    Yu, Honghui; Stowell, Sean R; Bernardo, Lidice; Hendrickson, Jeanne E; Zimring, James C; Amash, Alaa; Uchikawa, Makoto; Lazarus, Alan H

    2014-09-15

    Anti-D can prevent immunization to the RhD Ag on RBCs, a phenomenon commonly termed Ab-mediated immune suppression (AMIS). The most accepted theory to explain this effect has been the rapid clearance of RBCs. In mouse models using SRBC, these xenogeneic cells are always rapidly cleared even without Ab, and involvement of epitope masking of the SRBC Ags by the AMIS-inducing Ab (anti-SRBC) has been suggested. To address these hypotheses, we immunized mice with murine transgenic RBCs expressing the HOD Ag (hen egg lysozyme [HEL], in sequence with ovalbumin, and the human Duffy transmembrane protein) in the presence of polyclonal Abs or mAbs to the HOD molecule. The isotype, specificity, and ability to induce AMIS of these Abs were compared with accelerated clearance as well as steric hindrance of the HOD Ag. Mice made IgM and IgG reactive with the HEL portion of the molecule only. All six of the mAbs could inhibit the response. The HEL-specific Abs (4B7, IgG1; GD7, IgG2b; 2F4, IgG1) did not accelerate clearance of the HOD-RBCs and displayed partial epitope masking. The Duffy-specific Abs (MIMA 29, IgG2a; CBC-512, IgG1; K6, IgG1) all caused rapid clearance of HOD RBCs without steric hindrance. To our knowledge, this is the first demonstration of AMIS to erythrocytes in an all-murine model and shows that AMIS can occur in the absence of RBC clearance or epitope masking. The AMIS effect was also independent of IgG isotype and epitope specificity of the AMIS-inducing Ab. PMID:25122924

  19. Factors associated with persistence of red blood cell antibodies in woman after pregnancies complicated by fetal alloimmune haemolytic disease treated with intrauterine transfusions.

    Science.gov (United States)

    Verduin, Esther P; Brand, Anneke; van de Watering, Leo M G; Claas, Frans H J; Oepkes, Dick; Lopriore, Enrico; Doxiadis, Ilias I N; Schonewille, Henk

    2015-02-01

    Red blood cell (RBC) antibodies can persist for decades or decrease quickly to undetectable levels. Antibody persistence has not been systematically studied. Women whose children are treated with intrauterine transfusions (IUT) for haemolytic disease of the fetus (HDFN) often produce additional antibodies, which can be evoked by the intrauterine transfusion or by fetomaternal haemorrhage during the procedure. Factors associated with persistence of both the antibodies responsible for HDFN and additional antibodies were studied in 260 women whose children were treated with IUT between 1988 and 2008. They possessed 499 (205 anti-D and 294 non-D) antibodies after the last IUT. After a median follow-up of 8·7 years, all 260 antibodies primarily responsible for HDFN had persisted. Additional antibodies directed against antigens of the children persisted in 70·6%, and in 32·3% if they were not child-specific (P < 0·001). Antibodies induced by irradiated IUT persisted in only 7·1%. Multivariate analyses showed that non-HDFN antibody persistence was dependent on the antibody titre and specificity. In conclusion, persistence of antibodies mainly depends on antibody strength and specificity. Difference between fetal or non-fetal immunogens suggests maintenance of antigenic stimulation possibly by long-term fetomaternal chimerism. PMID:25244566

  20. 25-OH-Vitamin D deficiency and cellular alloimmunity as measured by panel of reactive T cell testing in dialysis patients

    OpenAIRE

    Sawinski, Deirdre; Uribarri, Jaime; Peace, Denise; Yao, Tina; Wauhop, Praeophayom; Trzcinka, Paulina; Ostrow, Katya; Poggio, Emilio D.; Heeger, Peter S.

    2010-01-01

    Primed anti-donor alloreactive T cells are detrimental to transplant outcome, but factors that impact the strength of this immune response prior to transplantation are unknown. We tested peripheral blood mononuclear cells from dialysis patients, against panels of allogeneic, primary B cell lines in a newly standardized IFNγ ELISPOT panel of reactive T cell (PRT) assays. Results were correlated with known alloantibody sensitizing events and other clinical parameters. As 25-OH-vitamin D deficie...

  1. Cumulative experience with a simplified solid-phase radioimmunoassay for the detection of bound antiplatelet IgG, serum auto-, allo-, and drug-dependent antibodies

    Energy Technology Data Exchange (ETDEWEB)

    Faig, D.; Karpatkin, S.

    1982-10-01

    A simplified, sensitive, solid-phase radioimmunoassay employing /sup 125/I-staphylococcal protein A has been developed that is capable of detecting bound antiplatelet IgG as well as serum auto-, allo-, and drug-dependent antiplatelet antibodies. The simplified assay employs a ratio of test over control platelet counts per minute (cpm) for detection of positive results. All reagents are commercially available. The assay can be performed with as little as 10/sup 6/ washed platelets (10 ..mu..l of whole blood) that have been stored for as long as 8 wk at 4/sup 0/C in microtiter plates. The assay time, employing stored platelets, is 4 hr. Bound platelet IgG is positive in 93% of 46 thrombocytopenic patients with autoimmune disease and correlates inversely with their platelet count, r = -0.65, p < 0.001. The ability of this assay to detect serum antibody was studied with a rabbit anti-human platelet antibody capable of giving optimal immunoprecipitation with solubilized platelet membranes at a titer of 1:10. The present assay increases the sensitivity of antibody detection 256-fold to a titer of 1:2560. Human serum antiplatelet membrane antibody was positive in 2 of 2 patients with anti-PLA-1 antibody (titers of 1:256 and >1:64); 7 of 12 multiply transfused patients who were refractory to platelet transfusion (2 had titers of >1:256 and 1:32); 5 of 19 patients with autoimmune thrombocytopenic purpura (2 had titers of 1:64 and 1:32); and 10 of 14 patients with clinical histories of drug-dependent antiplatelet antibody (2 had titers of 1:1280 for quinidine and 1:384 for phenazopyridine).

  2. The significance of platelet-associated immunoglobulin G in non-thrombocytopenic patients with systemic lupus erythematosus

    DEFF Research Database (Denmark)

    Sørensen, P G; Mickley, H; Fristed, P; Diederichsen, H

    1985-01-01

    The possible pathogenetic significance of platelet-associated immunoglobulin G in systemic lupus erythematosus (SLE) has been studied, using a semiquantitative immunofluorescence technique. The study included 22 patients suffering from SLE during the period 1973-81. Thirteen patients had various...

  3. Vitamin B12 and Vitamin D Deficiencies: An Unusual Cause of Fever, Severe Hemolytic Anemia and Thrombocytopenia

    Science.gov (United States)

    Mishra, Vikas A.; Harbada, Rishit; Sharma, Akhilesh

    2015-01-01

    The array of diagnostic workup for pyrexia of unknown origin (PUO) generally revolves in searching for infections, inflammatory/autoimmune, and endocrine etiologies. A differential diagnosis of fever, hemolytic anemia, and thrombocytopenia can have etiologies varying from infections like malaria, dengue, cytomegalovirus, Ebstein barr virus, Parvovirus, infective endocarditis, to autoimmune disorder (systemic lupus erythromatosis), vasculitis, hemolytic uremic syndrome, thrombotic thrombocytopenic purpura (TTP), autoimmune hemolytic anemia/Evan's syndrome, paroxysmal nocturnal hemoglobinuri (PNH), or drugs. Nutritional deficiencies (especially vitamin B12 deficiency) as a cause of fever, hemolytic anemia, and thrombocytopenia are very rare and therefore rarely thought of. Severe vitamin B12 deficiency may cause fever and if accompanied by concurrent hyper-homocysteinemia and hypophosphatemia can sometimes lead to severe hemolysis mimicking the above-mentioned conditions. We present a case that highlights vitamin B12 and vitamin D deficiency as an easily treatable cause of PUO, hemolytic anemia, and thrombocytopenia, which should be actively looked for and treated before proceeding with more complicated and expensive investigation or starting empiric treatments. PMID:25811010

  4. Inactivation of Escherichia coli O157:H7 by essential oil from Cinnamomum zeylanicum

    Directory of Open Access Journals (Sweden)

    Ouafae Senhaji

    2007-04-01

    Full Text Available Escherichia coli O157:H7 is a pathogen strain, which causes hemorrhagic colitis, hemolytic uremic syndrome and thrombotic thrombocytopenic purpura in humans. The control of bacterial cells in foods is an important factor to reduce foodborne diseases due to E. coli O157:H7. Assays to inactivate E. coli O157:H7 were carried out by using the cinnamon oil obtained by steam distillation for 6 hours. When E. coli O157:H7 cells were incubated at 37°C for 2 hours in the presence of 0.025% of the essential oil from cinnamon, a dramatic decrease was observed in the viable counts (from 10(7 to 3.10(4 CFU/mL-1. In the presence of 0.05% of the oil, most of cells were killed after 30 min, suggesting that the antimicrobial activity of essential oil is bactericidal against E. coli. The minimal inhibitory concentration of the essential oil from cinnamon was around 625 ppm against E. coli O157:H7 and E. coli ATCC 25921, around 1250 ppm against E. coli ATCC25922 and around 2500 ppm against E. coli ATCC11105.

  5. The endemic mimic: blastomycosis an illness often misdiagnosed.

    Science.gov (United States)

    Bradsher, Robert W

    2014-01-01

    One of the endemic fungi, Blastomyces dermatitidis, can cause epidemics of infection with multiple persons involved in a point source outbreak but more commonly causes sporadic cases of infection within the areas of endemicity. Blastomycosis can present as an acute pneumonia which is often misdiagnosed as acute pneumococcal pneumonia or the infection may present as a chronic pneumonia along with weight loss, night sweats, hemoptysis, and a lung mass suggesting tuberculosis or carcinoma of the lung. Extrapulmonary infection with B. dermatitidis is protean with many different manifestations. Most commonly, skin or subcutaneous lesions are found with either a verrucous or warty appearance or in an ulcerative form. Cases have been misidentified as keratoacanthoma, pyoderma gangrenosum, carcinoma, or as Weber-Christian panniculitis if there are nodular subcutaneous lesions. Essentially any site or organ can have lesions of disseminated blastomycosis. In our series, cases of laryngeal carcinoma, adrenal insufficiency, thyroid nodules, granulomatous hypercalcemia, abnormal mammograms thought to represent breast carcinoma, otitis media with cranial extension, immune thrombocytopenic purpura, and hemolytic anemia of unknown cause have been misdiagnosed and blastomycosis subsequently identified as the cause. This infection causes manifestations which mimic many other more commonly diagnosed conditions and must always be considered by clinicians practicing in the endemic region. PMID:25125734

  6. Thrombokinetics with In-111-oxine labelled platelets

    International Nuclear Information System (INIS)

    Indium-111-oxine has been employed as a redioactive platelet label for thrombosis imaging and thrombokinetic studies in man. To evaluate it's suitability for platelet survival and turnover, thrombokinetic studies were carried out in hematological normal subjects, in patients with idiopathic thrombocytopenic purpura (ITP) and chronic congestive splenomegaly. For In-111-oxine labelled platelets, platelets were collected by differential centrifugation from 44 ml of whole blood drawn into 6 ml of acid citrate dextrose solution. Platelet suspension was incubated with In-111-oxine, which was extracted before use by the method of Thakur and co-workers. The survival, recovery and turnover of In-111-labeled platelets were 8.6 +- 0.5 days, 63.0 +- 5.4% and 3.9 +- 0.3 x 104/ μl/day, respectively, which were similar with those of Cr-51 method. Platelet disappearance curves labelled with In-111 and Cr-51 simultaneously were similar in one case. In patients with ITP, platelet survival shortened in the same degree with Cr-51 method. The two simultaneous labeling studies between In-111 and Cr-51 showed no differences. In the patients with congestive splenomegaly, the same results were obtained. Thrombokinetic studies with In-111-oxine labelled platelets offer the advantages of reduced blood requirements, and the ability to perform external imaging of platelet distribution. (author)

  7. Radiotherapy for inoperable and refractory endometriosis presenting with massive hemorrhage: a case report

    Directory of Open Access Journals (Sweden)

    Nomiya Takuma

    2012-09-01

    Full Text Available Abstract Introduction Many patients with endometriosis are treated with medication or by surgical approaches. However, a small number of patients do not respond to medication and are inoperable because of comorbidities. This case report shows the effectiveness of radiotherapy for refractory endometriosis and includes a time series of serum estradiol levels. Case presentation A 47-year-old Asian woman presented to our facility with uncontrolled endometriosis refractory to medication. Our patient was considered inoperable because of severe idiopathic thrombocytopenic purpura, and underwent radiotherapy for massive genital bleeding requiring blood transfusions. A radiation dose of 20Gy in 10 fractions was delivered to the pelvis, including the bilateral ovaries, uterus, and myomas. An additional 10Gy in five fractions was delivered to the endometrium to control residual bleeding. Genital bleeding was completely inhibited on day 46 after radiotherapy. Hormonal analysis revealed that radiotherapy induced post-menopausal status. Two years after radiotherapy, atypical genital bleeding had not recurred and has been well controlled without side effects. Conclusions Disrupted ovarian function is an adverse effect of radiotherapy. However, radiotherapy can be useful for inducing menopause. In cases of medication-refractory or inoperable endometriosis, radiotherapy would be an effective treatment option.

  8. Assessment of spleen size using gamma camera scintigraphy in newly diagnosed patients with essential thrombocythaemia and polycythaemia vera

    Energy Technology Data Exchange (ETDEWEB)

    Carneskog, J.; Wadenvik, H.; Kutti, J. [Univ. of Goeteborg, Sahlgrenska Univ. Hospital, Dept. of Medicine, Haematology Section, Goeteborg (Sweden); Fjaeelling, M. [Univ. of Goeteborg, Sahlgrenska Univ. Hospital, Dept. of Clinical Physiology, Section of Nuclear Med., Goeteborg (Sweden)

    1996-03-01

    By using gamma camera imaging the spleen size was assessed in 18 consecutive patients with essential thrombocythaemia (ET) and in 18 consecutive patients with polycythaemia vera (PV). All ET and PV patients were newly diagnosed and had not received any myelosuppressive therapy prior to study. The spleen areas in both posterior and left lateral projections were determined. Eighteen consecutive patients with idiopathic thrombocytopenic purpura (ITP) served as a control group since by definition they do not present with splenic enlargement; in these latter subjects the mean posterior and left lateral splenic areas were almost identical (48 {+-} 15 and 47 {+-} 17 cm{sup 2}, respectively). In comparison with this control group patients with ET an dPV had significantly larger spleens. In both ET and in PV patients the left lateral spleen scan area exceeded the posterior one. Patients with PV had larger splenic areas in both projections than did patients with ET, but the differences were not statistically significant. Compared to the ITP patients it was found that at least 50% of the ET patients and at least 61% of the PV patients at diagnosis presented with splenomegaly. (au) 35 refs.

  9. The Alternative Pathway of Complement and the Evolving Clinical-Pathophysiological Spectrum of Atypical Hemolytic Uremic Syndrome.

    Science.gov (United States)

    Berger, Bruce E

    2016-08-01

    Complement-mediated atypical hemolytic uremic syndrome (aHUS) comprises approximately 90% of cases of aHUS, and results from dysregulation of endothelial-anchored complement activation with resultant endothelial damage. The discovery of biomarker ADAMTS13 has enabled a more accurate diagnosis of thrombotic thrombocytopenic purpura (TTP) and an appreciation of overlapping clinical features of TTP and aHUS. Given our present understanding of the pathogenic pathways involved in aHUS, it is unlikely that a specific test will be developed. Rather the use of biomarker data, complement functional analyses, genomic analyses and clinical presentation will be required to diagnose aHUS. This approach would serve to clarify whether a thrombotic microangiopathy present in a complement-amplifying condition arises from the unmasking of a genetically driven aHUS versus a time-limited complement storm-mediated aHUS due to direct endothelial damage in which no genetic predisposition is present. Although both scenarios result in the phenotypic expression of aHUS and involve the alternate pathway of complement activation, long-term management would differ. PMID:27524217

  10. Renal thrombotic microangiopathy revisited: When a lesion is not a clinical finding

    Directory of Open Access Journals (Sweden)

    De Serres Sacha

    2010-01-01

    Full Text Available Despite advances in the field of thrombotic microangiopathy (TMA and associated syndromes such as thrombotic thrombocytopenic purpura (TTP and hemolytic uremic syndrome (HUS, they still leave several issues unresolved. For instance, actual diagnostic criteria on which therapeutic decisions rely are relatively narrow and focused on TTP-HUS, with the consequence that non-idiopathic and atypically-presenting TMA are overlooked. In addition, nosologic classifi-cations of TMA disorders have varied substantially over the years, but are still devised from historical rather than mechanistic data. As such, it is perhaps not surprising that even today TMA is erroneously used as an interchangeable term with TTP-HUS, and missed or inappropriately diagnosed on various occasions. Yet, recognizing TMA is of crucial importance given that this lesion often manifests with potentially reversible renal failure. In this editorial, which is presented from a Nephrologist′s perspective, we propose that TMA disorders need to be reclassified to include most types of presentations and confirmed or excluded through more elaborate diagnostic approaches.

  11. Functional evolution of ADAMTS genes: Evidence from analyses of phylogeny and gene organization

    Directory of Open Access Journals (Sweden)

    Van Meir Erwin G

    2005-02-01

    Full Text Available Abstract Background The ADAMTS (A Disintegrin-like and Metalloprotease with Thrombospondin motifs proteins are a family of metalloproteases with sequence similarity to the ADAM proteases, that contain the thrombospondin type 1 sequence repeat motifs (TSRs common to extracellular matrix proteins. ADAMTS proteins have recently gained attention with the discovery of their role in a variety of diseases, including tissue and blood disorders, cancer, osteoarthritis, Alzheimer's and the genetic syndromes Weill-Marchesani syndrome (ADAMTS10, thrombotic thrombocytopenic purpura (ADAMTS13, and Ehlers-Danlos syndrome type VIIC (ADAMTS2 in humans and belted white-spotting mutation in mice (ADAMTS20. Results Phylogenetic analysis and comparison of the exon/intron organization of vertebrate (Homo, Mus, Fugu, chordate (Ciona and invertebrate (Drosophila and Caenorhabditis ADAMTS homologs has elucidated the evolutionary relationships of this important gene family, which comprises 19 members in humans. Conclusions The evolutionary history of ADAMTS genes in vertebrate genomes has been marked by rampant gene duplication, including a retrotransposition that gave rise to a distinct ADAMTS subfamily (ADAMTS1, -4, -5, -8, -15 that may have distinct aggrecanase and angiogenesis functions.

  12. Calpain Activator Dibucaine Induces Platelet Apoptosis

    Directory of Open Access Journals (Sweden)

    Jun Liu

    2011-03-01

    Full Text Available Calcium-dependent calpains are a family of cysteine proteases that have been demonstrated to play key roles in both platelet glycoprotein Ibα shedding and platelet activation and altered calpain activity is associated with thrombotic thrombocytopenic purpura. Calpain activators induce apoptosis in several types of nucleated cells. However, it is not clear whether calpain activators induce platelet apoptosis. Here we show that the calpain activator dibucaine induced several platelet apoptotic events including depolarization of the mitochondrial inner transmembrane potential, up-regulation of Bax and Bak, down-regulation of Bcl-2 and Bcl-XL, caspase-3 activation and phosphatidylserine exposure. Platelet apoptosis elicited by dibucaine was not affected by the broad spectrum metalloproteinase inhibitor GM6001. Furthermore, dibucaine did not induce platelet activation as detected by P-selectin expression and PAC-1 binding. However, platelet aggregation induced by ristocetin or α-thrombin, platelet adhesion and spreading on von Willebrand factor were significantly inhibited in platelets treated with dibucaine. Taken together, these data indicate that dibucaine induces platelet apoptosis and platelet dysfunction.

  13. Anti-D: mechanisms of action.

    Science.gov (United States)

    Ware, R E; Zimmerman, S A

    1998-01-01

    Immunoglobulin that recognizes and binds specifically to the erythrocyte D antigen (anti-D globulin, WinRho SDF; Nabi, Boca Raton, FL) has recently been shown to be an effective therapy for many patients with idiopathic thrombocytopenic purpura (ITP). Its mechanisms of action are not completely understood. Intravenous (IV) infusion of anti-D into a D-positive recipient leads to antibody coating of circulating erythrocytes that are cleared primarily by the spleen. This immune-mediated clearance of sensitized erythrocytes occupies the reticuloendothelial system and allows survival of antibody-coated platelets. Based on clinical observations, experimental data, and theoretical calculations, the efficacy of anti-D therapy in ITP depends on several factors that influence the amount of erythrocyte sensitization and the rate of immune-mediated erythrocyte clearance by the spleen. Antibody characteristics, including the antibody concentration, binding affinity, and dissociation constants, may be important, as well as the number of D-antigen binding sites on the erythrocytes. Although the primary mechanism of action of anti-D is believed to be immunologic blockade of Fc receptors (FcR) within the reticuloendothelial system (RES), other immunomodulatory effects are also possible. PMID:9523745

  14. A Brief Review of Infrequent Spontaneous Findings, Peculiar Anatomical Microscopic Features, and Potential Artifacts in Göttingen Minipigs.

    Science.gov (United States)

    McInnes, E F; McKeag, S

    2016-04-01

    Minipigs are now used in greater numbers in contract research organizations (CROs) as well as in the pharmaceutical industry. Most CROs or pharmaceutical companies use the Göttingen minipig, which displays a number of important background lesions. This review will discuss some of the more infrequent minipig background changes. Porcine stress syndrome is an autosomal recessive pharmacogenetic disorder in minipigs causing malignant hyperthermia and muscle necrosis. Possible triggers, clinical pathology as well as heart, muscle, liver, lung, and kidney histopathology are discussed. Additional spontaneous changes, background findings, and peculiar anatomical and histological features include thrombocytopenic purpura syndrome, spontaneous glomerulonephritis, osteochondritis, ellipsoids, or Schweigger-Seidel sheaths in the spleen, as well as the presence of a perimesenteric plexus adjacent to mesenteric lymph nodes, squamous epithelial metaplasia of the salivary gland, and cupping of the optic disk in the minipig eye. In order to maximize the data gained from minipig studies, the interpretation of pathology findings requires the input of experienced pathologists who understand the significance of artifacts and spontaneous, background lesions in minipigs and can distinguish these from induced lesions. PMID:26839330

  15. Rituximab Administration in Third Trimester of Pregnancy Suppresses Neonatal B-Cell Development

    Directory of Open Access Journals (Sweden)

    D. T. Klink

    2008-01-01

    Full Text Available We describe the effect on the neonate of administration of rituximab to a woman with idiopathic thrombocytopenic purpura (ITP. Rituximab, an anti-CD20 antibody, was given weekly for 4 weeks to a woman with ITP in her third trimester of pregnancy. One month after the last rituximab administration a healthy girl was born. She had normal growth and development during the first six months. At birth, B-lymphocytes were not detectable. Rituximab levels in mother and neonate were 24000 and 6700 ng/mL, respectively. Only 7 cases of rituximab administration during pregnancy were described. No adverse events are described for fetus and neonate. We demonstrate that rituximab passes the placenta and inhibits neonatal B-lymphocyte development. However, after 6 months B-lymphocyte levels normalized and vaccination titres after 10 months were adequate. No infection-related complications occurred. Rituximab administration during pregnancy appears to be safe for the child but further studies are warranted.

  16. Clinical spectrum of thrombocytopenia in adult population of karachi

    International Nuclear Information System (INIS)

    Objective: To determine the etiology and clinical features of patients presenting with bleeding due to thrombocytopenia. Design: A cross sectional study. Place and duration of study:.Th study was carries out at PNS Shifa Hospital, Karachi during the period form 1994-1996. Subjects and Methods: A total of 500 consecutive patients of 15 years or more age with a platelet count of less than 150 x 10/sup 9/L were included in the study. Complete blood count including platelets count was carried out by using electronic counter model T-890 for each patient. Very low platelet count was also confirmed by manual method. Results: among 500 patients of thrombocytopenia the commonest cause was malaria consisting of 216 (43.2%) cases. Megaloblastic anemia was the leading hematological cause, comprising of 31 (6.2%) patients. Other miscellaneous causes like dengue hemorrhagic fever, idiopathic thrombocytopenic purpura aplastic anemia and leukemias were responsible for the rest of cases of thrombocytopenia. Epistaxis followed by gum bleeding was the leading clinical manifestation. Conclusion: We conclude that malaria and viral infections are common causes of transient ghtombocytopenia. Epistaxis and gum bleeding are the leading clinical manifestations in various disease processes in adult population. (author)

  17. Clinical significance of infection with cag A and vac A positive helicobacter pylori strains

    Directory of Open Access Journals (Sweden)

    Sokić-Milutinović Aleksandra

    2004-01-01

    Full Text Available Clinical relevance of infection with different Helicobacter pylori strains was reviewed in this paper. Helicobacter pylori (H. pylori infection plays a role in pathogenesis of chronic gastritis, peptic ulcer disease, gastric adenocarcinoma and MALT lymphoma. Extragastric manifestations of H. pylori infection most probably include acne rosacea and chronic urticaria, while the importance of H. pylori infection for pathogenesis of growth retardation in children, iron deficiency anemia, coronary heart disease, stroke and idiopathic thrombocytopenic purpura remains vague. The expression of two H. pylori proteins, cytotoxin associated protein (cag A and vacuolization cytotoxin (vac A is considered to be related with pathogenicity of the bacterium. It is clear that presence of cag A+ strains is important for development of peptic ulcer; nevertheless, it is also protective against esophageal reflux disease. On the other hand, cag A+ strains are common in gastric adenocarcinoma and MALT lymphoma patients, but it seems that certain subtypes of vac A cytotoxin are more important risk factors. Infection with cag A+ strains is more common in patients with acne rosacea, stroke and coronary heart disease.

  18. Successful treatment of congenital TTP with a novel approach using plasma-derived factor VIII.

    Science.gov (United States)

    Naik, Swati; Mahoney, Donald H

    2013-10-01

    We describe a 19-year-old boy who was diagnosed with congenital thrombotic thrombocytopenic purpura (cTTP) at 7 months of age. He was subsequently treated with fresh frozen plasma infusions every 3 to 4 weeks for the next 15 years at which point he developed significant hypersensitivity reactions to fresh frozen plasma. He required immunosuppressive therapy with systemic desensitization in the intensive care unit but did not tolerate this regimen and suffered debilitating adverse effects. On the basis of the observations from United Kingdom, he was started on a trial with Koate, a plasma-derived factor VIII concentrate with ADAMTS-13 activity that is commercially available in the United States. He tolerated Koate without any complications and attained a target platelet count of>100,000/μL. He has now been in remission for 36 months and responds to exacerbations of cTTP with additional doses of Koate. For patients with cTTP who are intolerant to plasma infusions, therapy with select plasma-derived factor concentrates with ADAMTS-13 activity may represent a reasonable alternative therapy. PMID:23128332

  19. Interpretation and application of Helicobacter pylori in children in new international consensus%幽门螺杆菌新国际共识在儿科的解读及应用

    Institute of Scientific and Technical Information of China (English)

    朱莉; 宋亚萍; 秦丹

    2014-01-01

    儿童幽门螺杆菌(Helicobacter pylori,H.pylori)感染率逐年升高,相关性疾病的发病率也随之增高.Hp感染不仅可引起消化系统疾病发生,还与特发性血小板减少性紫癜、缺铁性贫血、消化不良等有关,从而影响儿童时期的生长发育及营养健康.根除H pylori已成为全球问题,国际及国内均相继制定了共识意见,现参考这些新国际共识对儿童感染H.pylori的诊断及治疗方案的选择进行解读.%Helicobacter pylori (H.pylori) infection rate is increasing these years.The incidence of related diseases subsequently rose.Hp infection can not only cause digestive diseases,but also lead to idiopathic thrombocytopenic purpura (ITP),iron deficiency anemia,and indigestion etc.which can affect the growth and nutritional health of children.H.pylori has become a global issue,both international and domestic colleagues have reached a consensus on treatment for adults.According to the new conscnsus,the aim of this article is to study the diagnosis and treatment options for children with Hp infection.

  20. A role for plasma cell targeting agents in immune tolerance induction in autoimmune disease and antibody responses to therapeutic proteins.

    Science.gov (United States)

    Rosenberg, A S; Pariser, A R; Diamond, B; Yao, L; Turka, L A; Lacana, E; Kishnani, P S

    2016-04-01

    Antibody responses to life saving therapeutic protein products, such as enzyme replacement therapies (ERT) in the setting of lysosomal storage diseases, have nullified product efficacy and caused clinical deterioration and death despite treatment with immune-suppressive therapies. Moreover, in some autoimmune diseases, pathology is mediated by a robust antibody response to endogenous proteins such as is the case in pulmonary alveolar proteinosis, mediated by antibodies to Granulocyte Macrophage-Colony Stimulating Factor (GM-CSF). In this work, we make the case that in such settings, when the antibody response is high titered, sustained, and refractory to immune suppressive treatments, the antibody response is mediated by long-lived plasma cells which are relatively unperturbed by immune suppressants including rituximab. However, long-lived plasma cells can be targeted by proteasome inhibitors such as bortezomib. Recent reports of successful reversal of antibody responses with bortezomib in the settings of ERT and Thrombotic Thrombocytopenic Purpura (TTP) argue that the safety and efficacy of such plasma cell targeting agents should be evaluated in larger scale clinical trials to delineate the risks and benefits of such therapies in the settings of antibody-mediated adverse effects to therapeutic proteins and autoantibody mediated pathology. PMID:26928739