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Sample records for alloimmune thrombocytopenic purpura

  1. Neonatal alloimmun trombocytopenisk purpura (NAITP

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    Bjørn Skogen

    2009-10-01

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

  2. Idiopathic thrombocytopenic purpura

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    L Kayal

    2014-01-01

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

  3. Tuberculosis presenting as immune thrombocytopenic purpura

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    Bahadir-Erdogan Beril

    2004-09-01

    Full Text Available Abstract Background Although various hematologic abnormalities are seen in tuberculosis, immune thrombocytopenic purpura is a rare event. Case Presentation We report a case of a 29 year-old male who was presented with immune thrombocytopenia-induced hemoptysis, macroscopic hematuria and generalized petechiae. The patient was found to have clinical, microbiological and radiological evidence of active pulmonary tuberculosis. The immune thrombocytopenic purpura was successfully treated with anti-tuberculous drugs combined with corticosteroids and high dose immune globulin therapy. Conclusion Immune thrombocytopenic purpura can be one of the hematological manifestations of tuberculosis which has a global prevalence with increasing incidence secondary to HIV infection.

  4. Moxifloxacin (Avelox Induced Thrombotic Thrombocytopenic Purpura

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    Sikander P. Surana

    2012-01-01

    Full Text Available 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 showed numerous schistocytes and she was diagnosed with thrombotic thrombocytopenic purpura. Moxifloxacin was identified as the offending agent. The patient was treated with prednisone and plasmapheresis. To the best of our knowledge, this is the first reported case of thrombotic thrombocytopenic purpura associated with the use of moxifloxacin. Although rare, physicians should be aware of this serious complication associated with its use.

  5. Thrombocytopenic purpura: Importance of early diagnosis

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    Linklater, David M.; Voth, Arnold

    1996-01-01

    Thrombotic thrombocytopenic purpura and hemolytic uremic syndrome are important diagnostic considerations for family physicians because therapy can be lifesaving. Treatment with plasmapheresis is usually successful. Therefore, early diagnosis is essential. A patient with microangiopathic hemolytic anemia is described. Diagnosis of Hodgkin's disease was confirmed after the initial episode. The case highlights the important role of family physicians in the diagnosis and treatment of uncommon he...

  6. Thrombotic Thrombocytopenic Purpura Associated with Pneumococcal Sepsis

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    Jeffrey R Schriber

    1993-01-01

    Full Text Available The first documented case of thrombotic thrombocytopenic purpura (TTP associated with pneumococcal septicemia is reported. This association has been previously demonstrated with hemolytic uremic syndrome. The patient presented with recurrent seizures, oliguric renal failure, fever, thrombocytopenia and microangiopathic hemolytic anemia; coagulation studies were normal. Blood and sputum cultures were positive for Streptococcus pneumoniae. The patient responded to therapy with plasmapheresis and antiplatelet agents as well as antibiotics. Coincident infection should be searched for in all cases of TTP.

  7. Idiopathic Thrombocytopenic Purpura Misdiagnosed as Hereditary Angioedema

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    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....

  8. Thrombotic thrombocytopenic purpura concomitant with autoimmune thyroiditis

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    Ali Bay

    2011-12-01

    Full Text Available Thrombotic thrombocytopenic purpura (TTP is characterized by disseminated thrombotic occlusions located in the microcirculation, microangiopathic hemolytic anemia, thrombocytopenia, fever, and renal and neurologic abnormalities. A 14 year old girl admitted to our hospital complaining bruising on her body and prolonged menstrual bleeding. On her physical examination there were very common bruising on four extremities. On the laboratory studies, Hemoglobin was 9 g/dL; Hematocrit, 24%; white blood count 11600/mm3 and thrombocyte count, 9.000/mm3. According to these findings our first diagnose was idiopatic thrombocytopenic purpura so intravenous immunoglobulin was given to patient for two days. Bone marrow aspiration was performed because of persisting thrombocytopenia despite two days IVIG therapy. Increased number of megakaryocytes was seen in bone marrow. Some accompanying symptoms like headache, numbness in per oral region and extremities, difficulty in speaking, and fluctuation in consciousness for short time occurred. The patient was reevaluated; because thrombocytopenia persisted and some neurological symptoms was observed. Due to these findings we thought that TTP was the diagnosed and plasma exchange was started. Increase was seen in platelet count in the second days of treatment. TTP should be considered in children presenting with atypical thrombocytopenia.

  9. Idiopathic thrombocytopenic purpura: laboratory diagnosis and management

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    Alvina Alvina

    2016-02-01

    Full Text Available Idiopathic thrombocytopenic purpura (ITP or immune thrombocytopenic purpura is a disease characterized by low platelet count (<150,000/ìL caused by autoantibody-mediated platelet destruction and the absence of other causes of thrombocytopenia. Acute primary ITP is more common in children 2-6 years of age, with similar incidence between males and females, while the chronic form is usually encountered in adults with median age of 40-45 years. The clinical signs of ITP are purpura, ecchymosis, petechiae and gastrointestinal tract bleeding, gingival bleeding, epistaxis, and urinary tract bleeding. Spontaneous mucosal, intracranial, and gastrointestinal hemorrhage may occur at platelet counts of <10000/ìL. To date, the diagnosis of ITP is still arrived at by exclusion, i.e. by elimination of other causes of thrombocytopenia. The diagnosis of ITP also requires a medical history (anamnesis, physical examination, platelet count, and examination of a peripheral blood smear. The latter examination in ITP shows low numbers of normal-sized platelets, occasionally also giant platelets, while erythrocytes and leukocytes have a normal morphology. The bone marrow is usually normal or shows increased megakaryocytes. Assessment of antithrombocyte antibody may assist in establishing the diagnosis of ITP. Management of ITP is based on platelet count and severity of bleeding. Treatment is aimed at interfering with antibodies that damage the platelets, by inhibiting the functions of macrophage Fcã receptors and decreasing the production of antiplatelet antibodies. Thrombopoietin (TPO receptor agonists including eltrombopag and romiplostim have offered an important new option in treating ITP.

  10. Thrombocytopenic purpura as initial presentation of acute hepatitis A.

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    Scott, Julius Xavier; Gnananayagam, Ebor Jacob; Gupta, Sanjay; Simon, Anna; Mukhopadhya, Ashish

    2003-01-01

    Extrahepatic immune manifestations are rare in hepatitis A virus infection. We report a 4 1/2-year-old girl who presented with thrombocytopenic purpura as initial manifestation of hepatitis A virus infection. She responded to steroid therapy.

  11. A rare combination of thrombotic thrombocytopenic purpura and antiphospholipid syndrome.

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    Viner, Maya; Murakhovskaya, Irina

    2016-11-24

    Thrombocytopenia, in the setting of microangiopathic hemolytic anemia and thrombotic events, is characteristic of both thrombotic thrombocytopenic purpura and primary antiphospholipid syndrome. Clinically, it is difficult to distinguish between these two syndromes. We present a 41-year-old woman with chronic, relapsing thrombotic thrombocytopenic purpura in the presence of antiphospholipid antibodies. She had clinical manifestations of antiphospholipid syndrome without meeting laboratory criteria of the Sydney classification system. In the literature, there have only been nine cases of thrombotic thrombocytopenic purpura associated with primary antiphospholipid syndrome. Seven of the nine cases suffered from one or multiple strokes, a common feature in antiphospholipid syndrome, but an uncommon finding in thrombotic thrombocytopenic purpura. We introduce the possibility of an association between thrombotic thrombocytopenic purpura and the presence of antiphospholipid antibodies. Systematic testing of ADAMTS13 activity and anti-ADAMTS13 antibodies in patients who present with neurological symptoms and thrombocytopenia, in the presence of antiphospholipid antibodies, may help with the diagnosis of the rare thrombotic thrombocytopenic purpura-antiphospholipid syndrome combination.

  12. Idiopathic Thrombocytopenic Purpura Misdiagnosed as Hereditary Angioedema

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    Michelle Fog Andersen

    2015-01-01

    Full Text Available 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.

  13. Refractory thrombotic thrombocytopenic purpura following acute pancreatitis

    Institute of Scientific and Technical Information of China (English)

    Ebisa Bekele; Bethel Shiferaw; Alexandra Sokolova; Arpan Shah; Phillip Saunders; Alida Podrumar; Javed Iqbal

    2016-01-01

    Thrombotic thrombocytopenic purpura (TTP) is a rare blood disorder with an estimated incidence of 4–5 cases per million people per year. It is characterized by small-vessel platelet-rich thrombi that cause thrombocytopenia, microangiopathic hemolytic anemia and organ damage. There are reports in literature that TTP and acute pancreatitis are associated, indicating each can be the cause of the other. However, acute pancreatitis triggering TTP is very rare. A 71 years old female presented with abdominal pain of 3 days, followed by dark urine. She had icteric sclera, petechial rash and mild epigastric tenderness. Lab findings were significant for hemolytic anemia, thrombocytopenia and elevated lipase. CT of abdomen showed evidence of pancreatitis and cholelithiasis. After admission, patient developed symptoms of stroke. Further investigation showed elevated lactate dehydrogenase and normal coagulation studied with peripheral blood smear showed 5–6 schistocytes/high power field. Disintegrin and metalloproteinase with thrombospondin motifs-13 (ADAMTS13) activity showed less than 3% with high ADAMTS13 inhibitor 2.2. Patient required 6–7 weeks of daily plasmapheresis until she showed complete response. Our patient presented with clinical features of pancreatitis prior to having dark urine and petechial rash. Therefore, we strongly believe that our patient had pancreatitis which was followed by TTP. Patient's ADMTS13 activity was 6%after 10 plasma exchanges, signifying refractory TTP and higher risk for morbidity and mortality. There are limited data and consensus on the management of refractory TTP. TTP and acute pancreatitis are associated. However, refractory TTP following acute pancreatitis is rarely mentioned in the literature. We would like to emphasize the importance of having higher clinical suspicion of the association of both disease entities.

  14. Chronic immune thrombocytopenic purpura. New agents.

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    Rodeghiero, F; Ruggeri, M

    2009-01-01

    First generation thrombopoietic growth factors (rhTPO and PEG-rHuMGDF), investigated in the early 2000s, proved effective in increasing platelet count in normal volunteers, in thrombocytopenia due to chemotherapy and also in a few cases of immune thrombocytopenic purpura (ITP). These agents did not complete their clinical development since one of them induced antibodies in the recipients that cross reacted with endogenous thrombopoietin (TPO), thus causing thrombocytopenia. This promoted the ingenious design of a new generation of thrombopoietic growth factors having no sequence homology with natural TPO. The two main agents are romiplostim, a peptibody already approved for clinical use in USA and eltrombopag, a non-peptide, orally active small molecule. In open label and placebo-controlled trials both agents proved to predictably increase platelet count in normal volunteers and in patients with ITP. With appropriate dosages (1-10 microg/kg weekly sub cutaneously for romiplostim; 50-75 mg/die per os for eltrombopag ) a platelet increase becomes significant after 7-10 days and peaks between 10-14 days. By discontinuing treatment, platelet count returns to baseline level in 10-15 days. The response rate with both agents is above 70-80%, also in patients that had undergone several lines of treatment, or that have failed splenectomy. The response is maintained during the treatment, but is almost invariably lost even after several months of successful administration. Due to the lack of a curative potential and to the incomplete knowledge of long-term side effects, the place of these new drugs in the management of ITP is still unsettled and their use is best restricted to refractory patients or in preparation of splenectomy. It seems however that a new paradigm in the treatment of ITP has been established where the focus is not on reducing platelet consumption but on increasing platelet production.

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

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    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

  16. The potential role of thrombopoietin in idiopathic thrombocytopenic purpura

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    von dem Borne, A; Folman, C; van den Oudenrijn, S; de Jong, S; de Haas, M

    2002-01-01

    Thrombopoietin (TPO) plays a central role in the pathogenesis of idiopathic thrombocytopenic purpura (ITP), as it does in other immune-mediated thrombocytopenias. Because TPO is bound and internalized by platelets, it is destroyed together with platelets at an accelerated rate in the macrophage syst

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

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    Fengyi Shen

    2014-01-01

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

  18. Acute immune thrombocytopenic purpura triggered by insect bite

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    Namdev R

    2009-03-01

    Full Text Available Idiopathic Thrombocytopenic Purpura (ITP is a blood disorder characterized by an abnormal decrease in the number of platelets in the blood. Acute thrombocytopenic purpura is most commonly seen in young children, with a sudden onset, following a viral illness, vaccination or an insect bite. The report is a rare case of ITP, which was triggered by a honey-bee bite and caused continuous intermittent bleeding from the gingival sulcular region of the maxillary left permanent first molar, and ecchymotic spots on the forehead, scalp, and lower limbs. A complete hemogram revealed severe thrombocytopenia with platelet count as low as 15,000/mm3. The patient was immediately hospitalized and administered platelet replacement and medication. With this report, the authors, as dentists, emphasize the significance of recognition, early diagnosis, and referral of such patients with bleeding disorders to specialized centers, for prompt treatment.

  19. Splenectomy during pregnancy: treatment of refractory immune thrombocytopenic purpura

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    Mahey, Reeta; Kaur, Simran Deep; Chumber, Sunil; Kriplani, Alka; Bhatla, Neerja

    2013-01-01

    Immune thrombocytopenic purpura (ITP) complicates 1–2/10 000 pregnancies and accounts for 5% of cases of pregnancy-associated thrombocytopenia. Corticosteroids and intravenous immunoglobulin remain the first-line therapy in pregnancy, and a majority of pregnant women respond to this conventional therapy. Other cytotoxic and immunosuppressive agents used for treatment in non-pregnant patients, for example, danazol, cyclophosphamide, vinca alkaloids and azathioprine, are potential teratogens an...

  20. Thrombotic thrombocytopenic purpura in the first trimester of pregnancy

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    Pooja Sikka

    2013-01-01

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

  1. Immune thrombocytopenic purpura might be an early hematologic manifestation of undiagnosed human immunodeficiency virus infection.

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    Lai, Shih-Wei; Lin, Hsien-Feng; Lin, Cheng-Li; Liao, Kuan-Fu

    2017-03-01

    Little research focuses on the association between immune thrombocytopenic purpura and human immunodeficiency virus infection in Taiwan. This study investigated whether immune thrombocytopenic purpura might be an early hematologic manifestation of undiagnosed human immunodeficiency virus infection in Taiwan. We conducted a retrospective population-based cohort study using data of individuals enrolled in Taiwan National Health Insurance Program. There were 5472 subjects aged 1-84 years with a new diagnosis of immune thrombocytopenic purpura as the purpura group since 1998-2010 and 21,887 sex-matched and age-matched, randomly selected subjects without immune thrombocytopenic purpura as the non-purpura group. The incidence of human immunodeficiency virus infection at the end of 2011 was measured in both groups. We used the multivariable Cox proportional hazards regression model to measure the hazard ratio and 95 % confidence interval (CI) for the association between immune thrombocytopenic purpura and human immunodeficiency virus infection. The overall incidence of human immunodeficiency virus infection was 6.47-fold higher in the purpura group than that in the non-purpura group (3.78 vs. 0.58 per 10,000 person-years, 95 % CI 5.83-7.18). After controlling for potential confounding factors, the adjusted HR of human immunodeficiency virus infection was 6.3 (95 % CI 2.58-15.4) for the purpura group, as compared with the non-purpura group. We conclude that individuals with immune thrombocytopenic purpura are 6.47-fold more likely to have human immunodeficiency virus infection than those without immune thrombocytopenic purpura. We suggest not all patients, but only those who have risk factors for human immunodeficiency virus infection should receive testing for undiagnosed human immunodeficiency virus infection when they develop immune thrombocytopenic purpura.

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

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    Arimoto M

    2012-03-01

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

  3. Treatment of immune thrombocytopenic purpura: focus on eltrombopag

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    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

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

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    Houwerzijl, Ewout Johan

    2008-01-01

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

  5. A Remarkable Improvement of Patients with Idiopathic Thrombocytopenic Purpura after appendectomy including Carcinoid tumor

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    Hamidreza Alizadeh-Otaghvar; Saeedeh Firoozbakht; Sahar Montazeri; Samaneh Khazraie; Marjan Bani Ahmad; Maryam Hajiloo

    2011-01-01

    The assistance of chronic idiopathic thrombocytopenic purpura and appendiceal carcinoid tumor is so rare and has not been reported before .we present here in a 11 year old boy that is the known case of chronic idiopathic thrombocytopenic purpura (with the platelet count of 20000 when admitted). He reffered due to anorexia, pain and tenderness of right lower quadrant of abdomen and vomiting. He also had leukocytosis and abdominal free fluid in sonographic report. These findings suggested the d...

  6. Postinfluenza Vaccination Idiopathic Thrombocytopenic Purpura in Three Elderly Patients

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    Joji Nagasaki

    2016-01-01

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

  7. Association of primary biliary cirrhosis with idiopathic thrombocytopenic purpura

    Institute of Scientific and Technical Information of China (English)

    Nobuyuld Toshikuni; Ryumei Yamato; Haruhiko Kobashi; Ken Nishino; Nobu Inada; Ritsuko Sakanoue; Mitsuhiko Suehiro; Yoshinori Fujimura; Gotaro Yamada

    2008-01-01

    Although both primary biliary cirrhosis (PBC) and idiopathic thrombocytopenic purpura (ITP) are autoimmune diseases, the association of the 2 diseases is rare. Here, we report a case of ITP that developed during the follow-up of PBC in a 74-yearold man. The patient had been diagnosed with PBC 12 years previously, and had received treatment with ursodeoxycholic acid. The platelet count decreased from approximately 60 × 109/L to 8 × 109/L, and the association of decompensated liver cirrhosis (PBC) with ITP was diagnosed. Steroid and immune gamma globulin therapy were successful in increasing the platelet count.Interestingly, human leukocyte antigen genotyping detected the alleles DQBI*0601 and DRBI*0803, which are related to both PBC and ITP in Japanese patients.This case suggests common immunogenetic factors might be involved in the development of PBC and ITP.

  8. Thrombopoietin mimetic agents in the management of immune thrombocytopenic purpura.

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    Newland, Adrian

    2007-10-01

    Thrombopoietin (TPO) is a potent endogenous cytokine and the principal regulator of platelet production. Advances in the understanding of the structure of TPO enabled development of the first generation of thrombopoietic growth factors, recombinant human thrombopoietin (rhTPO) and pegylated human recombinant megakaryocyte growth and development factor (PEG-rHuMGDF). Clinical results showed that these agents were effective in promoting increases in platelet counts in a variety of thrombocytopenic disorders. However, clinical development was halted when studies demonstrated risk for autoantibody formation with cross-reactivity to endogenous TPO. A second generation of thrombopoietic growth factors, including TPO peptide and nonpeptide mimetics and TPO agonist antibodies, utilizing different mechanisms from recombinant growth factors to promote platelet production, are currently in development. The TPO peptide mimetic AMG 531 and the nonpeptide mimetic eltrombopag are in advanced clinical trials and have both resulted in dose-dependent increases in platelets in healthy subjects and in significant increases in platelets in patients with chronic immune thrombocytopenic purpura (ITP). Clinical trials are also being conducted to examine the efficacy and safety of eltrombopag to treat thrombocytopenia in hepatitis C virus (HCV)-infected individuals. These agents appear to be well tolerated and the formation of autoantibodies appears to be limited to first-generation growth factors. Increases in marrow reticulin have been demonstrated with some growth factors, but this appears to be a reversible phenomenon and is not associated with formation of collagen fibrosis. There appears to be no increased incidence of thrombotic events in patients who achieve high platelet counts with growth factor treatments, and although occasional thrombotic events have been reported, their association to the treatment is uncertain. While there is evidence that activation of signaling pathways

  9. [Thrombotic thrombocytopenic purpura disclosing cancer: apropos of 2 cases].

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    Girard, P; Tardy, B; Page, Y; Mosnier, J F; Tardy-Poncet, B; Bertrand, J C

    1995-01-21

    Thrombotic thrombocytopenic purpura (TTP) causes severe haemolytic anaemia, thrombopenia, fever and neurological and renal involvement. Currently five large aetiologic groups have been identified: viral or bacterial infection, drugs, conjunctive tissue diseases, pregnancy and solid tumours. We observed two cases resulting from an adenocarcinoma. In the first case, a 71-year-old man with chronic silicosis, the presenting signs were asthenia, fever, epistaxis with diffus purpura and spontaneous haematomas of the lower limbs. Diagnosis of TTP was based on routine laboratory tests and the patient responded well to fresh frozen plasma. On the 5th day of treatment, haemoglobin level dropped sharply and melana occurred. Upper digestive tract endoscopy revealed a tumoural formation of the antrum-fundic junction and histology examination of the biopsy confirmed the diagnosis of adenocarcinoma. Ten months after gastrectomy the patient was in excellent health with no relapse of the TTP. In the second case, the presenting signs included spontaneous haematomas, rectorrhagia and low grade fever. Microscopic haematuria and renal failure were observed in addition to the biological syndrome of TTP. The patient responded poorly to fresh frozen plasma and packed cell transfusions. Plasma exchange was equally unsuccessful. The disease continued a fulminant course and the diagnosis of adenocarcinoma located in a pulmonary lymph nodes was made at autopsy. These rare cases of TTP caused by cancer emphasize the importance of a thorough aetiological research. Plasma exchange has been shown to be effective but mortality at 1 year approximately 85% in cancer related cases. Early diagnosis and specific anti-cancer therapy might improve prognosis. We report our personal experience with 16 other similar cases.

  10. Changes in splenic microcirculatory pathways in chronic idiopathic thrombocytopenic purpura.

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    Schmidt, E E; MacDonald, I C; Groom, A C

    1991-09-15

    The spleen plays a central role in the pathogenesis of chronic idiopathic thrombocytopenic purpura (ITP); it produces massive quantities of antiplatelet antibodies, leading to accelerated phagocytosis of platelets. Lymphoid hyperplasia typically occurs in the spleen, characterized by large numbers of lymphatic nodules with active germinal centers. Whether changes in splenic microcirculatory pathways also occur is not known. We have studied this question by scanning electron microscopy of corrosion casts, comparing spleens removed from patients with ITP with normal spleens obtained from organ transplant donors. The casts demonstrate two major changes in microcirculatory pathways in ITP. Firstly, a striking proliferation of arterioles and capillaries is found in the white pulp and marginal zone (MZ), seen as extensive vascularization in 92.3% of lymphatic nodules (n = 191) versus 0.6% (n = 224) in normal spleens. Secondly, the marginal sinus, a series of flattened, anastomosing vascular spaces between the white pulp and MZ, is absent in 89.4% of lymphatic nodules versus 4.9% in normal spleens. The cause of these microcirculatory changes, which may not be exclusive to ITP, is presently unknown. Absence of the marginal sinus may affect distribution of blood flow through the MZ such that platelets spend increased amounts of time in the proximity of macrophages. In the presence of antiplatelet antibodies found in ITP spleens, this delayed transit would lead to greatly increased platelet destruction.

  11. Pulmonary Hyalinizing Granuloma Associated with Idiopathic Thrombocytopenic Purpura

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    Christopher Coleman

    2014-01-01

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

  12. 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.

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

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

    2016-10-01

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

  14. Immune thrombocytopenic purpura as sole manifestation in a case of acute hepatitis A.

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    Tanir, Gönül; Aydemir, Cumhur; Tuygun, Nilden; Kaya, Ozge; Yarali, Neşe

    2005-12-01

    Acute hepatitis due to hepatitis A virus is usually a benign selflimiting disease during childhood. Although many viral infections such as hepatitis B virus, Parvovirus, and Epstein-Barr virus are associated with extrahepatic autoimmune phenomena, such manifestations are rare in patients with acute hepatitis A infection. Immune thrombocytopenia is a benign, self-limiting disease in children, responding well to treatment and generally associated with viral infections. Immune thrombocytopenic purpura is rarely reported as a manifestation of acute hepatitis A. We report a five-year-old boy with immune thrombocytopenic purpura as the sole manifestation of anicteric acute hepatitis A infection. Acute hepatitis A should be included in the differential diagnosis of immune thrombocytopenic purpura.

  15. Takotsubo cardiomyopathy and thrombotic thrombocytopenic purpura preceding a lupus diagnosis: a case report.

    Science.gov (United States)

    Georgiades, F; Demosthenous, S; Braimi, M; Tsitskari, T; Psarelis, S

    2015-11-01

    Takotsubo cardiomyopathy, a rare stress-related cardiomyopathy, has been observed in a few cases secondary to systemic lupus erythematosus (SLE). Herein, we report an unusual case where a postmenopausal woman presented initially with Takotsubo syndrome, later developed thrombotic thrombocytopenic purpura and cerebrovascular events, initially without clinical or laboratory features of SLE. During the course of her illness, she was found to satisfy four of the Systemic Lupus International Collaborating Clinics classification criteria for a SLE diagnosis. This unique presentation of our patient, initially with Takotsubo cardiomyopathy, the development of thrombotic thrombocytopenic purpura and cerebrovascular events preceding the diagnosis of SLE illustrates the importance of clinical observation and follow-up.

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

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    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.

  17. Treatment of 36 cases of thrombotic thrombocytopenic purpura

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    Suvajdžić-Vuković Nada D.

    2004-01-01

    Full Text Available Thirty-six patients (pts. with thrombotic thrombocytopenic purpura (TTP were treated between May 1990 and May 2003. There were 31 women and 5 men; the average age was 37 years. Twenty-five cases were idiopathic and 11 secondary (3 infection - related, 5 occurred during pregnancy and 3 were drug - associated. The mean lag period between the first symptoms and the diagnosis was 8.5 days (in 14 pts. £ 5; in 22 > 5. On diagnosis neurological symptoms were present in 31, bleeding in 33, fever in 21 and renal impairment in 27 patients. The mean hemoglobin was 67.5 g/L, the mean platelet count was 10´109/L, and the mean reticulocytosis was 17%. The mean serum LDH was 1457 IU. Treatment included plasma exchange (PE in 24 pts. and only plasma infusions in 12 pts. There were 24 complete responders (20 on PE and 12 deaths (4 on PE; PE significantly improved survival (p<0.01. There were 5 treatment-related complications due to the infection and bleeding, 17 exacerbations and 4 relapses. The mean time delay before the onset of symptoms and the treatment initiation lasted for 9 days suggesting the poor disease recognition; the mean time delay from diagnosis to PE institution was 6 days, indicating postponed PE. The mean treatment duration in all patients was 18 days; the mean number of PE cycles needed for the platelet count stabilization was 9. Good prognostic indicators of survival were: the longer prodromal period (>5 days, the secondary form of TTP and the absence of coma at presentation. The use of PE significantly improved survival. TTP is a severe disorder requiring early recognition and diagnosis in general medical care facilities, which should lead to the timely treatment with PE.

  18. Refractory thrombotic thrombocytopenic purpura in a 16-year-old girl: successful treatment with bortezomib

    NARCIS (Netherlands)

    Balen, T. van; Schreuder, M.F.; Jong, H. de; Kar, N.C.A.J. van de

    2014-01-01

    We present a case of a 16-year-old girl with autoimmune thrombotic thrombocytopenic purpura (TTP), refractory to plasma exchange and high-dose prednisone. Despite the additional treatment with rituximab, she developed renal and neurological complications with ongoing hemolysis and thrombocytopenia.

  19. 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.

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

    NARCIS (Netherlands)

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

    2006-01-01

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

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

    NARCIS (Netherlands)

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

    2007-01-01

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

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

    NARCIS (Netherlands)

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

    2004-01-01

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

  3. Efficacy of romiplostim in patients with chronic immune thrombocytopenic purpura : a double-blind randomised controlled trial

    NARCIS (Netherlands)

    Kuter, David J.; Bussel, James B.; Lyons, Roger M.; Pullarkat, Vinod; Gernsheimer, Terry B.; Senecal, Francis M.; Aledort, Louis M.; George, James N.; Kessler, Craig M.; Sanz, Miguel A.; Liebman, Howard A.; Slovick, Frank T.; de Wolf, J. Th M.; Bourgeois, Emmanuelle; Guthrie, Troy H.; Newland, Adrian; Wasser, Jeffrey S.; Hamburg, Solomon I.; Grande, Carlos; Lefrere, Francois; Lichtin, Alan Eli; Tarantino, Michael D.; Terebelo, Howard R.; Viallard, Jean-Francois; Cuevas, Francis J.; Go, Ronald S.; Henry, David H.; Redner, Robert L.; Rice, Lawrence; Schipperus, Martin R.; Guo, D. Matthew; Nichol, Janet L.

    2008-01-01

    Background Chronic immune thrombocytopenic purpura (ITP) is characterised by accelerated platelet destruction and decreased platelet production. Short-term administration of the thrombopoiesis-stimulating protein, romiplostim, has been shown to increase platelet counts in most patients with chronic

  4. A Remarkable Improvement of Patients with Idiopathic Thrombocytopenic Purpura after appendectomy including Carcinoid tumor

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    Hamidreza Alizadeh-Otaghvar

    2011-09-01

    Full Text Available The assistance of chronic idiopathic thrombocytopenic purpura and appendiceal carcinoid tumor is so rare and has not been reported before .we present here in a 11 year old boy that is the known case of chronic idiopathic thrombocytopenic purpura (with the platelet count of 20000 when admitted. He reffered due to anorexia, pain and tenderness of right lower quadrant of abdomen and vomiting. He also had leukocytosis and abdominal free fluid in sonographic report. These findings suggested the diagnosis of acute appendicitis and the patient underwent appendectomy, after the operation, the platelet count became normal. The pathologic report of the specimen of the appendix was carcinoid tumor. Now the question is whether the appendiceal carcinoid tumor can be the reason of symptoms of chronic idiopathic.

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

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    Gruber, O.; Cramon, D.Y. von [Day-Care Clinic for Cognitive Neurology, University of Leipzig (Germany); Max Planck Institute of Cognitive Neuroscience, Leipzig (Germany); Wittig, I. [Day-Care Clinic for Cognitive Neurology, University of Leipzig (Germany); Wiggins, C.J. [Max Planck Institute of Cognitive Neuroscience, Leipzig (Germany)

    2000-08-01

    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.)

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

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    Vivek Chowdhry

    2013-01-01

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

  7. Rotavirus-associated immune thrombocytopenic purpura in children: A retrospective study

    OpenAIRE

    Ai, Qi; Yin, Jing; Chen, Sen; Qiao, Lijin; Luo, Na

    2016-01-01

    Certain studies have previously indicated that an association may exist between rotavirus infection and primary immune thrombocytopenic purpura (ITP). The present retrospective study aimed to investigate whether rotavirus may cause ITP in children. Firstly, the incidence of ITP in children with or without rotavirus diarrhea was compared. A 14.58% incident rate was observed in children with rotavirus diarrhea compared with a 7.22% incident rate in children without rotavirus diarrhea. Subsequen...

  8. Squamous cell carcinoma larynx presenting as idiopathic thrombocytopenic purpura

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    Bekur R

    2015-01-01

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

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

    Science.gov (United States)

    2016-01-01

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

  10. Immune Thrombocytopenic Purpura and Gastritis by H. pylori Associated With Type 1 Diabetes Mellitus

    Science.gov (United States)

    Correa, Ricardo; Flores-Guevara, Igor; Espinoza Morales, Frank; Mejia, Christian R

    2016-01-01

    We present the 15th case reported worldwide and 3rd case reported in Latin America of immune thrombocytopenic purpura associated with Type 1 diabetes mellitus in Scopus, MEDLINE, and SciELO. An 11-year-old male patient of mixed ethnicity with immune thrombocytopenic purpura, Type 1 diabetes mellitus, and gastritis due to H. pylori presented to the emergency room with petechiae, ecchymosis, and gingival and conjunctival bleeding that had been worsening for the past three months. The patient had a body mass index of 18.85 kg/m2 (P75). A biochemical analysis showed 1×109 platelets/L, increased prothrombin time, increased partial thromboplastin time, and an HbA1C of 7.84% on admission. He was prescribed a single dose of intravenous methylprednisolone 750 mg in 100 mL of NaCl and daily oral 50 mg prednisolone, with intravenous 250 mg tranexamic acid every eight hours. The patient’s glycemic control was continued with the administration of insulin glargine (30 units every 24 hours) and prandial insulin glulisine (five to eight units per meal). Before admission, the patient was on a prescribed treatment of sitagliptin 50 mg and metformin 850 mg, but this was suspended in the emergency room. For the eradication of H. pylori he was prescribed amoxicillin 500 mg every eight hours, oral clarithromycin 335 mg every 12 hours, and IV omeprazole 40 mg. After 15 days, he showed disease resolution and he was discharged to his home with orders to follow-up with pediatrics, hematology, and endocrinology services. The first-line treatment for immune thrombocytopenic purpura patients with active bleeding and a platelet count < 30,000 platelets/μl is the administration of corticosteroids and inmunoglobulin. PMID:27026836

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

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    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.

  12. 601 Autoimmune Thrombocytopenic Purpura Associated with Common Variable Immunodeficiency

    Science.gov (United States)

    Mendieta, Elizabeth; Del Rivero, Leonel Gerardo

    2012-01-01

    Background Common variable immunodeficiency (CVID) is a condition characterized by antibody deficiency, and therefore susceptible to recurrent pyogenic infections, cancer and autoimmune diseases. It is a heterogeneous syndrome in primary immunodeficiencies and clinically the most important is often diagnosed in adulthood. Autoimmunity occurs in 5% of the general population, in patients with CVID the percentage increased to 20 to 48%, cytopenias being the most common cause of autoimmunity in these patients. Autoimmune thrombocytopenic purpura and autoimmune hemolytic anemia are the most common autoimmune consequences, occurring in 5% to 8% of all patients with CVID. Some patients develop these disorders before the diagnosis of CVID. Methods We present the case of a woman of 45 year old, with a history of lower respiratory tract and urinary tract infections in recurrent Pulmonary Tuberculosis. Enter the program short-course treatment strictly supervised for pulmonary tuberculosis with appropriate response. Autoinmune thrombocytopenic purpura refractory to steroids (WWTP) for performing splenectomy. Results Anti DNA antibodies, anti nuclear, anti-protease, C. ANCA/PR3 antimieloperoxidasa, serology for hepatitis B, C, HIV negative. Serum immunoglobulins were as follow: IgG, 158 mg/dL (normal 700 to 1600), IgM, 55 mg/dL (normal 40–230), IgA, 36 mg/dL (normal 70–400), and, IgE, 38.7 IU/mL (normal 0–100) in more than 2 occasions with values below 2 standard deviations. CD4 T lymphocytes (19%) CD4/CD8 ratio (0.54). Conclusions Meets diagnostic criteria for Common Variable Immunodeficiency (CVID) and starting treatment with intravenous immunoglobulin at a dose of 400 mg/kg (every 21 days) with significant clinical improvement and has even managed to integrate into your daily activities. Today, he continues with danazol for WWTP. Therefore, CVID is necessary to consider in the differential diagnosis of autoimmune thrombocytopenic purpura and autoimmune hemolytic anemia

  13. Severe Thrombocytopenic Purpura in a Child with Brucellosis: Case Presentation and Review of the Literature

    Science.gov (United States)

    Perogiannaki, Aikaterini; Chaliasos, Nikolaos

    2017-01-01

    Brucellosis is still endemic and a significant public health problem in many Mediterranean countries, including Greece. It is a multisystemic disease with a broad spectrum of clinical manifestations including hematological disorders, such as anemia, pancytopenia, leucopenia, and thrombocytopenia. Thrombocytopenia is usually moderate and attributed to bone marrow suppression or hypersplenism. Rarely, autoimmune stimulation can cause severe thrombocytopenia with clinically significant hemorrhagic manifestations. We present the case of a girl with severe thrombocytopenic purpura as one of the presenting symptoms of Brucella melitensis infection. Treatment with intravenous immunoglobulin and the appropriate antimicrobial agents promptly resolved the thrombocyte counts. A review of similar published cases is also presented. PMID:28127481

  14. 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.

  15. 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.

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

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    Subhash Chandra

    2014-04-01

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

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

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    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

  18. Multiple cycles of recombinant human thrombopoietin therapy in a patient with chronic refractory idiopathic thrombocytopenic purpura.

    Science.gov (United States)

    Hua, Baolai; Zou, Nong; Wang, Shujie; Zhu, Tienan; Zhao, Yongqiang

    2005-06-01

    We describe a 41-year-old woman with chronic idiopathic thrombocytopenic purpura who received recombinant human thrombopoietin (rhTPO) therapy. rhTPO was administrated subcutaneously at a dosage of 1.0 mug/kg daily for a maximum of 14 days until the platelet count was more than 50 x 10/l. The patient received three cycles (six, 13, and eight doses each) of rhTPO, each initiated when the platelet counts was less than 10 x 10/l. The platelet count increased to above 50 x 10/l on days 5, 11 and 8, and peaked at 456 x 10/l, 130 x 10/l and 82 x 10/l on days 9, 15 and 13 in the three respective cycles, each followed by a gradual decline. The durations of platelet counts at more than 50 x 10/l in the three cycles were 13, 7 and 10 days, respectively. rhTPO was well tolerated with no adverse event observed. Antibodies to rhTPO by enzyme-linked immunosorbent assay were not detected. Our observations suggested that rhTPO could transiently increase the peripheral platelet count in patients with chronic refractory idiopathic thrombocytopenic purpura. The reasons why the peak platelet counts decreased and the duration of response shortened after successive cycles of treatment were unclear.

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

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

    2003-10-01

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

  20. Effects of prednisone and splenectomy in patients with idiopathic thrombocytopenic purpura : only splenectomy induces a complete remission

    NARCIS (Netherlands)

    Louwes, H; Vellenga, E; Houwerzijl, EJ; de Wolf, JTM

    2001-01-01

    Idiopathic thrombocytopenic purpura (ITP) is a heterogeneous disease, whereby it is unclear if and in which way prednisone and splenectomy affect the platelet kinetics leading to a complete remission. To determine the effects of prednisone and splenectomy on the mean platelet life (MPL) and platelet

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

    NARCIS (Netherlands)

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

    2009-01-01

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

  2. Immune Thrombocytopenic Purpura Associated with Hepatitis A Infection in a Five-year Old Boy: A Case Report.

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    Urganci, N; Kilicaslan, O; Kalyoncu, D; Yilmaz, S

    2014-09-01

    Acute hepatitis A infection is usually a self-limiting disease and mostly asymptomatic in children younger than six years old. Extrahepatic autoimmune manifestations such as immune thrombocytopenic purpura (ITP) have been reported rarely in children with acute hepatitis A infection. We report herein a paediatric case with ITP which is due to hepatitis A virus infection.

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

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    Enver Ay

    2012-03-01

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

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

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    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.

  5. Dentoalveolar trauma in a patient with chronic idiopathic thrombocytopenic purpura: a case report.

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    Finucane, David; Fleming, Padraig; Smith, Owen

    2004-01-01

    A case is presented of a 13-year-old boy with chronic idiopathic thrombocytopenic purpura (ITP) who sustained traumatic labial luxation of both lower central incisors, with partial alveolar fracture resulting in displacement of the labial alveolar plate. Intravenous immunoglobulin (Fleibogamma, 1 g/kg body weight x 2 days) was administered, resulting in the patient's platelet count rising from 15,000/mm3 to 70,000/mm3. Under general anesthesia, the displaced lower labial alveolus and luxated teeth were repositioned and splinted 2 days following trauma. Healing was uneventful. Subsequently, both lower central incisors became nonvital and were endodontically treated. The dental treatment of this patient with ITP is discussed in terms of emergency management, and subsequent care.

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

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    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.

  7. Rotavirus-associated immune thrombocytopenic purpura in children: A retrospective study.

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    Ai, Qi; Yin, Jing; Chen, Sen; Qiao, Lijin; Luo, Na

    2016-10-01

    Certain studies have previously indicated that an association may exist between rotavirus infection and primary immune thrombocytopenic purpura (ITP). The present retrospective study aimed to investigate whether rotavirus may cause ITP in children. Firstly, the incidence of ITP in children with or without rotavirus diarrhea was compared. A 14.58% incident rate was observed in children with rotavirus diarrhea compared with a 7.22% incident rate in children without rotavirus diarrhea. Subsequently, the clinical features of ITP children with or without rotavirus infection were compared. The results indicated that ITP children with rotavirus infection were significantly younger, showed significantly decreased mean platelet volume (MPV) levels and presented a significantly higher frequency of bleeding score of 3 against ITP children without rotavirus infection. In conclusion, these findings suggest that rotavirus serves a causative role in ITP.

  8. Role of Helicobacter pylori Eradication Therapy on Platelet Recovery in Chronic Immune Thrombocytopenic Purpura

    Science.gov (United States)

    Sheema, Khan; Arshi, Naz; Farah, Naz; Imran, Sheikh

    2017-01-01

    Background. Idiopathic thrombocytopenic purpura (ITP) is a bleeding disorder in which the immune system destroys native platelets. In this condition an autoantibody is generated against a platelet antigen. ITP affects women more often than men and is more common in children than adults. Objective. To assess the effect of Helicobacter pylori eradication therapy (HPET) on platelet count in Helicobacter pylori associated chronic immune thrombocytopenic purpura (chronic ITP) in adult. Materials and Methods. It is an interventional prospective study conducted at Liaquat University of Medical and Health Sciences, Jamshoro, from 2014 to 2015. A set of 85 patients diagnosed with chronic ITP were included in the study via convenient sampling. Patients with platelets count 3 months were selected. They were posed to first-line investigations which comprised complete blood count (CBC) and peripheral blood smear examination followed by second-line tests including bone marrow examination and Helicobacter pylori stool specific antigen (HpSA-EIA). Standard H. pylori eradication therapy was offered and the patients were assessed at regular intervals for 6 months. Results. Of the 85 study patients, 32 (37.6%) were male and 53 (62.3%) were female. Mean ages of H. pylori positive and negative subjects were 43.89 ± 7.06 and 44.75 ± 7.91 years, respectively. Bone marrow examination confirmed the diagnosis and excluded other related BM disorders. H. pylori stool antigen (HpSA) was detected in 34 (40%) patients and hence regarded as H. pylori positive; the rest were negative. Treatment with eradication therapy significantly improved the mean platelet counts from 48.56 ± 21.7 × 109/l to 94.2 ± 26.8 × 109/l. Conclusion. We concluded that the anti-H. pylori eradication therapy improves blood platelet counts in chronic immune thrombocytopenia.

  9. Role of Helicobacter pylori Eradication Therapy on Platelet Recovery in Chronic Immune Thrombocytopenic Purpura

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    Khan Sheema

    2017-01-01

    Full Text Available Background. Idiopathic thrombocytopenic purpura (ITP is a bleeding disorder in which the immune system destroys native platelets. In this condition an autoantibody is generated against a platelet antigen. ITP affects women more often than men and is more common in children than adults. Objective. To assess the effect of Helicobacter pylori eradication therapy (HPET on platelet count in Helicobacter pylori associated chronic immune thrombocytopenic purpura (chronic ITP in adult. Materials and Methods. It is an interventional prospective study conducted at Liaquat University of Medical and Health Sciences, Jamshoro, from 2014 to 2015. A set of 85 patients diagnosed with chronic ITP were included in the study via convenient sampling. Patients with platelets count 3 months were selected. They were posed to first-line investigations which comprised complete blood count (CBC and peripheral blood smear examination followed by second-line tests including bone marrow examination and Helicobacter pylori stool specific antigen (HpSA-EIA. Standard H. pylori eradication therapy was offered and the patients were assessed at regular intervals for 6 months. Results. Of the 85 study patients, 32 (37.6% were male and 53 (62.3% were female. Mean ages of H. pylori positive and negative subjects were 43.89 ± 7.06 and 44.75 ± 7.91 years, respectively. Bone marrow examination confirmed the diagnosis and excluded other related BM disorders. H. pylori stool antigen (HpSA was detected in 34 (40% patients and hence regarded as H. pylori positive; the rest were negative. Treatment with eradication therapy significantly improved the mean platelet counts from 48.56±21.7 × 109/l to 94.2±26.8 × 109/l. Conclusion. We concluded that the anti-H. pylori eradication therapy improves blood platelet counts in chronic immune thrombocytopenia.

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

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    Ilçe HT et al.

    2011-05-01

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

  11. Case of twin pregnancy complicated by idiopathic thrombocytopenic purpura treated with intravenous immunoglobulin: Review of the literature.

    Science.gov (United States)

    Zhao, W X; Yang, X F; Lin, J H

    2017-01-01

    Idiopathic thrombocytopenic purpura (ITP) is an acquired thrombocytopenia without other clear cause of thrombocytopenia. It is not common in a singleton pregnancy and less common in twin pregnancy. We report a 33-year-old ITP pluripara whose first pregnancy was uneventful. She carried twin pregnancy, complicated by recurrent very low platelets, and gave birth to preterm twins. This patient received multiple courses of intravenous immunoglobulin (IVIG) and showed a significant platelet count improvement with IVIG therapy.

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

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    Nayereh Alizadeh

    2014-04-01

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

  13. Platelet destruction in autoimmune thrombocytopenic purpura: kinetics and clearance of indium-111-labeled autologous platelets

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    Stratton, J.R.; Ballem, P.J.; Gernsheimer, T.; Cerqueira, M.; Slichter, S.J.

    1989-05-01

    Using autologous /sup 111/In-labeled platelets, platelet kinetics and the sites of platelet destruction were assessed in 16 normal subjects (13 with and three without spleens), in 17 studies of patients with primary autoimmune thrombocytopenic purpura (AITP), in six studies of patients with secondary AITP, in ten studies of patients with AITP following splenectomy, and in five thrombocytopenic patients with myelodysplastic syndromes. In normal subjects, the spleen accounted for 24 +/- 4% of platelet destruction and the liver for 15 +/- 2%. Untreated patients with primary AITP had increased splenic destruction (40 +/- 14%, p less than 0.001) but not hepatic destruction (13 +/- 5%). Compared with untreated patients, prednisone treated patients did not have significantly different spleen and liver platelet sequestration. Patients with secondary AITP had similar platelet counts, platelet survivals, and increases in splenic destruction of platelets as did patients with primary AITP. In contrast, patients with myelodysplastic syndromes had a normal pattern of platelet destruction. In AITP patients following splenectomy, the five nonresponders all had a marked increase (greater than 45%) in liver destruction compared to five responders (all less than 40%). Among all patients with primary or secondary AITP, there was an inverse relationship between the percent of platelets destroyed in the liver plus spleen and both the platelet count (r = 0.75, p less than 0.001) and the platelet survival (r = 0.86, p less than 0.001). In a stepwise multiple linear regression analysis, total liver plus spleen platelet destruction, the platelet survival and the platelet turnover were all significant independent predictors of the platelet count. Thus platelet destruction is shifted to the spleen in primary and secondary AITP. Failure of splenectomy is associated with a marked elevation in liver destruction.

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

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    João Tadeu Damian Souto Filho

    2011-01-01

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

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

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    matteo molica

    2016-04-01

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

  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. Pregnancy-associated thrombotic thrombocytopenic purpura with anti-centromere antibody-positive Raynaud's syndrome.

    Science.gov (United States)

    Watanabe, Ryu; Shirai, Tsuyoshi; Tajima, Yumi; Ohguchi, Hiroto; Onishi, Yasushi; Fujii, Hiroshi; Takasawa, Naruhiko; Ishii, Tomonori; Harigae, Hideo

    2010-01-01

    Thrombotic thrombocytopenic purpura (TTP), scleroderma renal crisis (SRC), and hemolysis, elevated liver enzyme levels, and a low platelet count (HELLP) syndrome display common symptoms that include microangiopathic hemolytic anemia, thrombocytopenia, and renal failure. Therefore, it is important to distinguish between them because their treatments vary: however, the differential diagnosis is sometimes difficult. We report a 32-year-old woman who was referred to our department for further examination of microangiopathic hemolytic anemia, thrombocytopenia, and a slightly elevated serum creatinine level with anti-centromere antibody-positive Raynaud's syndrome in the early puerperal period. TTP, SRC, and HELLP syndrome were considered in the differential diagnosis, but the measurement of a disintegrin-like metalloprotease with thrombospondin type 1 motifs 13 (ADAMTS 13) activity and its inhibitor level led to the diagnosis of TTP. She was successfully treated by plasma exchange and high-dose prednisolone and angiotensin-converting enzyme inhibitor. If microangiopathic hemolytic anemia and thrombocytopenia are observed in perinatal women or patients with signs of systemic sclerosis, the measurement of ADAMTS13 activity and its inhibitor level are essential for diagnosis and therapeutic choice.

  18. DNMT3B promoter polymorphism and risk of immune thrombocytopenic purpura in pediatric Egyptians.

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    Shaheen, Iman A; Abukhalil, Reham E; Ali, Dina K; Afifi, Rasha A

    2012-10-01

    Idiopathic (immune) thrombocytopenic purpura (ITP) is a heterogeneous clinical disorder characterized by immune-mediated platelet destruction. Epigenetic changes in gene expression, including DNA methylation and histone modifications, might contribute to autoimmunity. Polymorphisms of the DNA methyltransferase 3B (DNMT3B) gene may influence DNMT3B activity on DNA methylation and increase the susceptibility to several diseases. The current study investigated the association between a single nucleotide polymorphism (SNP) in the promoter of DNMT3B gene and the risk for ITP in pediatric Egyptians. DNMT3B SNP was genotyped by PCR-restriction fragment length polymorphism in 71 pediatric ITP patients and 82 healthy controls matched for age and sex. The C/C wild genotype was not detected in ITP patients or in the controls. The frequencies of the T/T and C/T genotypes were 93.9 and 6.1% in the controls and 91.5 and 6.1% in ITP patients, respectively. There was no significant difference in either genotypes or allelic distribution between ITP patients and the controls. In conclusion, this polymorphism was almost equally distributed between ITP patients and the controls. These results demonstrated that this SNP may not be used as a stratification marker to predict the susceptibility to childhood ITP in Egypt.

  19. 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

    2009-06-08

    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.

  20. 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.

  1. 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.

  2. Acute pancreatitis-induced thrombotic thrombocytopenic purpura with recurrent acute pancreatitis.

    Science.gov (United States)

    Fujino, Yasuhisa; Inoue, Yoshihiro; Onodera, Makoto; Kikuchi, Satoshi; Sato, Masayuki; Kojika, Masahiro; Sato, Hisaho; Suzuki, Keijiro; Matsumoto, Masanori

    2016-04-01

    Recent successive reports on acute pancreatitis-induced thrombotic thrombocytopenic purpura (TTP) have revealed that TTP-related microvascular damage is an aggravating factor of acute pancreatitis. Here, we report the case of a 26-year-old man diagnosed with acute pancreatitis due to high alcohol consumption. The patient was unconscious as he had taken an overdose of medication, and presented with fever and renal failure due to acute pancreatitis on admission. Although the pancreatitis subsequently improved, the symptoms were still observed; on the next day, he exhibited hemoglobinuria, anemia, and thrombocytopenia. Moreover, general blood examinations indicated the presence of schistocytes and reduced activity of ADAMTS13 (a disintegrin-like metalloproteinase with thrombospondin type 1 motif 13) to 47 %. Thus, the patient was diagnosed with TTP, and plasma exchange was performed. After the development of TTP, the acute pancreatitis recurred, but a severe pathogenesis was prevented by plasma exchange. Thus, ADAMTS13 activity may be useful for predicting a severe pathogenesis of acute pancreatitis. In ADAMTS13-deficient cases, plasma exchange may be an effective technique for preventing aggravation of acute pancreatitis.

  3. 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

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

    Directory of Open Access Journals (Sweden)

    Alex Shimanovsky

    2016-01-01

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

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

    Directory of Open Access Journals (Sweden)

    Mariaserena Pioli Di Marco

    2013-12-01

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

  6. Rapidly calcified all of multiple intracranial hemorrhages occurred in a patient with Chronic idiopathic thrombocytopenic purpura

    Directory of Open Access Journals (Sweden)

    Seong Rok Han, M.D., Ph.D.

    2016-03-01

    Full Text Available We report a 38-year-old female patient with chronic idiopathic thrombocytopenic purpura (ITP who occurred in rapidly calcified all of multiple intracranial hemorrhage (ICH lesions. The patient was admitted with poor oral intake and confused mentality. Neurologic examination revealed drowsy consciousness but no motor weakness. She had been diagnosed ITP 5 years ago, however, she was not taking any medication at the time of presentation. Brain CT demonstrated that multiple ICHs, which were located in left frontal lobe, left temporal lobe and right cerebellar hemisphere. Platelet count was 10,000 cells/mm3. The patient was treated with conservative management, which included corticosteroids and platelet transfusion. Follow-up CT performed 12 days after the admission revealed that multiple ICHs were grossly resolving state. Interestingly, high attenuated lesions were seen all of multiple ICH sites, which were considered calcification. After 30 months after hemorrhage, follow-up brain CT showed prominent calcification of all of previous multiple ICH lesions. The patient was well- being state.

  7. Relapsing or refractory idiopathic thrombotic thrombocytopenic purpura-hemolytic uremic syndrome: the role of rituximab.

    Science.gov (United States)

    Caramazza, Domenica; Quintini, Gerlando; Abbene, Ignazio; Malato, Alessandra; Saccullo, Giorgia; Lo Coco, Lucio; Di Trapani, Rosa; Palazzolo, Roberto; Barone, Rita; Mazzola, Giuseppina; Rizzo, Sergio; Ragonese, Paolo; Aridon, Paolo; Abbadessa, Vincenzo; Siragusa, Sergio

    2010-12-01

    Idiopathic thrombotic thrombocytopenic purpura-hemolytic uremic syndrome (TTP-HUS) is a rare disease responsive to treatment with plasma exchange (PE) but with a high percentage of relapse or refractory patients. A severe deficiency of ADAMTS-13 (<5% of normal activity), congenital or caused by an autoantibody, may be specific for TTP and it has been proposed that severe ADAMTS-13 deficiency now defines TTP. B cells play a key role in both the development and the perpetuation of autoimmunity, suggesting that B-cell depletion could be a valuable treatment approach for patients with idiopathic TTP-HUS. This review of the literature focuses on the role of rituximab, a chimeric monoclonal antibody directed against CD20 antigen expressed by B lymphocytes, in patients with relapsing or refractory TTP-HUS with or without ADAMTS-13 deficiency, suggesting that rituximab may produce clinical remission in a significant proportion of patients. Rituximab therapy reduces plasma requirement and avoids complications related to salvage-immunosuppressive therapy. In conclusion, rituximab provides an effective, well-tolerated, and safe treatment option for patients with idiopathic TTP-HUS, thus giving an alternative approach to the current treatment based on PE.

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

    Institute of Scientific and Technical Information of China (English)

    卢燕

    2001-01-01

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

  9. Bleeding tendency and platelet function during treatment with romiplostim in children with severe immune thrombocytopenic purpura.

    Science.gov (United States)

    Suntsova, Elena V; Demina, Irina M; Ignatova, Anastasia A; Ershov, Nikolay M; Trubina, Natalia M; Dobrynina, Juliya; Serkova, Irina V; Supik, Zhanna S; Orekhova, Ekaterina V; Hachatryan, Lili A; Kotskaya, Natalia N; Pshonkin, Aleksey V; Maschan, Aleksey A; Novichkova, Galina A; Panteleev, Mikhail A

    2017-03-07

    It has been suggested that platelet function in chronic immune thrombocytopenic purpura (ITP) may be abnormal. Thrombopoietin mimetics used for treatment can affect it, but the data remain limited. We investigated platelet function of 20 children diagnosed with severe ITP (aged 1-16 years, 12 females and eight males). Platelet functional activity in whole blood was characterized by flow cytometry before and after stimulation with SFLLRN plus collagen-related peptide. Levels of CD42b, PAC1, and CD62P, but not CD61 or annexin V, were significantly increased (P < 0.05) in resting platelets of patients before treatment compared with healthy donors. On average, PAC1 and CD62P in patients after activation were also significantly elevated, although some patients failed to activate integrins. Romiplostim (1-15 μg/kg/week s.c.) was prescribed to seven patients, with clinical improvement in six. Interestingly, one patient had clinical improvement without platelet count increase. Eltrombopag (25-75 mg/day p.o.) was given to four patients, with positive response in one. Others switched to romiplostim, with one stable positive response, one unstable positive response, and one non-responding. Platelet quality improved with romiplostim treatment, and their parameters approached the normal values. Our results suggest that platelets in children with severe ITP are pre-activated and abnormal, but improve with treatment.

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

    Science.gov (United States)

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

    2016-01-01

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

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

    Directory of Open Access Journals (Sweden)

    Suella Martino

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

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

    DEFF Research Database (Denmark)

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

    2013-01-01

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

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

    Science.gov (United States)

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

    2013-04-01

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

  14. Autoimmune hepatitis-primary biliary cirrhosis overlap syndrome concomitant with immune hemolytic anemia and immune thrombocytopenic purpura (Evans syndrome).

    Science.gov (United States)

    Korkmaz, Huseyin; Bugdaci, Mehmet Sait; Temel, Tuncer; Dagli, Mehmet; Karabagli, Pinar

    2013-04-01

    Autoimmune hepatitis (AIH) and primary biliary cirrhosis (PBC) associated with Evans syndrome; combination of autoimmune hemolytic anemia (AIHA) and immune thrombocytopenic purpura (ITP) has rarely been reported. We report the case of a 53-year-old patient who presented with weakness, myalgia, arthralgia, shortness of breath and purpura. Initial laboratory investigations revealed liver dysfunction, anemia and thrombocytopenia. Anti-nuclear (ANA) and antimitochondrial M2 (AMA M2) antibodies were positive. Diagnose of PBC-AIH overlap was made by clinical, serological and histological investigations. AIHA and ITP was identified with clinical-laboratory findings and bone marrow puncture. She was treated with IVIG followed by prednisolone and ursodeoxycholic acid. Hemoglobin-thrombocytes increased rapidly and transaminases improved at day 8. We have reported the first case in the literature with AIH-PBC overlap syndrome concurrent by ITP and AIHA which suggest the presence of shared genetic susceptibility factors in multiple autoimmune conditions including AIH, PBC, ITP and AIHA.

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

    Institute of Scientific and Technical Information of China (English)

    高维强; 苏健; 白霞; 王兆钺; 阮长耿

    2004-01-01

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

  16. Mycophenolate mofetil (MMF) for the treatment of steroid-resistant idiopathic thrombocytopenic purpura.

    Science.gov (United States)

    Hou, Ming; Peng, Jun; Shi, Yan; Zhang, Chunqing; Qin, Ping; Zhao, Chuanli; Ji, Xuebin; Wang, Xueyong; Zhang, Maohong

    2003-06-01

    The treatment of chronic idiopathic thrombocytopenic purpura (ITP) is difficult in those unresponsive to corticosteroids and/or splenectomy. We attempted to induce durable response in 21 patients with refractory ITP by applying mycophenolate mofetil (MMF) (1.5-2.0 g/d), a novel immunosuppressive agent. Overall response rate was 62% (13 of 21), including 24% (five of 21) in complete response (CR), 29% (six of 21) in partial response (PR), and 10% (two of 21) in minor response (MR). The response rates for non-splenectomized and splenectomized ITP patients were 64% (nine of 14) and 57% (four of seven), respectively (P > 0.05). 39% (five of 13) responders relapsed as a result of dose reduction or withdraw of MMF, and 61% (eight of 13) responders maintained their effectiveness for a median of 24 wk. Sustained response was observed in three patients in whom MMF was withdrawn. MMF was well tolerated with only slight nausea and diarrhea recorded in 3 of 21 cases. No premature withdrawal was found in this study. CD3+ peripheral blood mononuclear cells (PBMC) and CD19+ PBMC were significantly reduced 12 wk after MMF administration in the responders. Platelet-associated antibodies against glycoproteins GPIIb/IIIa were detected in 13 of 21 (62%) patients before MMF treatment, and antibody levels were significantly decreased in responders 12 wk after MMF administration. This suggested that MMF might correct the immunologic abnormalities underlying the destruction of circulating platelets in ITP. We conclude that MMF could be used as a second-line agent for the treatment of steroid-resistant ITP before or after splenectomy and thereby is worth of further evaluation in randomized studies.

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

    Science.gov (United States)

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

    2014-06-01

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

  18. Treatment of 37 Patients with Refractory Idiopathic Thrombocytopenic Purpura by Shengxueling(升血灵)

    Institute of Scientific and Technical Information of China (English)

    SHAO Ke-ding; ZHOU Yu-hong; SHEN Yi-ping; YE Bao-dong; GAO Rui-lan; ZHANG Yu

    2007-01-01

    Objective:To explore the clinical effect and possible mechanism of Shengxueling(升血灵,SXL),a Chinese medical preparation mainly consisting of ginseng saponins,in treating refractory idiopathic thrombocytopenic purpura(ITP).Methods:The selected 69 patients with ITP were randomly assigned to two groups,the 37 patients in the treated group were treated orally by SXL with the dose for adult as 60 mg twice a day for two weeks.Then when no marked rise of platelet count after that,the dose would be doubled and administered for another two weeks.Then the dose could be gradually reduced to the initiative level in patients who responded to the treatment,and if they did not,the treatment was regarded as ineffective and be terminated.The 32 patients in the control group were treated with ampeptide elemente instead of SXL,0.4 g each time three times a day in the first two weeks,and,if that was ineffective,0.2 g would be added each time and 1.8 g would be administered a day for two more weeks.Four weeks' treatment was regarded as one therapeutic course for both groups and the observation lasted for two successive courses in patients showing positive response.Results:In the 37 patients in the treated group,markedly effective was obtained in 7(19.0%),favorably effective in 15(40.5%),improved in 5(13.5%) and ineffective in 10(27.0%),the total effective rate being 59.5%.The corresponding number in the 32 patients in the control group was 4(12.5%),6(18.8%),3(9.4%),19(59.4%)and 31.3% respectively.Comparison showed the difference in therapeutic efficacy between the two groups was significant(P<0.05).Conclusion:SXL is a safe and effective preparation for treatment of ITP,showing an immediate effect which is obviously superior to that of ampeptide elemente with less adverse effect.

  19. Opana ER abuse and thrombotic thrombocytopenic purpura (TTP)-like illness: a rising risk factor in illicit drug users.

    Science.gov (United States)

    Kapila, Aaysha; Chhabra, Lovely; Chaubey, Vinod K; Summers, Jeffery

    2014-03-03

    We report the case of a 22 year-old-woman who presented with upper extremity cellulitis secondary to an infiltration of illicit intravenous drug use. She confessed to the intravenous use of Opana ER (an extended release oral formulation of oxymorphone) which is an opioid drug approved only for oral use. She was found to have clinical evidence of profound thrombotic microangiopathy which resulted due to the intravenous use of Opana ER. She showed full clinical improvement after withholding drug and supportive clinical care. Recent report of Opana ER intravenous abuse was published from Tennessee county and has now been increasingly recognised as one of the causes of thrombocytopenia which mimicks clinically as thrombotic thrombocytopenic purpura. Physicians should be aware of this association as the lack of familiarity to this can pose serious management dilemmas for our patients (especially the polysubstance abusers).

  20. Multidrug resistance-1 in T lymphocytes and natural killer cells of adults with idiopathic thrombocytopenic purpura: effect of prednisone treatment.

    Science.gov (United States)

    López-Karpovitch, Xavier; Graue, Gerardo; Crespo-Solís, Erick; Piedras, Josefa

    2008-07-01

    High P-glycoprotein-mediated multidrug resistance-1 (P-gp/MDR1) activity in lymphocytes from idiopathic thrombocytopenic purpura (ITP) patients may affect disease outcome. ITP treatment includes glucocorticoids that are substrates of P-gp; hence, P-gp functional activity and antigenic expression were assessed by flow cytometry in T and natural killer (NK) cells from ITP patients before and after prednisone therapy. Herein, patients' T and NK cells did not show increased MDR1 functional activity, whereas P-gp antigenic expression was significantly enhanced in both therapy-free and prednisone-treated patients. Prednisone treatment did not significantly modify the function and expression of MDR1 in T and NK cells of ITP patients.

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

    Institute of Scientific and Technical Information of China (English)

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

    2005-01-01

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

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

    Science.gov (United States)

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

    2012-06-01

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

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

    Science.gov (United States)

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

    2014-04-01

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

  4. Idiopathic combined, autoantibody-mediated ADAMTS-13/factor H deficiency in thrombotic thrombocytopenic purpura-hemolytic uremic syndrome in a 17-year-old woman: a case report

    Directory of Open Access Journals (Sweden)

    Patschan Daniel

    2011-12-01

    Full Text Available Abstract Introduction Thrombotic thrombocytopenic purpura-hemolytic uremic syndrome is a life-threatening condition with various etiopathogeneses. Without therapy approximately 90% of all patients die from the disease. Case presentation We report the case of a 17-year-old Caucasian woman with widespread hematomas and headache. Due to hemolytic anemia, thrombocytopenia, and schistocytosis, thrombotic thrombocytopenic purpura-hemolytic uremic syndrome was suspected and plasma exchange therapy was initiated immediately. Since her thrombocyte level did not increase during the first week of therapy, plasma treatment had to be intensified to a twice-daily schedule. Further diagnostics showed markedly reduced activities of both ADAMTS-13 (a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13 - also known as von Willebrand factor-cleaving protease and factor H. Test results for antibodies against both proteins were positive. While plasma exchange therapy was continued, rituximab was given once weekly for four consecutive weeks. After the last dose, thrombocytes and activities of ADAMTS-13 and factor H increased into the normal range. Our patient improved and was discharged from the hospital. Conclusions Since no clinical symptoms/laboratory findings indicated a malignant or specific autoimmune-mediated disorder, the diagnosis made was thrombotic thrombocytopenic purpura-hemolytic uremic syndrome due to idiopathic combined, autoantibody-mediated ADAMTS-13/factor H deficiency.

  5. Treatment of plasmapheresis refractory thrombotic thrombocytopenic purpura with double-filtration membrane plasmapheresis.

    Science.gov (United States)

    Karakus, Volkan; Deveci, Burak; Kurtoğlu, Erdal

    2013-06-01

    Thrombotic thrombocytic purpura (TTP) is a life-threatening disorder. Without plasma exchange treatment (PET) the mortality rate is quite high. Double-filtration plasmapheresis is an alternative opportunity for TTP patients refractory to PET. Here we report our experience in a refractory TTP patient who was successfully treated by means of double-filtration plasmapheresis therapy.

  6. DNMT3B 579G>T promoter polymorphism and the risk for idiopathic thrombocytopenic purpura in a Chinese population.

    Science.gov (United States)

    Zhao, Haifeng; Du, Weiting; Gu, Dongsheng; Wang, Donghai; Xue, Feng; Ge, Jing; Sui, Tao; Yang, Renchi

    2009-01-01

    Epigenetics may influence the expression of numerous genes, which might contribute to autoimmune diseases. DNA methylation is mediated by DNA methyltransferases, especially DNA methyltransferase 3B (DNMT3B). Polymorphisms of the DNMT3B gene may influence DNMT3B activity on DNA methylation and increase the susceptibility to several diseases. The current study investigated the association between DNMT3B 579G>T and the risk for idiopathic thrombocytopenic purpura (ITP). The DNMT3B 579G>T polymorphisms were analyzed by PCR-RFLP. There was no significant difference in genotype and allele distribution between the ITP patient and the controls (p = 0.722 and 0.667, respectively). Similar results were observed between the 2 groups when stratified by age and disease course, including acute in childhood, chronic in childhood, acute in adult and chronic in adult. Importantly, this study showed a statistical difference in the distribution of SNP of DNMT3B between Chinese and Koreans or Americans. It is shown that the SNP of DNMT3B 579G>T may not be used on its own as a marker to predict the susceptibility to ITP in a Chinese population and that DNMT3B 579G>T promoter SNP varies from one ethnic population to another.

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

    Directory of Open Access Journals (Sweden)

    Işın Yaprak

    2010-09-01

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

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

    Energy Technology Data Exchange (ETDEWEB)

    Savolainen, S.

    1992-01-01

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

  9. Effect of Protein Kinase C on Proliferation and Apoptosis of T Lymphocytes in Idiopathic Thrombocytopenic Purpura Children

    Institute of Scientific and Technical Information of China (English)

    Changlin Wu; Fang Liu; Xuemin Zhou; Zhengwei Cheng; Xiaomeng Yang; Hong Xiao; Qun Chen; Kangrong Cai

    2005-01-01

    It is well-documented that T lymphocyte proliferation and apoptosis are abnormal in idiopathic thrombocytopenic purpura (ITP) children. However, the underlying regulation mechanisms especially in terms of signal transduction remain unknown. In this paper, we reported the changes of protein kinase C (PKC) activity in peripheral blood T lymphocytes and the effect of PKC on T lymphocyte proliferation and apoptosis. We demonstrated that in ITP children, the activator (PMA) and inhibitor (H-7) of PKC affected on T lymphocyte proliferation and apoptosis dramatically, but they altered little in healthy children. PKC activity was significantly enhanced in ITP children together with an increased expression of FasL on CD3+ T, CD4+ T and CD8+T cells, resulting in a positive correlation between PKC activity and the expression of FasL on T cells. While the PKC activity and the platelet count were negatively correlated. Taken together, our findings suggest that the PKC activation may enhance T lymphocytes activity, suppress T cell apoptosis and be involve in thrombocytes damage as a mechanism related to immune pathogenesis of ITP.

  10. Level of IL-16 and Reticulated Platelets Percentage during the Clinical Course of Immune Thrombocytopenic Purpura in Children.

    Science.gov (United States)

    Abd El-Glil, Reem R; Assar, Effat H

    2015-01-01

    Immune thrombocytopenic purpura (ITP) is an immune-mediated acquired disease with transient or persistent decrease of thrombocytes number in the blood. Cytokines play important roles in the immune regulation and are known to be deregulated in autoimmune diseases. This study aimed to investigate serum IL-16 levels in relation to reticulated platelets in children with ITP and platelet count. Twenty six children with ITP (11 with newly diagnosed ITP, 9 with persistent ITP and 6 with chronic ITP) and 12 age-matched healthy children controls were studied. Serum level of IL-16 and reticulated platelets count were assessed by Enzyme Linked Immunosorbent Assay (ELISA) and flow cytometry respectively. Serum IL-16 levels were significantly higher in patients as compared to controls (P platelets were also elevated in patients compared to controls and the increase was significant in newly diagnosed group (P platelets and platelets counts (r = -0.284, P = 0.028, r = 0.274 P = 0.25) respectively. It is concluded that IL-16 may be valuable in predicting the clinical course of pediatrics ITP. Measurement of reticulated platelets may provide significant information about thrombopoietic activity during the clinical course of ITP in children.

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

    Science.gov (United States)

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

    2012-07-01

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

  12. 儿童特发性血小板减少性紫癜急性转慢性危险因素分析%Risk factors of acute idiopathic thrombocytopenic purpura developing into chronic idiopathic thrombocytopenic purpura

    Institute of Scientific and Technical Information of China (English)

    王缉干; 罗建明

    2012-01-01

    目的 探讨影响小儿急性特发性血小板减少性紫癜(AITP)发展成慢性特发性血小板减少性紫癜(CITP)的危险因素.方法 选择2006年5月至2010年4月于广西医科大学一附院诊断AITP住院患儿138例,对患儿临床表现、实验室检查、治疗方案等16个相关因素分别进行单因素成组对照研究.对有意义的单因素,再运用非条件Logistic多因素回归模型分析,以期找到有意义的因素.结果 病程(患儿起病至治疗时间)、ORh(D)+型血、用含有大剂量丙种球蛋白的治疗方案、血小板上升正常时间都是影响AITP患儿转为慢性的主要因素,而与发病年龄、治疗初用血小板、血小板开始回升时间、性别、有无前驱显性感染、治疗前血小板数、血小板平均体积、血小板平均分布系数、骨髓巨核细胞数、幼稚巨核细胞数、颗粒型巨核细胞数、有无幼稚淋巴细胞数无关.结论 AITP患儿早期治疗时用有大剂量丙种球蛋白的治疗方案是改善预后的关键,治疗时血小板回升正常时间晚、ORh(D)+血型(相对于A和B血型)是转为慢性的高危因素.%Objectives To investigate the risk factors for children with acute idiopathic thrombocytopenic purpura (AITP) developing into chronic idiopathic thrombocytopenic purpura (CITP). Methods A total of 138 patients with AITP from May 2006 to April 2010 in the First Affiliated Hospital of Guangxi Medical University were Selected and telephoned for retrospective analysis. The 16 related factors, including clinical presentation, laboratory tests and treatment programs, were performed single case-control study. For those significant single factors, multi-factor non-conditional Logistic regression analysis was used to find meaningful factors. Results The main influencing factors were the onset-to-treatment time,0 Rh (D) + type blood, treatment with gamma globulin and platelets increasing to normal time. The age of onset, early treatment

  13. 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.

  14. Thrombotic thrombocytopenic purpura secondary to ABO group incompatible blood transfusion in a patient after cardiac surgery

    Directory of Open Access Journals (Sweden)

    Yalcin Solak

    2013-01-01

    Full Text Available The triggers of secondary thrombotic thrombopcytopenic purpura (TTP include drug toxicity, radiation and high-dose chemotherapy, angioinvasive infections, surgery and acute graft versus host disease. TTP secondary to surgery have been reported in a number of cases. Most of the cases have been occurred after open heart surgery. Extensive endothelial damage is held responsible as the initiating mechanism in postoperative TTP cases. However, there is no report of secondary TTP describing development owing to ABO incompatible blood transfusion. Here, we describe a patient who developed TTP after transfusion of ABO incompatible blood during hospitalization for bypass surgery. We also propose a hypothesis which may account for the possible underlying mechanism.

  15. The etiology of childhood immune thrombocytopenic purpura: how complex is it?

    Science.gov (United States)

    Chanock, Stephen

    2003-12-01

    Recent developments in genomics and basic immunology have provided a new set of tools for investigation into the etiology and treatment of childhood immune thrombocytopenia purpura (ITP). The genomic revolution is generating a catalog of germ-line common genetic variants, some of which could influence the susceptibility or outcome of ITP. Similarly, in vitro analyses and animal models have been employed to probe the basic alterations underlying ITP. The emergence of a more refined understanding of complex diseases such as ITP has important implications for prevention, therapy, and follow-up. The relative contribution of the genetic component and its interaction with the strong environmental stimulus, such as an acute, antecedent viral infection, remains to be determined.

  16. 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)

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

    Institute of Scientific and Technical Information of China (English)

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

    2008-01-01

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

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

    Directory of Open Access Journals (Sweden)

    Frederico Figueiredo Amâncio

    2014-06-01

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

  19. 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.

  20. Kinetics and sites of destruction of /sup 111/In-oxine-labeled platelets in idiopathic thrombocytopenic purpura: a quantitative study

    Energy Technology Data Exchange (ETDEWEB)

    Heyns, A.D.; Loetter, M.G.; Badenhorst, P.N.; de Kock, F.; Pieters, H.; Herbst, C.; van Reenen, O.R.; Kotze, H.; Minnaar, P.C.

    1982-04-01

    Kinetics and quantification of the sites of destruction of /sup 111/In-oxine-labeled autologous platelets were investigated in eight patients with idiopathic thrombocytopenic purpura. The mean platelet count was 17 +/- 9 X 10(9)/liter; platelets were separated by differential centrifugation and labeled with 5.6 +/- 2.5 MBq /sup 111/In. Whole body and organ /sup 111/In-platelet distribution was quantitated with a scintillation camera and a computer-assisted imaging system acquisition matrix. Areas of interest were selected with the computer and organ /sup 111/In-radioactivity expressed as a percentage of whole body activity. Mean platelet survival was 49.5 +/- 29.6 hr and the survival curves were exponential. Equilibrium percentage organ /sup 111/In-radioactivity was (normal values in parentheses): spleen 33.7 +/- 8.8 (31.1 +/- 10.2); liver 16.1 +/- 9.5 (13.1 +/- 1.3); thorax 22.8 +/- 3.7 (28.2 +/- 5.6). Percentage organ /sup 111/In-activity at the time when labeled platelets had disappeared from the circulation was: spleen 44.5 +/- 16.4 (40 +/- 16); liver 16.0 +/- 11.5 (32.4 +/- 7.2); thorax 19.7 +/- 6.0 (17.7 +/- 10.3). Thorax activity corresponds to bone marrow radioactivity. Three patterns of platelet sequestration were evident. Three patients had mainly splenic sequestration, two mainly hepatic sequestration, and three diffuse reticuloendothelial system sequestration with a major component of platelets destroyed in the bone marrow. Splenectomy was performed in two patients. The pattern of /sup 111/In-platelet sequestration was not predictive of response of glucocorticoid therapy or indicative of the necessity for splenectomy. Quantitative /sup 111/In-labeled autologous platelet kinetic studies provide a new tool for the investigation of platelet disorders.U

  1. Nursing experience of idiopathic thrombocytopenic purpura%特发性血小板减少性紫癜的护理体会

    Institute of Scientific and Technical Information of China (English)

    袁玖莲

    2015-01-01

    目的:本文通过回顾性分析56例特发性血小板减少性紫癜患者的临床资料,阐述了本病的心理护理、病情观察、出血的护理、用药护理、饮食护理、健康教育等护理措施。对特发性血小板减少性紫癜患者采取综合性的护理干预措施,可以有效地提高临床治疗效果,降低并发症的发生率,降低死亡率。%In this paper,through retrospectively analyzing the clinical datas of 56 patients with idiopathic thrombocytopenic purpura,the author expounded the mental nursing,observation,bleeding nursing,drug nursing,diet nursing and health education. Comprehensive nursing intervention measures for patients with idiopathic thrombocytopenic purpura can effectively improve the clinical treatment effect,reduce the incidence of complications and mortality.

  2. A rare association of localized scleroderma type morphea, vitiligo, autoimmune hypothyroidism, pneumonitis, autoimmune thrombocytopenic purpura and central nervous system vasculitis. Case report

    Directory of Open Access Journals (Sweden)

    Bonilla-Abadía Fabio

    2012-12-01

    Full Text Available Abstract Background The localized scleroderma (LS known as morphea, presents a variety of clinical manifestations that can include systemic involvement. Current classification schemes divide morphea into categories based solely on cutaneous morphology, without reference to systemic disease or autoimmune phenomena. This classification is likely incomplete. Autoimmune phenomena such as vitiligo and Hashimoto thyroiditis associated with LS have been reported in some cases suggesting an autoimmune basis. To our knowledge this is the first case of a morphea forming part of a multiple autoimmune syndrome (MAS and presenting simultaneously with autoimmune thrombocytopenic purpura and central nervous system vasculitis. Case presentation We report an uncommon case of a white 53 year old female patient with LS as part of a multiple autoimmune syndrome associated with pneumonitis, autoimmune thrombocytopenic purpura and central nervous system vasculitis presenting a favorable response with thrombopoietin receptor agonists, pulses of methylprednisolone and cyclophosphamide. Conclusion Is likely that LS have an autoimmune origin and in this case becomes part of MAS, which consist on the presence of three or more well-defined autoimmune diseases in a single patient.

  3. Systemic Lupus Erythematosus Presenting as Refractory Thrombotic Thrombocytopenic Purpura: A Diagnostic and Management Challenge. A Case Report and Concise Review of the Literature

    Science.gov (United States)

    Abu-Hishmeh, Mohammad; Sattar, Alamgir; Zarlasht, Fnu; Ramadan, Mohamed; Abdel-Rahman, Aisha; Hinson, Shante; Hwang, Caroline

    2016-01-01

    Patient: Female, 34 Final Diagnosis: Refractory thrombotic thrombocytopenic purpura Symptoms: Fatigue Medication: — Clinical Procedure: Plasma exchange Specialty: Rheumatology • Hematology and Critical Care Objective: Rare co-existance of disease or pathology Background: Thrombotic thrombocytopenic purpura (TTP) is one of the thrombotic microangiopathic (TMA) syndromes, caused by severely reduced activity of the vWF-cleaving protease ADAMTS13. Systemic lupus erythematosus (SLE), on the other hand, is an autoimmune disease that affects various organs in the body, including the hematopoietic system. SLE can present with TMA, and differentiating between SLE and TTP in those cases can be very challenging, particularly in patients with no prior history of SLE. Furthermore, an association between these 2 diseases has been described in the literature, with most of the TTP cases occurring after the diagnosis of SLE. In rare cases, TTP may precede the diagnosis of SLE or occur concurrently. Case Report: We present a case of a previously healthy 34-year-old female who presented with dizziness and flu-like symptoms and was found to have thrombocytopenia, hemolytic anemia, and schistocytes in the peripheral smear. She was subsequently diagnosed with TTP and started on plasmapheresis and high-dose steroids, but without a sustained response. A diagnosis of refractory TTP was made, and she was transferred to our facility for further management. Initially, the patient was started on rituximab, but her condition continued to deteriorate, with worsening thrombocytopenia. Later, she also fulfilled the Systemic Lupus International Collaborating Clinics (SLICC) criteria for diagnosis of SLE. Treatment of TTP in SLE patients is generally similar to that in the general population, but in refractory cases there are few reports in the literature that show the efficacy of cyclophosphamide. We started our patient on cyclophosphamide and noticed a sustained improvement in the platelet

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

    Science.gov (United States)

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

    2011-12-01

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

  5. Idiopathic thrombocytopenic purpura (ITP)

    Science.gov (United States)

    Blood tests will be done to check your platelet count . A bone marrow aspiration or biopsy may also be done. ... the disease usually goes away without treatment. Some children ... the platelet count in about half of people. However, other drug ...

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

    Science.gov (United States)

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

    2015-01-01

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

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

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    George James N

    2008-02-01

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

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

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    Sherif Ali Eltawansy

    2014-01-01

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

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

    Science.gov (United States)

    Khorshied, Mervat Mamdooh; El-Ghamrawy, Mona Kamal

    2012-12-10

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

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

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    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.

  11. Efficacy of a rituximab regimen based on B cell depletion in thrombotic thrombocytopenic purpura with suboptimal response to standard treatment: Results of a phase II, multicenter noncomparative study.

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    Benhamou, Ygal; Paintaud, Gilles; Azoulay, Elie; Poullin, Pascale; Galicier, Lionel; Desvignes, Céline; Baudel, Jean-Luc; Peltier, Julie; Mira, Jean-Paul; Pène, Frédéric; Presne, Claire; Saheb, Samir; Deligny, Christophe; Rousseau, Alexandra; Féger, Frédéric; Veyradier, Agnès; Coppo, Paul

    2016-12-01

    The standard four-rituximab infusions treatment in acquired thrombotic thrombocytopenic purpura (TTP) remains empirical. Peripheral B cell depletion is correlated with the decrease in serum concentrations of anti-ADAMTS13 and associated with clinical response. To assess the efficacy of a rituximab regimen based on B cell depletion, 24 TTP patients were enrolled in this prospective multicentre single arm phase II study and then compared to patients from a previous study. Patients with a suboptimal response to a plasma exchange-based regimen received two infusions of rituximab 375 mg m(-2) within 4 days, and a third dose at day +15 of the first infusion if peripheral B cells were still detectable. Primary endpoint was the assessment of the time required to platelet count recovery from the first plasma exchange. Three patients died after the first rituximab administration. In the remaining patients, the B cell-driven treatment hastened remission and ADAMTS13 activity recovery as a result of rapid anti-ADAMTS13 depletion in a similar manner to the standard four-rituximab infusions schedule. The 1-year relapse-free survival was also comparable between both groups. A rituximab regimen based on B cell depletion is feasible and provides comparable results than with the four-rituximab infusions schedule. This regimen could represent a new standard in TTP. This trial was registered at www.clinicaltrials.gov (NCT00907751). Am. J. Hematol. 91:1246-1251, 2016. © 2016 Wiley Periodicals, Inc.

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

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    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.

  13. Thrombotic thrombocytopenic purpura misdiagnosed as autoimmune cytopenia: Causes of diagnostic errors and consequence on outcome. Experience of the French thrombotic microangiopathies reference centre.

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    Grall, Maximilien; Azoulay, Elie; Galicier, Lionel; Provôt, François; Wynckel, Alain; Poullin, Pascale; Grange, Steven; Halimi, Jean-Michel; Lautrette, Alexandre; Delmas, Yahsou; Presne, Claire; Hamidou, Mohamed; Girault, Stéphane; Pène, Frédéric; Perez, Pierre; Kanouni, Tarik; Seguin, Amélie; Mousson, Christiane; Chauveau, Dominique; Ojeda-Uribe, Mario; Barbay, Virginie; Veyradier, Agnès; Coppo, Paul; Benhamou, Ygal

    2017-01-30

    Thrombotic thrombocytopenic purpura (TTP) has a devastating prognosis without adapted management. Sources of misdiagnosis need to be identified to avoid delayed treatment. We studied 84 patients with a final diagnosis of severe (<10%) acquired ADAMTS13 deficiency-associated TTP from our National database that included 423 patients, who had an initial misdiagnosis (20% of all TTP). Main diagnostic errors were attributed to autoimmune thrombocytopenia, associated (51%) or not (37%) with autoimmune hemolytic anemia. At admission, misdiagnosed patients were more frequently females (P = .034) with a history of autoimmune disorder (P = .017) and had organ involvement in 67% of cases; they had more frequently antinuclear antibodies (P = .035), a low/undetectable schistocyte count (P = .001), a less profound anemia (P = .008), and a positive direct antiglobulin test (DAT) (P = .008). In multivariate analysis, female gender (P = .022), hemoglobin level (P = .028), a positive DAT (P = .004), and a low schistocytes count on diagnosis (P < .001) were retained as risk factors of misdiagnosis. Platelet count recovery was significantly longer in the misdiagnosed group (P = .041) without consequence on mortality, exacerbation and relapse. However, patients in the misdiagnosed group had a less severe disease than those in the accurately diagnosed group, as evidenced by less organ involvement at TTP diagnosis (P = .006). TTP is frequently misdiagnosed with autoimmune cytopenias. A low schistocyte count and a positive DAT should not systematically rule out TTP, especially when associated with organ failure.

  14. 服用阿莫西林母亲哺乳的婴儿出现血小板减少性紫癜%Thrombocytopenic purpura in a infant breast-fed by a mother receiving amoxicillin

    Institute of Scientific and Technical Information of China (English)

    陈月; 谢黎崖

    2011-01-01

    1例56 d男婴因血小板减少性紫癜入院.入院前2d其母行输卵管结扎手术,术后口服阿莫西林预防感染,服药期间未停止哺乳.服药第2天晚患儿下肢皮肤出现散在针尖大小红点,第3天左下肢出现瘀斑.入院时血小板4×109/L,经骨髓细胞学检查,确诊为血小板减少性紫癜.停止哺乳,予以支持治疗,2周后患儿治愈出院.%A 56-day-old male infant was hospitalized with thrombocytopenic purpura. Two days before admission, his mother underwent tubal ligation surgery and received oral amoxicillin to prevent infections after surgery. Breast-feeding was not stopped during the period of drug use. On day 2 of drug therapy, the infant developed scattered pin-sized red spots on his lower leg skin and, on day 3, petechia appeared on his lower left leg. On admission, his PLT level was 4 x 10VL, thrombocytopenic purpura was confirmed by bone marrow cytological analysis. Breast-feeding was stopped and the infant was given supportive treatments. Two weeks later, he recovered and was discharged.

  15. Púrpura trombocitopênica imunológica como manifestação inicial de lúpus eritematoso sistêmico juvenil Idiopathic thrombocytopenic purpura as initial manifestation of juvenile systemic lupus erythematosus

    Directory of Open Access Journals (Sweden)

    Josefina Aparecida Pellegrini Braga

    2003-12-01

    salientar a importância da determinação de auto-anticorpos para lúpus eritematoso sistêmico nas crianças com a forma crônica desta patologia.Patients with Idiopathic Thrombocytopenic Purpura (ITP present a high trend to develop Systemic Lupus Erythematosus (SLE, especially those with chronic presentation. Some authors observed that female gender, older patients and familial history of autoimmune disease in patients with Idiopathic Thrombocytopenic Purpura are factors that lead to increased susceptibility for the development of Systemic Lupus Erythematosus. Based on these facts, we decided to study 5 children with chronic Idiopathic Thrombocytopenic Purpura and late Systemic Lupus Erythematosus. In this paper, we describe the clinical and laboratorial features of 5 female children with Idiopathic Thrombocytopenic Purpura that later developed Systemic Lupus Erythematosus. All patients were girls, 3 Caucasian, with Idiopathic Thrombocytopenic Purpura onset age ranged between 6 years and 3 months and 12 years and 1 month (mean - 9 years and 2 months. The age at Systemic Lupus Erythematosus diagnosis ranged between 8 years and 13 years and 8 months (mean - 10 years and 10 months. Though, the gap between Idiopathic Thrombocytopenic Purpura and Systemic Lupus Erythematosus diagnosis ranged between 11 months and 2 years and 9 months (mean - 1 year and 10 months. All patients presented chronic Idiopathic Thrombocytopenic Purpura (thrombocytopenia lasts longer than 6 months. The Systemic Lupus Erythematosus classification criteria were (in decreasing frequency: malar erythema and positivity of ANA in 5 patients; arthritis, hematological (thrombocytopenia and immunological alterations (positivity of anti-DNA in 4 patients; photosensitivity and positivity of anti-cardiolipin in 3 patients. Other manifestations included oral ulcers, renal involvement, leukopenia and auto-immune hemolytic anemia, serositis (pericarditis, neurological involvement and positivity of anti-Sm antibody

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

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    McMillan Robert

    2007-02-01

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

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

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

    2008-01-01

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

  18. The analysis of factors of acute Idiopathic thrombocytopenic purpura conversing to chronic Idiopathic thrombocytopenic purpura in children%儿童特发性血小板减少性紫癜急性转慢性危险因素分析

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    周丹; 杨萍; 田立新; 冯玉兰; 宋丽琼

    2013-01-01

    Objective:To analyze the factors of cute Idiopathic thrombocytopenic purpura (AITP) conversing to chronic Idiopathic thrombocytopenic purpura (CITP). Methods:Choosed 156 children of AITP to analyze by lab, diagnosis and treatment, effect and prognosis. Methods:There were 12 index having difference, among these 2 index were continuous variable (weight, peak platelet). Implement multi-factor analysis of these 12 factors, and screened medical history, age, low count of WBC, weight,high count of marrow megakaryocyte and peak of platelet. Conclusion:Dignosising AITP patients with count of marrow megakaryocyte, peak of platelet to determine prognosis is effective.%目的:分析致使 AITP(即为:儿童特发性急性的血小板减少性紫癜)转变为 CITP(即为:儿童特发性慢性的血小板减少性紫癜)的相关因素。方法:随机对156例患儿分组,行调查分析、实验室分析、诊治方法分析、诊治效果分析、机体预后情况分析。结果:在所有因素指标之中,12项指标存在显著差异,P<0.05,其中,有2项是连续型变量,包括体重(生病之时)、血小板峰值(诊治以后),对存在显著意义且经单因素分析的12项因素实施多因素分析(Logistic非条件回归模型),各危险因素分别为诊治前较长的病史、年龄大(发病之时)、白细胞计数在病初阶段降低或者正常、体重于发病时较大、骨髓巨核细胞于病初阶段数目高、血小板峰值于诊治后降低。结论:在初诊AITP病儿之时,检查骨髓巨核细胞,并于诊治中对血小板最大值予以观察,对判断预后很有价值,对AITP预后的改善有利的因素即为早确诊、早诊治。

  19. 重组人血小板生成素在难治性血小板减少性紫癜中的应用%Application of recombinant human thrombopoietin on refractory idiopathic thrombocytopenic purpura

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    曹慧琴; 任羽; 苏保雄; 郝彩霞; 刘卉; 刘宇宏

    2012-01-01

    Objective To evaluate the efficacy and safety of human recombinant thrombopoietin ( rhTPO ) on refractory idiopathic thrombocytopenic purpura ( ITP ). Methods 35 patients with ITP were divided into three groups: test group 1 ( treated with rhTPO combined danazol,n = 12 ),test group 2( only use rhTPO,n = 8 ),control group ( only use danazol,n = 15 ). Results The median counts of platelet in test group 1 was 6( 1. 0 ~ 15. 0 ) ×109/L before medi- cation;the maximum value increased to 97( 43 ~ 136 )× 109/L after 14 d of treatment(P 0.05).结论 rhTPO治疗难治性ITP安全、有效,rhTPO联合达那唑比单用达那唑起效快,能更好地控制出血症状.

  20. Thrombotic Thrombocytopenic Purpura:Clinical Analysis of 12 Cases%血栓性血小板减少性紫癜12例临床分析

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    刘景华; 周凡; 刘彦琴; 王吉刚; 白颖; 刘洋; 惠玉; 张晓琳; 李敏燕

    2014-01-01

    目的:总结分析血栓性血小板减少性紫癜患者的临床表现,实验室特点及治疗,以提高对血栓性血小板减少性紫癜的诊断及治疗水平。方法回顾性分析我院2008年1月~2013年12月诊断的12例血栓性血小板减少性紫癜患者的临床表现、实验室检查及治疗方法。结果①12例患者表现为五联征(微血管病性溶血性贫血、血小板减少、神经精神症状、发热、肾脏损害)者8例,三联征(微血管病性溶血性贫血、血小板减少、神经精神症状)者11例。②所有患者乳酸脱氢酶水平均增高,外周血破碎红细胞比例2~15%。③原发病:病因淋巴瘤1例,抗磷脂抗体综合征LPS 1例,系统性红斑狼疮SLE 1例,免疫因素5例,感染2例,原因不明2例。④治疗以血浆置换、血浆输注、利妥昔单抗、糖皮质激素为主,治疗有效率82.7%。结论血栓性血小板减少性紫癜的早期诊断仍然依赖临床表现,在进行血浆置换、利妥昔单抗等抢先治疗的同时完善相关检查进行排除性诊断。%Objective To investigate clinical features,outcome and laboratory characteristics of thrombotic thrombocytopenic purpura (TTP) and improve diagnostic ability for physician in TTP management. Methods Totally 12 diagnosed TTP from 2008.1 to 2013.12 were analyzed clinical features, laboratory characteristics and treatment. Results ①There were 8 patients with the pentad of TTP,including hemolytic anemia, thrombocytopenia, neurologic abnormalities, fever and kidney injury. 11 patients had triad of TTP. ②Al the patients had high level of lactate dehydrogenase, and the schistocytes of peripheral blood smears were present in al cases with a arrange of 2%~15%. The primary disease: 1 cases of lymphoma, antiphospholipid antibody syndrome and 1 cases of LPS, 1 cases of systemic lupus erythematosus SLE, immune factors in 5 cases, infection in 2 cases, 2 cases with unknown causes

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

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    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

  2. A Case Report of Gastric Carcinoma with Bone Marrow Metastasis and Thrombotic Thrombocytopenic Purpura%胃癌骨髓转移并血栓性血小板减少性紫癜一例报告

    Institute of Scientific and Technical Information of China (English)

    丁宁; 宋永波

    2011-01-01

    Objective To improve the recognition of thrombotic thrombocytopenic purpura(TTP)and reduce misdiagnosis rate.Methods The clinical data of a patient with gastric carcinoma and bone marrow metastasis and TTP were retrospectively analyzed.Results The patient was admitted to our hospital for unknown fever and anemia.Then he had hematemesis and was diagnosed as having gastric carcinoma by gastroscope.Manifested as having progressive consciousness disorders,thrombocytopenia, hemolytic anemia gradually during the course of disease, the patient underwent blood smear detection,which showed lobe erythrocytes, and then he was diagnosed as having gastric carcinoma with bone marrow metastasis and TTP.Conclusion We should pay attention to TTP when a patient with non-hematological system disease suffers from progressive anemia and thrombocytopenia.%目的 提高对血栓性血小板减少性紫癜(thrombotic thrombocytopenic punpura,TTP)的认识,以减少误诊.方法 回顾分析1例胃癌骨髓转移并TTP的临床资料.结果 患者以发热、贫血原因待查入院,入院后出现呕血,胃镜检查确诊为胃癌.病程中出现进行性意识障碍、血小板减少及溶血性贫血,血涂片可见裂片红细胞,确诊为胃癌骨髓转移并TTP.结论 对非血液系统恶性肿瘤患者出现进行性贫血、血小板减少等表现时,应注意合并TTP.

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

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    Khwaja Saifullah Zafar

    2015-01-01

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

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

    Institute of Scientific and Technical Information of China (English)

    刘巧

    2010-01-01

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

  5. Analysis of clinical feature of infantile idiopathic thrombocytopenic purpura%婴儿特发性血小板减少性紫癜临床特点分析

    Institute of Scientific and Technical Information of China (English)

    刘小丹; 李学荣; 孙立荣

    2011-01-01

    目的 分析婴儿特发性血小板减少性紫癜(idiopathic thrombocytopenic purpura,ITP)的特点.方法 从性别、临床表现、实验室检查、治疗效果等方面对77例ITP患儿进行观察和分析评价.结果婴儿组男性占75%,年长儿组男性占60%; 诱因为疫苗接种:婴儿组占28%,年长儿组占4%;诱因为感染:婴儿组为38%,年长儿组为58%;临床表现:婴儿组多见皮肤出血,出血程度较轻,年长儿组多见黏膜出血,或皮肤和黏膜混合出血,出血程度较重;治疗效果:婴儿组治愈+显效者30/32例(94%),年长儿组36/45例(84%),婴儿组无一例发展为慢性型,年长儿组3例发展为慢性型.结论 与年长儿组比较,婴儿组ITP患儿常见于男性,发病诱因主要为感染和免疫接种,出血程度较轻,疗效反应好,起效快,不易发展为慢性.%Objective To analyze the characteristics of infantile idiopathic thrombocytopenic purpura (ITP).Methods Clinical data of 77 ITP inpatients ( including 32 infants and 45 children older than 1 year of age)were observed.Clinical features, manifestations, laboratory findings and the treatment effect were analyzed and evaluated.Results Male accounted for 75% and 60% in infants and older children respectively; 28% of infants vs.4% of older children were induced by immunizations; 38% of infants vs.58% of older children were induced by infections; skin bleeding or mild hemorrhage were most commonly found in infants, while mucosal bleeding, mixed bleeding of skin and mucous membrane and severe hemorrhage were most commonly found in older children; the cure rate of infants was 30/32 (94%) compared with 36/45 (86%) in older children.None of infants vs.3 of older children developed into chronic ITP.Conclusion Infantile ITP preferentially affects boys.It is mainly induced by infection and immunization.It has mild hemorrhage, favorable therapeutic effect and lower susceptibility to chronic ITP.

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

    Institute of Scientific and Technical Information of China (English)

    王晓格; 胡姬婷

    2014-01-01

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

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

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    Emel Okulu

    2011-09-01

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

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

    Institute of Scientific and Technical Information of China (English)

    白松婷; 赵晓明; 盛光耀; 方营旗

    2011-01-01

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

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

    Institute of Scientific and Technical Information of China (English)

    龙远雄; 马利; 何丽云; 闫世艳; 王彬; 王双连

    2015-01-01

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

  10. Clinical observation for cytomegalovirus infection of infant idiopathic thrombocytopenic purpura%婴儿特发性血小板减少性紫癜巨细胞病毒感染临床观察

    Institute of Scientific and Technical Information of China (English)

    王欣; 乔丽津

    2011-01-01

    Objective To investigate the effect of cytomegalovirus (CMV) infection on the patho-genesis of infant idiopathic thrombocytopenic purpura (FTP). Methods IgM/DNA of serum CMV were detected in 178 cases of infants ITP in our hospital from June of 2004 to December of 2009. According to the results of positive or negative, they were divided into observation group and control group. Their ages of onset, course of disease, inducing factors, bleeding degrees, lab examinations and outcome were analyzed retrospectively. Results The median ages of observation group (81 cases) and control group (97 cases) were three months and six months respectively; The prodromal infection rate for both groups were 37% and 42% respectively and there was no statistical difference (P >0. 05). The prodromal infection was mainly upper respiratory tract infection; 72% and 75% patients in two groups had vaccination history and there was no statistical difference ( P > 0.05); The degrees of bleeding in two groups had no statistical difference (P >0.05). The platelet counts of over half patients were less than 10 ×109/L when admitted into hospital. The mean values were 12.28 × 109/L and 11.67 × 109/L respectively and showed no statistical difference ( P > 0.05); The bone marrow smears of two groups appeared normal or increased number of megakaryocyte combined with maturation arrest and thrombocyte dysplasia; 86% (31/36) and 81% (26/32) patients in two groups had abnormal immunoglobulin and there was no statistical difference (P > 0.05). After treatment of prednisone and/or venous gamma globulin, the platelet counts of 75 cases (93% ) and 93 cases (96% ) in two groups turned to normal and there was no statistical difference ( P > 0. 05 ). Conclusions CMV infection is very common in small children and may be one of inducing factors for thrombocytopenic purpura. Clinical manifestation of ITP patients has no significant difference whether they have CMY infection or not. Infant ITP with or without

  11. 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

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    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

  12. Púrpura trombocitopênica idiopática na infância: estudo de base populacional no Catar Idiopathic thrombocytopenic purpura in childhood: a population-based study in Qatar

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    Naima Al-Mulla

    2009-06-01

    Full Text Available OBJETIVO: Definir o padrão da púrpura trombocitopênica idiopática (PTI (aguda/crônica, e descrever seus sintomas e características clínicas em crianças com menos de 14 anos de idade em uma sociedade árabe recentemente desenvolvida. MÉTODOS: Este estudo descritivo retrospectivo foi realizado no Departamento de Pediatria do Hospital Geral de Hamad, Hamad Medical Corporation, Catar. Foram incluídas neste estudo 50 crianças com idade inferior a 14 anos e diagnóstico de PTI durante o período de 2000 a 2005. RESULTADOS: Das crianças estudadas (50, 62% foram diagnosticadas com PTI aguda e 38% com PTI crônica. A PTI aguda foi mais prevalente em meninos (64,5% em comparação com meninas (35,5%, enquanto que a PTI crônica apresentou uma distribuição quase igual em meninos (57,9% e meninas (42,1%. História de infecção viral foi comum em casos de PTI tanto aguda (71% quanto crônica (63,2%; 68% das crianças com PTI apresentaram contagem de plaquetas abaixo de 20x10(9/L ao diagnóstico. A maioria das crianças estudadas (74% foi tratada com imunoglobulina intravenosa. CONCLUSÕES: O estudo revelou uma alta incidência de PTI entre as crianças no Catar. As descobertas do estudo são semelhantes às de outros relatos internacionais.OBJECTIVE: To find the pattern of idiopathic thrombocytopenic purpura (ITP (acute/chronic and to describe presenting features and clinical characteristics of the disease in children below 14 years of age in a newly developed Arabian society. METHODS: This retrospective, descriptive study was carried out at the Pediatric Department of the Hamad General Hospital, Hamad Medical Corporation, Qatar. A total of 50 children below 14 years of age who were diagnosed with ITP during the period 2000-2005 were included. RESULTS: Among the studied children (50, 62% were diagnosed with acute ITP and 38% with chronic ITP. Acute ITP was more prevalent in boys (64.5% when compared with girls (35.5%, whereas for chronic ITP

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

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    张永卓

    2013-01-01

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

  14. Clinical Significance of Helicobacter Pylori in Children with Idiopathic Thrombocytopenic Purpura%幽门螺杆菌在儿童特发性血小板减少性紫癜中的临床意义

    Institute of Scientific and Technical Information of China (English)

    唐瑛; 王书春; 王鲁娟; 刘永; 王海英; 王占聚

    2013-01-01

    This study was aimed to investigate the clinic significance of helicobacter pylori (HP) in children with idiopathic thrombocytopenic purpura( ITP). The infection of HP in 92 ITP children was determined by 13C-Urea Breath Test, the same test was also performed on 66 healthy children. The 68 children infected with HP were randomly divided into 2 groups: single drug group treated only with corticosteroid and; combined drug group treated with corticosteroid and anti-helicobacter pylori treatment. The results showed that 68 patients infected with HP were found in 92 ITP children(74. 7% ), 26 patients infected with HP were observed in 66 healthy children (39.4% ), which was lower than that in ITP children (74.7% , P<0.05). After anti-helicobacterpylori therapy, the total effective rate and cure rate of ITP patients increased respectively from 73.5% to 94.1 % , and the total recurrence rate( 17.0% ) was much lower than single drug group( 47. 1% , P < 0. 05). Otherwise, after therapy the platelet count in both two groups increased continuously, and at the same time point, the platelet count in snti-helicobacterium pylori group was higher than that in the single drug group(P < 0. 05). It is concluded that the ITP children have a higher infection rate of HP, which may be involved in the pathogenesis of ITP. Anti-helicobacterium pylori therapy would help to improve the therapeutic efficacy and reduce the recurrence of ITP children.%本研究旨在探讨幽门螺杆菌(helicobacter pylori,HP)在儿童特发性血小板减少性紫癜(ITP)中的临床意义.用13C尿素呼气试验检测92例ITP儿童患者的感染情况,并检测66名健康儿童作为对照组.将ITP患儿中68例阳性者随机分为2组:复合用药治疗组34例,应用糖皮质激素+抗菌治疗;单药治疗组34例,用糖皮质激素治疗.结果表明:ITP患儿HP的感染率为74.7%,明显高于对照组(39.4%)(P<0.05).ITP患儿经治疗后,单药治疗组的总有效率(73.5

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

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    Lucia M. A. Campos

    2013-02-01

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

  16. Celiac Disease Presenting with Immune Thrombocytopenic Purpura

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    Hakan Sarbay

    2017-01-01

    Full Text Available Celiac disease (CD is an immunological disorder. Clinical manifestations occur as a result of intestinal mucosa damage and malabsorption. CD is also associated with extraintestinal manifestations and autoimmune disorders. The coexistence of CD and autoimmune diseases has been described before. In this article, a patient with CD presenting with thrombocytopenia is discussed.

  17. Neonatal alloimmune thrombocytopenia

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    Mella MT

    2015-06-01

    Full Text Available Maria Teresa Mella, Keith A Eddleman Department of Obstetrics and Gynecology, Division of Maternal Fetal Medicine, Mount Sinai School of Medicine, New York, NY, USA Abstract: Neonatal alloimmune thrombocytopenia occurs in one in 1,000–1,500 live births and is the most common cause of severe thrombocytopenia and intracranial hemorrhage in term infants. It is the equivalent of red blood cell alloimmunization and is due to transplacental passage of maternal antibodies against paternally derived fetal platelet antigens. A diagnosis of neonatal alloimmune thrombocytopenia should be considered for any neonate with unexplained thrombocytopenia. Once the diagnosis is made, it is known that all subsequent pregnancies are at risk for severe disease. In order to prevent the devastating and potentially life-threatening manifestations of the disease, the goal is to initiate treatment early with serial percutaneous umbilical blood sampling, intravenous immunoglobulin administration, prednisone, and/or fetal platelet transfusions. Timing of delivery is variable with delivery for severe disease recommended at an earlier gestational age. Vaginal delivery can be considered if the fetal platelet count is greater than 50,000–100,000 µL. Thrombocytopenia due to neonatal alloimmune thrombocytopenia usually resolves spontaneously within 1–2 weeks after delivery, but a platelet transfusion may be necessary to prevent a serious hemorrhagic event. In all cases, a multidisciplinary approach to care should be undertaken with delivery at a tertiary care center. Keywords: neonatal thrombocytopenia, fetal therapy, intracranial hemorrhage, intravenous immunoglobulin, alloimmunization

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

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    Ricardo Zorrón

    2004-08-01

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

  19. 婴儿期特发性血小板减少性紫癜的临床特点分析%Clinical Analysis of Infantile Idiopathic Thrombocytopenic Purpura

    Institute of Scientific and Technical Information of China (English)

    王功僚; 邓仕华; 李慧娴; 黄积德

    2013-01-01

    Objective To analysis clinical characteristics of infantile idiopathic thrombocytopenic purpura (ITP).Methods From Jaunary 2006 to December 2011,a total of 130 ITP patients (including 67infants and 63 children) were enrolled in this study.They were divided into two groups according to their age,infant group (n=67) and young children group (n=63).The study protocol was approved by the Ethical Review Board of Investigation in Human Being of Guangxi Baise Maternal and Child Health-Care Hospital.Informed consent was obtained from all participates' parents.Clinical features of gender distribution,predisposing factors,clinical manifestations,blood test on admission,treatment effect and outcome were retrospectively reviewed.Results No significant difference of gender distribution in two groups were found (U=0.6499,P> 0.05).Prodromal infections within 4 weeks before the onset were found in 40.29% (27/67) of infant group and 60.32% (38/63) of young children group (U=2.2827,P<0.05).The history of vaccination within 4 weeks were found in 23.88% (16/67) of infant group,and zero (0.00) of young children group (U=4.2909,P<0.01).The median of platelet counts on admission in infant group (8 × 109/L) were significant lower than that in young children group (13× 109/L) (t =2.864,P<0.01).Clinical presentation of infant group were skin bleeding or mile hemorrhage,young children group showed mucosal bleeding or mixed bleeding of skin and mucous membrane and severe hemorrhage.Bleeding in other sites was observed predominantly in infant group compared with that in young children group (U=2.3238,P<0.05).The rates of therapeutic effects (complete remission + valid) in infant group was 100.00% with no chronic cases,and 92.06% (58/63) in young children group,11 cases (17.46%) were developed into chronic ITP in young children group.Conclusions The history of vaccination was the crux of infantile ITP.Infantile ITP has good efficacy,rapid response,lower susceptibility

  20. 儿童特发性血小板减少性紫癜脾切除术的围手术期处理%The perioperative management of splenectomy for Idiopathic thrombocytopenic purpura in children

    Institute of Scientific and Technical Information of China (English)

    金晟; 吴晔明

    2015-01-01

    Objetive To explore the perioperative methods of idiopathic thrombocytopenic purpura (ITP)in children. Methods Clinical data of 12 children suffering from ITP,who underwent splenectomy, their perioperative treatments and their effects were retrospectively analyzed. Results Our group included 12 children with the mean age of 7.1 years old (range 3y to 12y).The mean day of their illness was 3 years (range 1 .5y to 7y).Preoperatively,the platelet counts of 10 children were lower than normal count,with the mean count of 29.4 ×109 /L.Glucocorticoids combined with platelet transfusion were employed for 7 children whose platelet counts were lower than 30 ×109 /L.A second platelet transfusion combined with a large dose of glucocorticoids and immunoglobulin injections were done for 4 children whose platelet counts were not improved after the first transfusion.When their platelet counts were improved to >60 ×109 /L,they were treated by e-mergency splenectomy.All patients were successfully treated.The platelet counts of 2 patients,who were posi-tive in PAIg,were dropped to below normal count.During the follow-up of 10 months to 8 year,8 children were curing,while 2 patients were with durable remission. Conclusions Splenectomy is a safe and effective method for the children who suffer from chronic ITP and ITP which is ineffective for drug therapy.In addition, PAIg should be considered as a routine examination in children with ITP.%目的:探讨儿童特发性血小板减少性紫癜(ITP)脾切除的围手术期处理方法。方法回顾性分析2006年至2014年作者收治的12例 ITP 行脾切除术的患儿临床资料,探讨其围手术期的处理及治疗效果。结果本组12例平均年龄7.1(3~12)岁,病程1.5~7年,平均3年。10例术前血小板低于正常值,平均为29.4×109/L,最低5×109/L。术前对7例血小板<30×109/L 的患儿采用激素联合输血小板治疗,其中4例输注后血小板无法提升,

  1. Clinical Characteristics of Idiopathic Thrombocytopenic Purpura in 401 Infants%婴幼儿特发性血小板减少性紫癜401例临床特点

    Institute of Scientific and Technical Information of China (English)

    潘艳莎; 贾苍松; 陈婷婷

    2011-01-01

    Objective To analyze clinical characteristics of infants with idiopathic thrombocytopenic purpura(ITP) from 1 month to 3 years old, and compare the therapeutic effects. Methods The data of 401 ITP children were collected. All of the patients were given hormone shock treatment and intravenous immunoglobulin. According to the extent of platelet count and bleeding improvement, the outcome included:complete remission, effective, invalid. The patients were divided into younger group( ≤1 year old) and older group( > 1 -3 years old)based on age, acute group( ≤6 months) and chronic group( >6 months)based on the course of disease, their clinical data were retrospectively analyzed by SPSS 12.0 software. Results 1. Although both the younger group and the older group had higher male/female ratio, there was no significant difference of the percentage of the male between younger group and older group(x2 =0. 682 ,P >0. 05 ). 2. The median of platelet count at admission in the younger group were statistically lower than those in the older group ( Z = 2. 668, P < 0. 05 ). 3. Both the median of megakrocytes( which produce platelets) and the increase ratio of megakrocytes in the younger group were statistically lower than that in the o1der group( Z = 2. 065 ,P < 0.05 ;x2 = 16. 322, P < 0. 001 ). 4. Under the same treatment, the time needed for the platelets to become normal ( ≥ 100 × 109 L -1 ) in the younger group were statistically lower than that in the older group( Z = 3. 542,P < 0. 001 ). 5. The median of platelet count at admission in the acute group were statistically lower than that in the chronic group ( Z = 2. 100,P < 0.05 ). 6. There was no significant difference in terms of hospital stays between the transfused patients and untransfused patients( Z = 1. 385, P > 0. 05 ). Conclusions The infants with ITP have higher male/female ratio, most possess unobvious incentive and mucocutaneous hemorrhage. The younger patients ( ≤ 1 year old) with lower

  2. 不同剂量丙种球蛋白联合地塞米松治疗小儿特发性血小板减少性紫癜的临床疗效分析%Effect observation of different doses Dexamethasone combined with gamma globulin in the treatment of idiopathic thrombocytopenic purpura with children

    Institute of Scientific and Technical Information of China (English)

    覃黄正

    2014-01-01

    Objective To explore the clinical effect of different dose Dexamethasone combined with gamma globulin in the treatment of idiopathic thrombocytopenic purpura with children. Methods 120 patients with Idiopathic thrombocytopenic purpura in our hosipta were randomly divided intocontrol group, low-dose group and high dose group, each group had 40 cases. All patients were received treatment using dexamethasone injection, children with high-dose group were given Globulin 400mg/(kg·d), 1 times a day, once every 5d;children withlow-dose group were given Globulin 200mg/(kg·d), 1 times a day, once every 5d;the clinical efficacy, platelets rise time, platelet recovery time, time to stop bleeding of three group were observation and compared. Results The clinical efficacy of high-dose group, the rise time of platelets and platelet recovery time, time to stop the bleeding was compared between low-dose group and high dose group, the difference was not statistically significant (P > 0.05), compared with control group, the differences were statistically significant (P < 0.05). Conclusion Gamma globulin and dexamethasone can significantly improve pediatric idiopathic thrombocytopenic purpura clinical symptoms, the rapid increase platelet count, and small doses of gamma globulinhad similartreatment effect with high dose, it is worthy of wide application in clinical.%目的:探讨不同剂量丙种球蛋白联合地塞米松治疗小儿重症特发性血小板减少性紫癜的疗效观察。方法将120例小儿特发性血小板减少性紫癜患儿随机分为对照组,小剂量组和大剂量组,每组各40例,所有患者均接受采用地塞米松注射液治疗,大剂量组患儿给予丙种球蛋白400mg/(kg·d),1次/d,连用5d;小剂量组患儿给予丙种球蛋白200mg/(kg·d),1次/d,连用5d,观察比较三组临床疗效,血小板上升时间,血小板恢复正常时间,出血停止时间。结果小剂量组与大剂量组临床疗

  3. An effect analysis of different doses of gamma globulin combined with prednisone in the treatment of idiopathic thrombocytopenic purpura%不同剂量的丙种球蛋白联合强的松治疗特发性血小板减少性紫癜的效果分析

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    潘凤娟; 钟翠鸣; 钟华

    2015-01-01

    Objective To compare the efficacy of low dose and high dose gamma globulin combined with prednisone in the treatment of idiopathic thrombocytopenic purpura for obtaining a better cost - effect ratio treatment scheme. Methods Between March 2010 and May 2014 a total of 64 pediatric patients with idio-pathic thrombocytopenic purpura cared at Pediatrics Department of our hospital were enrolled in this study,a hidden random number table method was used to allocate the patients to two groups.Thirty- two cases in group A were given 400 mg/(kg · d)gamma globulin for 5 days,after that they received oral prednisone. Thirty-two cases in group B were given 200 mg/(kg·d)gamma globulin for 5 days,after that they received oral prednisone,after drug administration,platelet count was measured,and the platelet elevating time,mean hemostasis time,time to platelet peak and the platelet value were all recorded every day.The efficacy rate was compared between the two groups. Results After treatment,comparison of mean platelet elvating time,av-erage hemostasis time,mean platelet peak value between the two groups of pediatric children showed no signif-icant differences (P > 0.05 ).Group A patients had shorter average peak time than group B,group paired comparison yielded statistical difference (P 0.05 ). Conclusion Low dose gamma globulin combined with prednisone in treatment of idiopathic thrombocytopenic purpura can obtain high potency ratio,can quickly increase platelet and control of hemorrhage.%目的:比较大剂量和小剂量丙种球蛋白联合强的松治疗特发性血小板减少性紫癜(ITP)的效果,寻求效价比高的治疗方案。方法选择我院儿科2010年3月~2014年5月住院的64例特发性血小板减少性紫癜患儿,采用隐匿数字随机法分为两组,A 组32例患儿给予400 mg/(kg·d)丙种球蛋白治疗5 d 后口服强的松,B 组32例患儿给予200 mg/(kg·d)丙种球蛋白治疗5 d 后口服强的松,用药后每天检测血小板计数,

  4. Platelet alloimmunization after transfusion

    DEFF Research Database (Denmark)

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

    1997-01-01

    BACKGROUND AND OBJECTIVES: The frequency of platelet-specific antibodies after one series of blood transfusions has not been reported, and in multiply transfused patients is controversial. MATERIALS AND METHODS: We studied the frequency of alloimmunization against platelet antigens in 117 patient...

  5. Trombotisk trombocytopenisk purpura hos barn med lavt ADAMTS13 enzymniveau

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    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...

  6. 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

  7. Clinical Analysis of Thrombocytopenic Purpura Given Different Measurement of Gamma Globulin Combined with Prednisone%血小板减少性紫癜给予不同剂量丙种球蛋白联合强的松治疗的临床分析

    Institute of Scientific and Technical Information of China (English)

    何飞

    2016-01-01

    目的:观察并探讨血小板减少性紫癜给予不同剂量丙种球蛋白联合强的松治疗的临床疗效分析。方法:选取2013年12月至2015年12月来我院治疗的90例血小板减少性紫癜患儿进行回顾性分析,随机分成两组:给予观察组患儿静注400mg/(kg·d)丙种球蛋白,治疗5d后联合强的松2mg/(kg·d)(每日3次),30d为一疗程;给予观察组患儿静注200mg/(kg·d)丙种球蛋白,联合强的松的用量及疗程均为观察组相同。观察两组患者在经过各自治疗后的血小板上升、达峰值以及止血时间对比。结果:经比较得知,两组患者在血小板上升时间、止血时间以及临床疗效上无明显差异(P<0.05),观察组在达峰值时间上要少于对照组,两组患者存在显著差异(P<0.05),具有统计学意义。结论:丙种球蛋白联合强的松治疗临床血小板减少性紫癜的临床疗效明确,不良反应少,大剂量丙种球蛋白联合强的松的达峰值时间更短,小剂量丙种球蛋白联合强的松的临床疗效基本等同于大剂量,疗效可靠。%Objective: To observe and evaluate the effect of thrombocytopenic purpura given different measurement of gamma globulin combined with methylprednisolone therapy.Methods: 90 cases of platelet from December 2013 to December 2015 in our hospital for treatment of idiopathic thrombocytopenic purpura were retrospectively analyzed, were randomly divided into two groups. The observation group were given intravenous injection of 400mg/kg·d gamma globulin (1 times daily), for 5d 2mg/kg·d combined with prednisone (3 times daily), 40d for a course of treatment. Observation group were given intravenous injection of 200mg/kg·d globulin (1 times daily), dosage and treatment combined with prednisone were same as observation group. Patients in two groups were observed on the rise after the treatment of platelets, peak and hemostasis time comparison. Results: By comparison

  8. Purpura fulminans

    DEFF Research Database (Denmark)

    Jordan, Karina; Kristensen, Kim

    2010-01-01

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

  9. Role of cytotoxin associated protein A in the pathogenesis of idiopathic thrombocytopenic purpura%幽门螺杆菌细胞毒素相关蛋白A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)

    雷瑚仪; 赵谢兰

    2012-01-01

    Objective Helicobacter Pylori (Hp) infection is now considered one of the causes of idiopathic thrombocytope-nic purpura (ITP) . This study aimed to explore the mechanism of Hp infection causing ITP by investigating the relation of cytotoxin associated protein A of Hp with the PA-IgG level and PLT count in ITP patients. Methods We detected Hp using the rapid urease test (RUT) and 14C urea breath test (14C UBT). We divided 60 patients with ITP into three groups, Hp- , Hp+ cagA- , and Hp+ cagA+ , determined the PLT counts and levels of cagA and PA-IgG before and after anti-Hp therapy, and compared them among the three groups. Results The PA-IgG level was significantly higher ( P 0. 05 ) . Compared with the baseline, the PA-IgG level was significantly decreased and PLT count remarkably increased in the Hp + cagA + group ( P 0. 05). Conclusion Helicobacter pylori increases the PA-IgG level and reduces the PLT count in ITP patients, which may be associated with cagA and involved the pathogenesis of ITP.%目的 目前,认为幽门螺杆菌( helicobacter pylori,Hp)感染是引起特发性血小板减少性紫癜(idiopathic thrombocytopenic purpura,ITP)的病因之一.研究幽门螺杆菌细胞毒素相关基因蛋白A(cytotoxin-associated gene protein,cagA)与特发性血小板减少性紫癜的血小板相关IgG抗体(platelet associated IgG,PA-IgG)及血小板数量变化的关系有助于探索Hp感染引起ITP的机制.文中通过检测ITP患者血清中cagA、PA-IgG和PLT数量,分析Hp感染与ITP发生的关系. 方法 快速尿素酶试验(rapid urease test,RUT)及14C尿素呼气试验(UBT)检测Hp.60例ITP患者分为Hp-、Hp+ cagA -及Hp+ cagA+3组,酶联免疫法测定外周血cagA、PA-IgG,用血细胞仪测定血小板计数,并比较抗Hp治疗前后的变化. 结果 Hp+ cagA+组的PA-IgG水平与Hp-和Hp+ cagA -组比较明显升高(P<0.05),血小板计数与Hp-和Hp+ cagA -组比较明显降低(P<0.05).Hp+ cagA -组的PA-IgG水平及血小板计数

  10. EB病毒感染与儿童急性免疫性血小板减少性紫癜关系的初步探讨%Preliminary Exploration of the Relationship between Epstein-Barr Virus Infection and Acute Immune Thrombocytopenic Purpura in Children

    Institute of Scientific and Technical Information of China (English)

    颜慕霞; 张力; 林涛

    2011-01-01

    目的 探讨Epstein-Barr病毒(EBV)感染与儿童急性免疫性血小板减少性紫癜(ITP)的关系.方法 分别用实时荧光定量聚合酶链反应(FQ-PCR)方法和血清酶联免疫吸附试验(ELISA)方法检测115例急性ITP患儿和50例健康儿童的EBV DNA和EBV IgM抗体.结果 急性ITP患儿EBV DNA、EBV IgM阳性率均明显高于健康对照儿童,差异均有统计学意义(P<0.01).结论 儿童感染EB病毒可能是儿童发生急性免疫性血小板减少性紫癜的重要因素之一.%Objective To explore the relationship between Epstein-Barr Virus ( EBV ) infection and acute immune thrombocytopenic purpura ( aITP ) in children.Methods EBV-DNA in peripheral blood and serum EBV-IgM in 115 cases with ITP and 50 healthy children were detected by fluorescence quantitative-polymerase chain reaction( FQ-PCR ) and enzyme linked immunosorbent assay( ELISA ).Results The positive rates of EBV DNA,EBV IgM in the aITP group were higher than in the control group.There were significant difference between the two groups( P < 0.01 ).Conclusion EBV infection may be an important pathogenic factor of aITP in children.

  11. 升血小板胶囊联合激素治疗特发性血小板减少性紫癜疗效观察%Clinical effect of platelet increasing capsules combined with prednisone on treatment of idiopathic thrombocytopenic purpura

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    梁利杰; 游晓英

    2011-01-01

    目的 观察升血小板胶囊联合激素治疗特发性血小板减少性紫癜(ITP)的临床疗效.方法 选择临床及实验室确诊的ITP患者,将之分为3组:单用泼尼松组、单用升血小板胶囊组、泼尼松联用升血小板胶囊组.结果 泼尼松组有效率为88.23%,升血小板胶囊组有效率为80.00%,泼尼松和升血小板胶囊联用组有效率为94.44%.结论 治疗特发性血小板减少性紫癜,升血小板胶囊配合激素更能提高疗效,减少不良反应.%Objective The purpose of this study is to observe the clinical effect of platelet Increasing capsules (Traditional Chinese Medicine) combined with prednisone on the treatment of idiopathic thrombocytopenic purpura(ITP).Methods Patients were diagnosed by ITP based on their clinical and laboratory examinations.The patients with ITP were then divided into three groups: prednisone group, platelet increasing capsule group and platelet increasing capsule combined with prednisone group.Results The efficiency rate is 88.23% in patients treated with prednisone, 80.00% in patients treated with platelet increasing capsules, and 94.44% in patients treated with both.Conclusions The platelet increasing capsules combined with prednisone greatly increased plateletan improv clinical symptoms, and reduce side effect and toxicity of prednisoe, provide a better strategy for treating ITP.

  12. Evidence-based Treatment for a Newly Diagnosed Acute Idiopathic Thrombocytopenic Purpura in Children%一例初诊儿童急性特发性血小板减少性紫癜患者的循证治疗

    Institute of Scientific and Technical Information of China (English)

    吴星; 徐之良

    2010-01-01

    目的 结合1例初诊儿童急性特发性血小板减少性紫癜患者的循证治疗经过,总结评价治疗儿童急性特发性血小板减少性紫癜的最佳证据,为临床实践提供参考.方法 充分评估患者情况后,提出临床问题,从cochrane图书馆(2009年第4期)Medline(PbuMed网站1993年1月至2009年10月)中国知网知识总库上进行检索.检索检索主题词为:ildren acute immune thrombocytopenic purpura, platelet count, intracranial hemorrhage, Corticosteroids ,intravenous immuneglobulin,mega-dose methylprednisolon refractory itp, Rituximaba, splenectomy, human, children 0~8 years, systematic review, meta-analysis, randomized controlled trials.结果 检索出与不同问题相关的随机对照实验30篇,系统评价或Meta分析6篇,通过对检索结果进行分析,为患者制定了合理的治疗方案.经1年随访证实,该方案适合患者.结论 采用循证治疗的方法,为初治的儿童特发性血小板减少性紫癜患者确定合理的治疗目标和治疗方案,可有效提高治疗效果.

  13. 小剂量利妥昔单抗治疗难治性血小板减少性紫癜序贯维持的临床研究%Clinical research of low-dose rituximab therapy and sequential maintenance for patients with refractory idiopathic thrombocytopenic purpura

    Institute of Scientific and Technical Information of China (English)

    王吉刚; 周凡; 刘彦琴; 刘景华; 白颖; 徐宪平; 董援利; 孟广晗; 佟丹江

    2016-01-01

    Objective To investigate the efficacy and safety of low-dose rituximab therapy and sequential maintenance for patients with refractory idiopathic thrombocytopenic purpura. Methods Thirty-three patients with refractory idiopathic thrombocytopenic purpura received intravenous rituximab at the dose of 100 mg once a week for 4 consecutive weeks. Complete blood cell count and serum concentrations of immunoglobulin (IgG,IgM and IgA) were monitored regularly. The numbers of CD3+ and CD19+ CD20+ lymphocyte cells were assayed by flow cytometry before and after therapy. Twenty-five patients with responses(complete response and response) were divided into maintained group (12 patients) and control group (13 patients) by random digits table method. The patients in maintained group were treated with rituximab 100 mg every 6 months. The efficacy of maintenance therapy was evaluated through long-term follow-up. Results The complete response(CR) rate, response (R) rate and no response(NR) rate were 48.48%(16/33), 27.27%(9/33) and 24.24% (8/33), respectively. As a result, total effective rate was 75.76% (25/33). There were no significant changes of peripheral blood white blood cell count,hemoglobin,serum immunoglobulin and CD3+lymphocyte counts before and after treatment (P>0.05). However, CD19+ CD20+ cells were almost depleted in patients treated with rituximab: (3.71±2.64)×106/L vs. (279.33±92.78)×106/L, P<0.01. Five patients suffered from allergic response, and 1 patient developed pneumonia and respiratory failure. The relapse rates of maintained group and control group were 1/12 and 4/13, respectively. Conclusions Treatment with low-dose rituximab may be an effective and safe approach in patients with idiopathic thrombocytopenic purpura. Relapse rates can be decreased through maintenance therapy with refractory low-dose rituximab. However, the optimal therapeutic schedule need further investigation.%目的:探讨小剂量利妥昔单抗治疗难治性特发性血小板

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

    Directory of Open Access Journals (Sweden)

    PlínioCarlos Baú

    2007-12-01

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

  15. 白介素-11联合激素治疗慢性特发性血小板减少性紫癜的起效时间及血小板变化情况研究%STUDY OF THE ONSET TIME AND THE CHANGE IN TREATMENT FOR CHRONIC IDIOPATHIC THROMBOCYTOPENIC PURPURA WITH INTERLEUKIN -11 AND HORMONE

    Institute of Scientific and Technical Information of China (English)

    郑冬丽

    2011-01-01

    [Objective]To investigate the onset time and platelet changes in the treatment of chronic idiopathic thrombo-cytopenic purpura with IL -11 combined with dexamethasone.[Methods]90 chronic idiopathic thrombocytopenic puipura patients as research subjects in our hospital from January 2006-January 2010 were randomly divided into three groups, of which A group carried out IL -11 treatment, B group treated with dexamethasone, C group were combined hormone Interleukin -11 treated, observed and compared the onset time and platelet changes in three groups.[Results]After treatment, the total effective rate in C group was significantly higher than that in group A, B, and the time that C group reached the normal level of platelets was significantly shorter with the A group, B group (P all < 0.05).After beingtreated, platelets were significantly higher in three groups than before treatment, C group with more obvious increase, showed statistically significant differences between groups (P all < 0.05).The side effects were insomnia, hypertension, hyperglycemia, secondary infection, moon face, central obesity, and gastrointestinal bleeding.The incidence of side effects in C group was significantly lower than A, B groups, it showed statistically significant difference (P both <0.05), but between A, B groups, it showed no significance (P > 0.05).[Conclusion]Interleukin -11 combined hormone treatment for chronic idiopathic thrombocytopenic purpura is with faster onset time and less side reactions, significantly improves platelet function, which is worthy of clinical application.%[目的]探讨白介素-11联合地塞米松治疗慢性特发性血小板减少性紫癜的起效时间及血小板变化情况.[方法]选取某院2006年1月~2010年1月住院治疗的90例慢性特发性血小板减少性紫癜患者作为研究对象,随机分为3组各30例,其中A组采用白介素-11进行治疗,B组采用地塞米松进行治疗,C组采用白介素-11联合

  16. Paroxysmal drastic abdominal pain with tardive cutaneous lesions presenting in Henoch-Sch(o)nlein purpura

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    Xiao-Liang Chen; Hong Tian; Jian-Zhong Li; Jin Tao; Hua Tang; Yang Li; Bin Wu

    2012-01-01

    Henoch-Sch(o)nlein purpura (HSP) is a small-vessel vasculitis mediated by IgA-immune complex deposition.It is characterized by the clinical tetrad of non-thrombocytopenic palpable purpura,abdominal pain,arthritis and renal involvement.The diagnosis of HSP is difficult,especially when abdominal symptoms precede cutaneous lesions.We report a rare case of paroxysmal drastic abdominal pain with gastrointestinal bleeding presented in HSP.The diagnosis was verified by renal damage and the occurrence of purpura.

  17. 个体化剂量丙种球蛋白联合地塞米松治疗儿童重症特发性血小板减少性紫癜78例疗效观察%Therapeutic effects of individual-dose intravenous immunoglobulin combined with dexamethasone on 78 children with severe idiopathic thrombocytopenic purpura

    Institute of Scientific and Technical Information of China (English)

    李建厂; 贾秀红; 唐慎华; 朱淑霞; 杨华琴

    2011-01-01

    目的 观察个体化剂量静脉注射用丙种球蛋白(IVIG)联合地塞米松治疗儿童特发性血小板减少性紫癜(ITP)的疗效.方法 重症ITP患儿入院后均给地塞米松及IVIG[400 mg/(kg·d)],3 d后测外周血血小板计数,≥100×109/L停用IVIG,继用地塞米松;若<100×109/L,再继续用原剂量IVIG 2 d.结果 治疗3 d后,78例患儿中,37例外周血血小板计数升至100×109/L以上,余41例继续治疗后,31例升至100×109/L以上.结论 个体化剂量IVIG联合地塞米松是治疗儿童重症ITP的有效办法,可节省一定医疗费用.%Objective To evaluate the effect of individual-dose immunoglobulin combined with dexamethasone on 78 children with severe idiopathic thrombocytopenic purpura (ITP). Methods All the children with ITP were treated with dexamethasone and immunoglobulin (400 mg/kg · d). The platelet counts were detected after 3 day ' s treatment. The patients with platelet ≥ 100 × 109/L continued the treatment of dexamethasone. The others with platelet < 100 × 109/L added the treatment of immunoglobulin (400 mg/kg · d) for 2 days. Results The platelet counts of 37 children were elevated more than 100 ×109/L, and those of 41 children were still lower than 100 × 109/L after 3 day' s treatment. After additional treatment of dexamethasone and immunoglobulin for 2 days, 31 cases got a platelet increase of more than 100 × 109/L. Conclusions Treatment of individual-dose immunoglobulin combined with dexarnethasone is an effective way to treat children with severe ITP. It reduced a lot of medical cost for patients and their family.

  18. Clinical study on recombinant human thrombopoietin in the treatment of refractory idiopathic thrombocytopenic purpura%重组人血小板生成素治疗难治性特发性血小板减少性紫癜临床观察

    Institute of Scientific and Technical Information of China (English)

    张晓丽; 刘延方; 孙慧; 谢新生; 姜中兴; 孙玲; 万鼎铭; 刘林湘

    2010-01-01

    Objective To observe the efficacy and toxicity of recombinant human thrombopoietin (rhTPO)in the treatment of chronic refractory idiopathic thrombocytopenic purpura(ITP).Methods Twenty- four patients with chronic ITP were administered with rhTPO subcutaneously at 300 IU/(kg · d)for 14 days or until the platelet count > 100 × 109/L.Results Out of 24 patients with ITP, 12 cases showed significant response and 5 cases showed good response to the treatment with rhTPO.The peak platelet count after treatment was(129 ± 97)× 109/L, significantly higher than the mean platelet count [(10 ±5)× 109/L]before treatment(t = 5.822, P <0.01).The agent was well tolerated in most patients.Conclusions rhTPO exerts satisfactory efficacy in the treatment of chronic ITP with mild side effects.%目的 观察重组人血小板生成素(rhTPO)治疗慢性难治性特发性血小板减少性紫癜(ITP)的临床疗效及不良反应.方法 24例慢性难治性ITP患者皮下注射rhTPO 300 IU/(kg·d),疗程14 d或血小板>100×109/L后停药.结果 24例患者rhTPO治疗后,显效12例,有效5例,用药前血小板平均值为(10±5)×109/L,治疗后血小板计数的升高的最高值为(129±97)×109/L,与用药前比较差异有统计学意义(t=5.822,P<0.01).多数患者对药物耐受良好.结论 rhTPO治疗慢性难治性ITP具有良好的疗效,不良反应轻微.

  19. B淋巴细胞激活因子表达水平在儿童特发性血小板减少性紫癜诊断及预后判断中的意义%Value of B-cell activating factor expression in the diagnosis and prognosis of children with idiopathic thrombocyto-penic purpura

    Institute of Scientific and Technical Information of China (English)

    仲玉强; 王清

    2014-01-01

    Objective To study the value of B-cell activating factor (BAFF) expression levels in the diagnosis and prog-nosis of children with idiopathic thrombocytopenic purpura. Methods Children with idiopathic thrombocytopenic purpura (ITP) admitted to our hospital from September 2011 to September 2013 were enrolled in observation group, and healthy check-up chil-dren during the same period were enrolled as controls. The serum levels of B-cell activating factor, platelet antibodies (PAIgG, PAIgM, PAIgA) and platelet count were detected, and curative effect in children with ITP was observed. Results The BAFF, PAIgG, PAIgM and PAIgA levels were higher and platelet count were less in both acute and chronic ITP children than those in normal controls. The difference was signiifcant (P<0.05). The results of linear regression analysis indicated that BAFF levels were positively correlated with PAIgG, PAIgM and PAIgA levels (β=1.87~2.25, P<0.05), and were negatively correlated with platelet count (β=-2.42, P<0.05). Meanwhile, BAFF levels were negatively correlated with curative effect in ITP children (β=-1.88, P<0.05). When the value of BAFF was 1.35, the sensitivity for predicting effective treatment was 0.84 and the speciifcity was 0.75. The area under the ROC curve was 0.85 (95%CI:0.73~0.98, P=0.000). Conclusions The serum BAFF expression was increased and closely related to the level of antiplatelet antibody, platelet count and treatment effect in ITP children, and therefore, was a good indicator for the diagnosis and prognosis of ITP.%目的:研究B淋巴细胞激活因子(BAFF)表达水平在儿童特发性血小板减少性紫癜(ITP)诊断及预后中的应用价值。方法将2011年9月—2013年9月期间收治的初诊ITP患儿纳入研究组,同期健康体检儿童纳入对照组,检测两组对象血清中BAFF、抗血小板抗体PAIgG、PAIgM、PAIgA水平及血小板计数,观察治疗效果。结果急性型和慢性型ITP患儿外周血BAFF、PAIgG

  20. 特发性血小板减少性紫癜患者血小板相关抗体的表达及意义%The Expression and Significance of Platelet-associated Antibodies in Patients with Idiopathic Thrombocytopenic Purpura

    Institute of Scientific and Technical Information of China (English)

    李杪

    2014-01-01

    目的:探讨特发性血小板减少性紫癜(ITP)患者患病时PAIg的表达评价其在ITP中的临床意义。方法选取40例ITP患儿作为研究对象,同期门诊体检正常儿童20例为对照组,应用酶联免疫吸附法(ELISA法)测定血小板相关抗体(PAIgA、PAIgG)、采取单克隆抗体特异性俘获血小板抗原(MAIPA法)测定血小板特异性抗体抗血小板膜糖蛋白抗体(GPIIb/IIIa、GPIb/IX),全自动血液分析仪测定血小板计数。结果 PAIgA、PAIgG、GPIIb/IIIa、GPIb/IX、血小板计数对照组分别为(4.22±0.78)ng/107PA、(23.17±3.46)ng/107PA、(0.33±0.01)A值、(0.31±0.01)A值、(137.82±42.37)×109/L,ITP组分别为(45.58±5.32) ng/107PA、(243.34±12.21)ng/107PA、(0.42±0.06)A值、(0.41±0.05)A值、(35.50±21.35)×109/L,两组以上各项指标之间比较均差异有统计学意义(P<0.05);ITP组血小板计数与特异性抗体GPIIb/IIIa、GPIb/IX之间具有负相关,相关系数分别为-0.24(P<0.05)、-0.31(P<0.05)。结论在ITP患儿中血小板相关抗体及特异性抗体均有明显升高,呈现阳性表达,ELISA 法、MAIPA法联合检测有助于鉴别免疫性与非免疫性血小板减少型紫癜,能提高早期诊断率,并且有助于指导临床治疗。%Objective To understand the expression of PAIg in patients with idiopathic thrombocytopenic purpura (ITP) and evalu-ate its clinical significance in ITP. Methods 40 children with ITP were selected as the subjects, and 20 normal children underwent outpatient physical examination during the same period were selected as the control group. Enzyme linked immunosorbent assay (ELISA) was used to detect platelet-associated antibodies (PAIgA, PAIgG), monoclonal antibody immobilization of platelet antigen assay (MAIPA) was adopted to determine platelet specific antibodies against platelet membrane glycoprotein antibody (GPIIb/IIIa, GPIb/IX), and full

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

    Institute of Scientific and Technical Information of China (English)

    方治; 张翼鷟; 蔡挺; 李克强; 余静; 罗央清; 赵海丰

    2012-01-01

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

  2. 儿童慢性特发性血小板减少性紫癜与幽门螺杆菌感染的临床研究%CLINICAL STUDY ON CHRONIC IDIOPATHIC THROMBOCYTOPENIC PURPURA AND HELICOBACTOR PYLORI IN CHILDREN

    Institute of Scientific and Technical Information of China (English)

    武国霞; 彭建霞; 张建菊; 武建辉; 任蓉

    2012-01-01

    [Objective] To investigate the relationship of chronic idiopathic thrombocytopenic purpura (cITP) and heli-cobactor pylori (HP) in children, and observe the clinical effect on HP eradiating therapy in children with cITP. [Methods] Children with cITP were delected by 13C urea breath teat. 14 children, who had positive results, were randomly divided into study group (n = 9) and control group (n = 5). The children were treated with immunotherapy and HP eradiating therapy in study group, the children were only treated with immunotherapy in control group. Clinical effects and the platelets before treatment and in the 7th day, 1st month, 2nd month and 3rd month after treatment were compared between groups. [Results] 14 of 66 children with cITP were positive for HP infection (21.2%). The clearances of HP were achieved in 7 out of 9 children with FTP in study group (77.8%). The analysis showed the platelets in the study group were higher than in the control group (P < 0.01) in the 7ui day, 1st month, 2nd month, 3rd month after treatment. The analysis of clinical effects of children showed the study group were better than the control group (P < 0.05). [Conclusion] The study had shown the prevalencde of HP infection in cITP children was 21.2%, the bacterium eradication were obtained in 7 of 9 HP positive children (77.8%) in study group. It is helpful to increase the platelets count and get clinical effects in cITP children combined with HP eradication treatment%[目的]探讨儿童慢性特发性血小板减少性紫癜(cITP)与幽门螺杆菌(HP)的关系,观察根除HP对儿童cITP的疗效.[方法]对cITP患儿进行13℃尿素呼吸试验检测,将确诊HP感染的14例患儿随机分成研究组(9例)和对照组(5例).研究组采用免疫治疗加根除HP治疗,而对照组只采用免疫治疗,比较两组治疗前以及治疗后d7、第1,2,3月血小板的数目和临床效果.[结果]66例cITP患儿中有HP感染14例(21.2%),研究组的9例中,其中7

  3. Predict Value of Serum B-Cell Activating Factor on Treatment Effectiveness of Children with Idiopathic Thrombocytopenic Purpura in Diagnosis%血清B淋巴细胞激活因子水平对初诊特发性血小板减少性紫癜患儿疗效的预测价值

    Institute of Scientific and Technical Information of China (English)

    葛小丽; 韩志君; 过毅; 陈洪敏

    2012-01-01

    目的 评价血小板减少性紫癜(ITP)患儿初诊时血清B淋巴细胞激活因子(BAFF)对其疗效的预测价值.方法 采用ELISA法检测初诊37例ITP患儿与37例隐睾及腹股沟疝患儿(对照组)血清BAFF水平,分析ITP患儿血清BAFF水平与其血小板计数、治疗效果的关系.采用受试者工作曲线法(ROC)分析血清BAFF水平对治疗效果的预测价值.结果 ITP患儿血清BAFF水平较对照组增高(P=0.02),且与血小板计数呈负相关(R2=0.39,P<0.01).初诊时血清BAFF水平较低的患儿,其治疗效果相对较好(P<0.01).采用初诊血清BAFF水平预测患儿治疗效果,其曲线下面积(AUC)为0.85(95% CI0.72 ~0.97,P<0.01),当血清BAFF值为850 ng·L-1时,预测敏感性为0.71(95% CI 0.48 ~0.89),特异性为0.88(95% CI 0.62 ~0.98).结论 初诊血清BAFF水平有助于ITP患儿最终治疗效果的预测.%Objective To evaluate the predictive efficiency of serum B - cell activating factor (BAFF) on the treatment effectiveness in children with idiopathic thrombocytopenic purpura (ITP) in diagnosis. Methods Serum BAFF level of 37 children with ITP and controls ( consisted of cryptorchidism and inguinal hernia) were detected by enzyme - linked immunosorbent assay (ELISA). The correlations between serum BAFF and platelet count and treatment effectiveness were analyzed. The predictive efficiency of BAFF on ITP patients' treatment effectiveness was evaluated by receiver operating characteristics (ROC) curve analysis. Results Increased serum BAFF, which negatively correlated with platelet count, was observed in children with ITP. Higher serum BAFF at diagnosis was associated with worse treatment effectiveness, and vice versa. For treatment effectiveness predicting, the area under curve (AUC) for serum BAFF level was 0. 85 (95%CI0.72 -0.97, P<0.01). At the cut-off value of 850 ng·L-1 , the sensitivity and specificity for serum BAFF level were 0.71 (95%CI 0.48 -0. 88) and 0. 88 (95% CI0.62 - 0

  4. Clinical Analysis and Clinical Economic Evaluation of Two Treatments for Children with Idiopathic Thrombocytopenic Purpura%特发性血小板减少性紫癜2种治疗方案比较及经济学评价

    Institute of Scientific and Technical Information of China (English)

    韩金芬; 何志旭; 张昌华

    2012-01-01

    Objective To investigate the clinical efficacy and economic evaluation of 2 treatments for 70 children with idiopathic throm-bocytopenic purpura(ITP). Methods The glucocorticoid (GC) alone treatment and GC combined with intravenous immunoglobulin(IVIG) treatment were retrospectively analyzed, and the economic evaluation was made by cost - effectiveness analysis methods. The data were analyzed by SPSS 14.0 software. Results There was no statistical difference in the side effects and follow - up visits between 2 groups. The cases with platelet counts of (10 -25) x 109 L ' or less than 10 X 109 L"1 and severe bleeds in IVIG plus GC treatment group were more than that in GC treatment group. The days which the platelets reached the normal level from the 1" day to the 7' day, the effective rate and significant effective rate in IVIG plus GC treatment group were higher or better than that in GC treatment group. The result of economic evaluation in GC treatment group was better than that in IVIG plus GC treatment group. Conclusions The cases with platelet counts of (10 -25) x 10' L1 or less than 10 x 109 L1, severe bleeds should be treated with IVIG plus GC treatment, while the others with GC treatment. Many factors should be considered to choose the ways of clinical treatment, including the clinical curative effect and economics.%目的 探讨70例特发性血小板减少性紫癜(ITP)患儿中2种治疗方案的临床疗效及经济学评价.方法 对静脉用丙种球蛋白(IVIG)与地塞米松(GC)静脉联合治疗组及单用GC治疗组进行回顾性分析,并应用成本-效果分析法进行临床经济学评价.采用SPSS 14.0软件进行统计学分析.结果 2组ITP患儿治疗后不良反应、回访情况比较差异均无统计学意义.IVIG+ GC组PLT下降极重度[ (10 ~25) ×109 L-1]、重度(<10×109L-1)、严重出血例数均高于GC组.2组治疗第1-7天PLT上升至正常天数比较,有效率、显效率比较,IVIG+ GC组均优于GC组.应用

  5. 重组人血小板生成素治疗难治性特发性血小板减少性紫癜的循证评价%Evidence-based Evaluation of Recombinant Human Thromobopoietin for Refractory Idiopathic Thrombocytopenic Purpura

    Institute of Scientific and Technical Information of China (English)

    黄琳; 任晓蕾; 刘一; 李玉珍

    2011-01-01

    目的:评价重组人血小板生成素(rhTPO)治疗成人慢性难治性特发性血小板减少性紫癜(ITP)的有效性与安全性.方法:采用循证医学的文献分析评价方法,计算机检索CBM、CNKI、MEDLINE、EMbase、Cochrane图书馆临床对照试验资料库等数据库,收集rhTPO治疗成人慢性难治性ITP的临床研究证据.结果:检索到rhTPO治疗成人慢性难治性ITP的文献有:自身前后对照试验5篇,随机对照试验2篇.按Jadad量表评分:1篇随机对照研究质量较高,为4分,其余文献均小于3分.5项自身对照研究结果基本一致,认为rhTPO可升高成人难治性ITP患者的血小板计数,且不良反应轻微.2项随机对照试验认为rhTPO联合达那唑治疗难治性ITP的效果明显优于单用达那唑,且对于单用达那唑治疗无效者,接受rhTPO治疗后血小板计数亦明显升高.结论:现有研究显示rhTPO在治疗成人慢性难治性ITP方面疗效确切、安全性好,但试验设计存在不足,尚需进行设计良好的大样本研究.%OBJECTIVE: To evaluate the efficacy and safety of recombinant human thromobopoietin (rhTPO) for adult chronic refractory idiopathic thrombocytopenic purpura (ITP).METHODS: By literature analysis based on evidence-based medicine, clinical trials about rhTPO in the treatment of adult chronic refractory ITP were retrieved from CMB, CNKI, MEDLINE, EMbase and Cochrane library.RESULTS: Of collected pertinent literature about rhTPO in the treatment of adult chronic refractory ITP, five were self-control studies and two were randomized controlled trials (RCTs).The quality of seven studies was evaluated according to Jadad scale, of which one scored four points, and the others below three points.The five self-control studies all supported that rhTPO could effectively elevate the platelet counts and was safe for patients with adult chronic refractory ITP.Two RCTs indicated that rhTPO combined with danazol was more effective than danazol for

  6. 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

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

    Institute of Scientific and Technical Information of China (English)

    张付华; 刘珍

    2014-01-01

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

  8. 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.

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

    DEFF Research Database (Denmark)

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

    2009-01-01

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

  10. [Clinical analysis of 200 cases of idiopathic thrombocytopenic purpura].

    Science.gov (United States)

    García-Stivalet, Lilia Adela; Muñoz-Flores, Aarón; Montiel-Jarquín, Alvaro José; Barragán-Hervella, Rodolfo Gregorio; Bejarano-Huertas, Ruth; García-Carrasco, Mario; López-Colombo, Aurelio

    2014-01-01

    INTRODUCCIÓN: la púrpura trombocitopénica idiopática se caracteriza por la extravasación de sangre en el tejido subcutáneo, membranas, mucosas o piel, que puede generar manifestaciones clínicas de sangrado como lesiones equimóticas, petequias de aparición brusca, epistaxis, gingivorragia y complicaciones graves como hemorragia intracraneal, debido a destrucción plaquetaria mediada por anticuerpos dirigidos contra la superficie de las plaquetas. El objetivo de este informe es presentar las características clínicas de los pacientes con púrpura trombocitopénica idiopática en un hospital de tercer nivel de atención, con la finalidad de tener estadísticas para estudios analíticos posteriores. MÉTODOS: se realizó un estudio descriptivo de 200 pacientes atendidos en el servicio de hematología con diagnóstico de púrpura trombocitopénica idiopática. Se describen sus manifestaciones clínicas, el diagnóstico y el tratamiento médico y quirúrgico empleados.

  11. Supraclavicular lymph node tuberculosis presenting with immune thrombocytopenic purpura

    Institute of Scientific and Technical Information of China (English)

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

    2007-01-01

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

  12. Platelet matching for alloimmunized patients

    Institute of Scientific and Technical Information of China (English)

    S H.Hsu

    2010-01-01

    @@ Platelets play an essential role in blood coagulation,hemostasis and maintenance of vascular integrity.Platelets are utilized primarily to prevent or treat bleeding in thrombocytopenic patients and patients with impaired platelet production in the bone marrow and/or with dysfunctional platelets.In current practice,platelet transfusion begins with randomly selected platelet products:either pooled platelets prepared from whole blood derived platelets; or single donor platelets prepared by apheresis procedures.

  13. Penile Involvement and Henoch-Schönlein Purpura: A Case Report

    Directory of Open Access Journals (Sweden)

    Önder YAVAŞCAN

    2012-01-01

    Full Text Available Henoch-Schönlein purpura (HSP, characterized by non-thrombocytopenic purpura accompanied by arthritis/arthralgia, gastrointestinal and genitourinary system involvement is the most common vasculitis in childhood. Associated penile involvement is very rare in patients with genitourinary system involvement. We report a 4.5 year-old boy with penile involvement and arthritis after a shortduration remission of HSP. Prednisolone (1mg/kg/day, orally and conservative measures resulted in complete recovery within five days. In conclusion, steroid treatment could be administered in HSP children with penile involvement although controversial.

  14. Fetal/neonatal alloimmune thrombocytopenia.

    Science.gov (United States)

    Sachs, Ulrich J

    2013-01-01

    In fetal/neonatal alloimmune thrombocytopenia (FNAIT), antibodies against paternal antigens present on fetal platelets are produced by the mother. These antibodies will cross the placenta and can cause thrombocytopenia of the unborn. One severe consequence of thrombocytopenia is intracranial bleeding which may lead to death or severe sequelae. FNAIT index cases in one family are usually detected at birth only since antenatal screening programmes have not been installed so far. Subsequent pregnancies of immunized mothers may require special diagnostic and prophylactic interventions, and interdisciplinary counselling and treatment involving obstetricians, pediatricians and immunohematologists may prove useful for optimized care. This short review covers pathogenesis, clinical presentation, diagnostic, and therapeutic options in FNAIT.

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

    Directory of Open Access Journals (Sweden)

    Luan Li

    2013-01-01

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

  16. Anti-HPA-1b Mediated Posttransfusion Purpura: A Case Report

    Directory of Open Access Journals (Sweden)

    O. P. Arewa

    2013-01-01

    Full Text Available Posttransfusion purpura (PTP is an uncommon, but potentially fatal, transfusion reaction characterized by profound thrombocytopenia and bleeding. PTP is caused by alloimmunization to human platelet specific antigens following blood component transfusion. Although there is evidence of a wide serological spectrum of culprit antibodies implicated, Anti-human-platelet-antigen- (HPA- 1a is the most common antibody in cases reported. We report a case of posttransfusion purpura in an African American. The patient was negative for HPA-1a antibodies, but anti-HPA-1b was identified with a platelet phenotype of HPA-1a/HPA-1a. Although less common, HPA-1b antibody may be an important consideration in posttransfusion purpura diagnosed in patients of African descent.

  17. 不同诱因致获得性血栓性血小板减少性紫癜20例临床研究%Clinical study on 20 cases of acquired thrombotic thrombocytopenic purpura caused by different inducements

    Institute of Scientific and Technical Information of China (English)

    王吉刚; 周凡; 刘彦琴; 刘景华; 白颖; 张海婷; 孟广晗; 佟丹江; 李敏燕

    2016-01-01

    目的:通过分析获得性血栓性血小板减少性紫癜(TTP)患者的临床资料,提高对TTP的认识。方法回顾性分析20例获得性T T P患者的临床特征、实验室检查、治疗策略及转归。结果20例获得性TTP患者中,16例有诱发因素,包括:自身免疫异常、系统性红斑狼疮、抗磷脂抗体综合征、病毒感染、细菌感染、药物、淋巴瘤及异基因造血干细胞移植。初诊时仅5例(25%)表现为典型“五联”征,18例患者(90%)伴血小板减少,14例(70%)伴微血管病性溶血性贫血,17例(85%)伴神经精神症状,15例(75%)伴发热,8例(40%)伴肾功能不全。16例(80%)网织红细胞百分比升高,18例(90%)检出破碎红细胞,其中3例同时检出小球形红细胞;20例(100%)患者乳酸脱氢酶均升高。20例患者均接受了以血浆疗法(血浆置换或/和血浆输注)为主的治疗,其中9例患者接受了糖皮质激素治疗,2例患者接受了环磷酰胺治疗,2例接受人免疫球蛋白治疗,4例患者接受小剂量利妥昔单抗治疗(100 mg每周1次,连用4次)。11例(55%)患者完全缓解,3例(15%)患者部分缓解,总有效率为70%;4例(15%)患者死亡,治疗有效的14例患者中3例(21%)复发。结论TTP诊断依赖临床资料的综合分析,早期诊断和以血浆疗法为主要手段的早期治疗可改善患者的预后。%Objective To enhance the recognition of thrombotic thrombocytopenic purpura(TTP) by analyzing the clinical data of acquired TTP .Methods The clinical features ,laboratory detection ,treatment strategy and outcome of 20 patients with ac‐quired TTP were retrospectively analyzed .Results Among 20 patients with acquired TTP ,16 cases had the inducing factors ,inclu‐ding abnormal autoimmune ,systemic lupus erythematosus(SLE) ,antiphospholipid antibody syndrome(APS) ,viral infection

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

    Institute of Scientific and Technical Information of China (English)

    叶华觅; 郝建萍; 赵芳; 王欢; 郭新红

    2013-01-01

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

  19. Clinical efficacy of lower dose rituximab for chronic refractory immune thrombocytopenic purpura%小剂量利妥昔单抗治疗复发难治性原发免疫性血小板减少症的临床观察

    Institute of Scientific and Technical Information of China (English)

    李燕; 王晓敏; 毛敏; 张晓燕; 富玲; 艾合买江; 张莲兴

    2012-01-01

    目的 探讨小剂量利妥昔单抗治疗复发难治性原发免疫性血小板减少症(ITP)的疗效及安全性.方法 研究纳入20例复发难治性ITP患者,给予利妥昔单抗100 mg静脉滴注,每周1次,连用4周,动态观察血常规、肝肾功能及凝血功能.采用流式细胞术检测治疗前后CD3+、CD4+、CD8+、CD19+淋巴细胞数.免疫比浊法定量检测治疗前后血清免疫球蛋白(IgG、lgM、IgA)水平.用ELISA方法检测血小板膜糖蛋白抗体.治疗前后各项检测指标比较采用配对t检验.结果 治疗后中位起效时间为18d,PLT达峰值时间为(24±7)d.治疗后PLT[(124±106)×109/L]显著高于治疗前[(13±5)×109/L](P<0.01).11例(55%)患者达完全反应(CR),4例(20%)有效(R),5例(25%)无效(NR).中位疗效持续时间为8(5~23)个月.治疗前后外周血WBC、HGB、血清免疫球蛋白以及CD3+、CD4+、CD8+淋巴细胞数无明显变化,CD19+淋巴细胞数治疗后[(50.53±29.11)×106/L]较治疗前[(125.65±14.12)×106/L]明显下降(P<0.01).3例患者治疗前血小板自身抗体检测阳性,治疗后均为阴性.1例患者在首次输注利妥昔单抗后发生轻微不良反应.结论 小剂量利妥昔单抗是一种治疗复发难治性ITP安全有效的药物,但其最佳用药方案、长期疗效以及不良反应有待临床进一步观察验证.%Objective To investigate the efficacy and safety as well as the effects of lower dose of rituximab on B-lymphocytes,serum immunoglobulin,and platelet glycoprotein-specific antibodies in patients with chronic refractory immune thrombocytopenic purpura (ITP).Methods Twenty chronic refractory ITP patients,median age 47(20 to 60) years old,received intravenous rituximab at the dose of 100mg once weekly for 4 consecutive weeks.Laboratory studies included complete blood cell count,regular monitoring of liver and kidney functions,blood coagulation and serum concentrations of IgG,IgM and IgA. CD3 +,CD4 +,CD8 +,CD19 +,CD20 + cell

  20. 利妥昔单抗治疗糖皮质激素无效的特发性血小板减少性紫癜疗效观察%Rituximab treatment for adults with steroid-resistant idiopathic thrombocytopenic purpura

    Institute of Scientific and Technical Information of China (English)

    王文; 俞庆宏; 张海燕; 初晓霞; 陈峰; 张春青; 周郁鸿; 侯明

    2008-01-01

    Objective To investigate the efficacy and safety as well as the effects of rituximab on B-lymphocytes and anti-platelet glycoprotein-specific antibodies,in patients with steroid-resistant idiopathic thrombocytopenic purpura(ITP).Methods Twelve steroid-resistant ITP patients,16 to 54 years old,received intravenous rituximab at the dose of 375 mg/m2 once-weekly for 4 weeks.Lab studies included CBC,serum concentrations of IgG,IgM and IgA.CD+3,CD+4,CD+8,CD+19,CD+20 cell numbers were assayed by flow cytometry and anti-platelet glycoprotein-specific antibodies(GP Ⅱ b/Ⅲ a,GP Ⅰ b/Ⅸ)were assayed by monoclonal antibody-specific immobilisation of platelet antigens prior to and following rituximab therapy.Results A complete response(platelet counts ≥100×109/L)was observed in 4 cases,a partial response (platelet counts between 50 and 100×109/L)in 3 cases,a minor response(platelet counts between 30 and 50×109/L)in 2 cases,and non response(platelet counts<30×109/L)in 3 cases.Responses were sustained 0.5 to 12 months(median 5 months).After 4 weeks of rituximab therapy,anti-platelet glycoprotein-specific antibodies(GP Ⅱ b/Ⅲ a,GP Ⅰ b/Ⅸ)disappeared except one NR patient and CD+19/CD+20 cells were almost depleted in all patients(295.0±86.4)×106/L vs(4.1±2.2)×106/L(P<0.01).As expected,the T cell counts,and the serum concentrations of IgG,IgM and IgA were not changed after therapy.No severe side effects were observed.Conclusion Rituximab may be an effective and safe treatment for adults with steroid-resistant ITP.%目的 探讨利妥昔单抗治疗特发性血小板减少性紫癜(ITP)的疗效、安全性及治疗前后B细胞、血小板膜糖蛋白(GP)特异性自身抗体的变化.方法 利妥昔单抗(375 mg/m2,每周1次,连用4周)治疗12例糖皮质激素治疗无效的ITP患者,监测治疗前后的血常规、血清免疫球蛋白定量(IgG、IgM、IgA)、血小板GPⅡb/Ⅲa和(或)GP Ⅰ b/Ⅸ特异性自身抗体、CD+3、CD+4、CD+8、CD+19

  1. 重组人血小板生成素治疗特发性血小板减少性紫癜的临床疗效%Clinical efficacy and safety of rhTPO in the treatment of idiopathic thrombocytopenic purpura: a randomized controlled trial

    Institute of Scientific and Technical Information of China (English)

    张春青; 王琳; 秦平; 陈峰; 陈春燕; 侯明

    2011-01-01

    目的 评价重组人血小板生成素(rhTPO)对糖皮质激素治疗无效的成人特发性血小板减少性紫癜(ITP)的有效性与安全性.方法 选择对糖皮质激素治疗无效的ITP患者74例,分为rhTPO+达那唑组(观察组)37例,达那唑组(对照组)37例.观察组皮下注射rhTPO 1.0μg/kg(300U/kg),1次/d,共14 d,停用rhTPO后再观察14 d.对照组口服达那唑14 d后,如血小板≤20×109/L可皮下注射rhTPO(1.0μg/kg),1次/d,14 d,停用rhTPO后观察14 d.两组在整个试验阶段口服达那唑200mg/次,3次/d.比较两组前14 d血小板计数的最高值和不同时间点血小板计数值的曲线下面积及有效率,比较对照组应用rhTPO前后的血小板计数及其差值.结果 观察组前14 d血小板计数和升高值均显著高于对照组(P<0.01).14 d内血小板计数值的曲线下面积中位数明显高于对照组(P<0.001);观察组rhTPO治疗14d内显效者(血小板计数升高至≥100×109/L)(16例、43.24%)明显高于对照组(3例、8.10%)(P<0.01).观察组良效者(血小板计数升高≥30×109/L)(9例、24.32%)与对照组(10例、27.03%)差异无统计学意义(P>0.01).观察组近期有效observation率(显效+良效)明显高于对照组(P<0.05).对照组14 d血小板升高未达有效的患者给予rhTPO治疗后血小板计数与使用rhTPO前相比明显升高,有效率达到62.50%.两组均无严重不良事件发生.结论 rhTPO治疗糖皮质激素治疗无效的ITP患者安全、有效.%Objective To investigate the efficacy and safety of recombinant human thrombopoietin (rhTPO) in the treatment of adult steroid-resistant idiopathic thrombocytopenic purpura (ITP). Methods 74 patients with steroid-resistant ITP were randomly divided into the observation group and the control group, 37 in each group. Patients in the observation group were treated with 1.0 μg/kg · d of rhTPO, ih(300 U/kg · d)and danazol, 600 mg/d, three times a day, for 14 d. Patients in the control group were

  2. Alloimmune refractoriness to platelet transfusions.

    Science.gov (United States)

    Sandler, S G

    1997-11-01

    Patients who are transfused on multiple occasions with red cells or platelets may develop platelet-reactive alloantibodies and experience decreased clinical responsiveness to platelet transfusion. This situation, conventionally described as "refractoriness to platelet transfusions," is defined by an unsatisfactory low post-transfusion platelet count increment. If antibodies to HLAs are detected, improved clinical outcomes may result from transfusions of HLA-matched or donor-recipient cross-matched platelets. Because refractoriness is an expected, frequently occurring phenomenon, prevention of HLA alloimmunization is an important management strategy. Prevention strategies include efforts to decrease the number of transfusions, filtration of cellular components to reduce the number of HLA-bearing leukocytes, or pretransfusion ultraviolet B irradiation of cellular components to decrease their immunogenicity. Other investigational approaches include reducing the expression of HLAs on transfused platelets, inducing a transient reticuloendothelial system blockade by infusions of specialized immunoglobulin products, or transfusing semisynthetic platelet substitutes (thromboerythrocytes, thrombospheres) or modified platelets (infusible platelet membranes, lyophilized platelets).

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

    Science.gov (United States)

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

    2015-01-01

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

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

    Institute of Scientific and Technical Information of China (English)

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

    2009-01-01

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

  5. The clinical significance of immune-related marker detection in idiopathic thrombocytopenic purpura%特发性血小板减少性紫癜患者免疫相关指标的检测及其临床意义

    Institute of Scientific and Technical Information of China (English)

    陈剑芳; 杨林花; 冯建军; 常丽贤; 刘秀娥; 鹿育晋

    2010-01-01

    Objective To assess the clinical significance of detecting the immune markers in idiopathic thrombocytopenic purpura (ITP). Methods The frequencies of circulating B cells secreting platelet-specific antibody, platelet-specific antibody, the percentage of T lymphocyte subsets, the percentage of reticulated platelet and the level of thrombopoietin in 64 ITP patients and 31 healthy controls were measured with enzyme-linked immunospot assay (ELISPOT),modified monoclonal antibody immunobilization of platelet antigens assay (MAIPA), flow cytometry and sandwich enzyme-linked innnunosorbent assay respectively. Results Compared with the controls[1.3 ± 0. 5/105 peripheral blood mononuclear cell (PBMC), (0.33±0.06,0.41±0.03), (22.08±4.54)% and (8.19±2.46)%], the frequencies of circulating B cells secreting platelet-specific antibody (7.6±4.6/105 PBMC in acute ITP group, 5.3±3.0/105 PBMC in chronic ITP group), platelet-specific antibody (including the anti-GP Ⅱ b/Ⅲa antibody, anti-GP Ⅰ b/X antibody) (0.51 ±0.11, 0.48±0.06 in acute ITP group; 0.49±0.10,0.46±0.09 in chronic ITP group), the percentage of CD8+ T Lymphocyte (27.09±9.86 ) %, the percentage of reticulated platelet in ITP patients[the megakaryocyte cytosis group (24. 85 ± 19. 18)%, the normal megakaryocyte group (23.89±18.90)%]were significantly increased ( all P<0.05).The frequencies of circulating B cells secreting platelet-specific antibody in acute ITP patients were notably increased (P<0.05) compared to the chronic ITP patients. In T lymphocyte subsets, the percentage of CD3+T lymphocyte and CD4+ T lymphocyte and the ratio of CD4+/CD8+ in the patients with ITP[(60.88±14.59)%, (28.41±10.55)%, 1.18±0.59]were notably decreased than those in the healthy controls [(69.89±6.43)%, (35.38±5.05) %, 1.64±0.29, P<0.05]. There was no apparent difference of the level of thrombopoietin between ITP patients with megakaryocyte cytosis (72. 09 ± 41.64 ) and health controls (75.37± 26. 32, P > 0

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

    Institute of Scientific and Technical Information of China (English)

    王莉; 张连生; 柴晔; 曾鹏云; 吴重阳

    2012-01-01

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

  7. Hematological outcome in neonatal alloimmune hemolytic disease

    NARCIS (Netherlands)

    Rath, Mirjam Eva Aafke

    2013-01-01

    This thesis focuses on several aspects related to the hematological outcome of infants with hemolytic disease of the fetus and newborn (HDFN) due to red blood cell alloimmunization, including pathogenesis and management of the disease. The presence of leukocytopenie and thrombocytopenia support the

  8. Purpura Fulminans following Thermal Injury

    Directory of Open Access Journals (Sweden)

    Jiongyu Hu

    2013-01-01

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

  9. Bathtub suction-induced purpura.

    Science.gov (United States)

    Landers, Maeran C; Schroeder, Theresa L

    2004-01-01

    We report two cases of bathtub suction-induced purpura. Two girls, ages 9 and 14 years, developed recurring purpura "spontaneously" in the same lower back location and in the same U-shaped distribution. The patients had similar histopathologic findings, with extravasated erythrocytes and a sparse superficial inflammatory infiltrate. After extensive evaluation, one patient eventually admitted to self-inducing her lesions by creating suction against a bathtub. The second patient denied self-inflicting her lesions; however, her purpura improved when she stopped taking baths.

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

    Directory of Open Access Journals (Sweden)

    Hauck-Dlimi B

    2014-08-01

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

  11. 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.

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

    Institute of Scientific and Technical Information of China (English)

    王莉; 张连生; 柴晔; 曾鹏云; 吴重阳

    2012-01-01

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

  13. Advances in alloimmune thrombocytopenia: perspectives on current concepts of human platelet antigens, antibody detection strategies, and genotyping.

    Science.gov (United States)

    Hayashi, Tomoya; Hirayama, Fumiya

    2015-07-01

    Alloimmunisation to platelets leads to the production of antibodies against platelet antigens and consequently to thrombocytopenia. Numerous molecules located on the platelet surface are antigenic and induce immune-mediated platelet destruction with symptoms that can be serious. Human platelet antigens (HPA) cause thrombocytopenias, such as neonatal alloimmune thrombocytopenia, post-transfusion purpura, and platelet transfusion refractoriness. Thirty-four HPA are classified into 28 systems. Assays to identify HPA and anti-HPA antibodies are critically important for preventing and treating thrombocytopenia caused by anti-HPA antibodies. Significant progress in furthering our understanding of HPA has been made in the last decade: new HPA have been discovered, antibody-detection methods have improved, and new genotyping methods have been developed. We review these advances and discuss issues that remain to be resolved as well as future prospects for preventing and treating immune thrombocytopenia.

  14. 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.

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

    Science.gov (United States)

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

    2016-02-01

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

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

    Science.gov (United States)

    O'Connell, B A

    1995-01-01

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

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

    Directory of Open Access Journals (Sweden)

    Gérald Bertrand

    2016-07-01

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

  18. A clinical study on incipient patients with acquired thrombotic thrombocytopenic purpura treated with plasma therapy and low-dose rituximab%血浆疗法联合小剂量利妥昔单抗治疗初发获得性 TTP

    Institute of Scientific and Technical Information of China (English)

    王吉刚; 周凡; 刘彦琴; 刘景华; 白颖; 张海婷; 孟广晗; 佟丹江; 李敏燕

    2015-01-01

    目的:探讨血浆疗法联合小剂量利妥昔单抗治疗初发获得性血栓性血小板减少性紫癜( TTP )的疗效。方法回顾性分析6例初发获得性TTP患者的临床特征、实验室检查,观察血浆疗法联合小剂量利妥昔单抗治疗反应及转归。结果本组6例获得性TTP患者中,4例有诱发因素,包括自身免疫性异常、系统性红斑狼疮及抗磷脂抗体综合征。初诊时仅1例表现为典型“五联”征,5例表现为“三联”征;5例网织红细胞百分比升高,6例均检出破碎红细胞,其中,1例同时检出小球形红细胞;6例初诊时乳酸脱氢酶均升高,4例总胆红素升高,但仅1例肌酐升高。6例患者均接受了血浆疗法(血浆置换或/和血浆输注)联合小剂量利妥昔单抗(100 mg每周1次,连用4次)治疗,结果6例患者均达CR,中位随访12(6~18)个月,6例患者均无复发。结论 TTP早期诊断依赖临床资料的综合分析,血浆疗法联合小剂量利妥昔单抗可有效治疗初发获得性TTP。%Objective To enhance the understanding of thromboticthrombocytopenic purpura( TTP) and to evaluate the efficacy of plasma therapy and low-dose rituximab in treating incipient patients with acquired TTP,and the clinical data of acquired TTP were investigated. Methods The clinical features,laboratory characteristics,treatment and outcome of six patients with TTP were retro-spectively analyzed and investigated. All 6 cases received plasma therapy and low-dose rituximab which was administered at the dose of 100 mg once weekly,for 4 consecutive weeks. Results Of all 6 patients with TTP,4 cases had causative factors,including abnor-mal autoimmune,systemic 1upus erythematosus(SLE)and antiphospholipid antibody syndrome(APS). When the patients were diag-nosed primarily,only one of them had typical pentalogy of TTP,while,the others had triad. Five patients had high percentage of retic-ulocyte. The schistocytes of were detected in the peripheral blood

  19. 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.

  20. Alloimmunization in multitransfused liver disease patients: Impact of underlying disease

    Directory of Open Access Journals (Sweden)

    Meenu Bajpai

    2016-01-01

    Full Text Available Introduction: Transfusion support is vital to the management of patients with liver diseases. Repeated transfusions are associated with many risks such as transfusion-transmitted infection, transfusion immunomodulation, and alloimmunization. Materials and Methods: A retrospective data analysis of antibody screening and identification was done from February 2012 to February 2014 to determine the frequency and specificity of irregular red-cell antibodies in multitransfused liver disease patients. The clinical and transfusion records were reviewed. The data was compiled, statistically analyzed, and reviewed. Results: A total of 842 patients were included in our study. Alloantibodies were detected in 5.22% of the patients. Higher rates of alloimmunization were seen in patients with autoimmune hepatitis, cryptogenic liver disease, liver damage due to drugs/toxins, and liver cancer patients. Patients with alcoholic liver disease had a lower rate of alloimmunization. The alloimmunization was 12.7% (23/181 in females and 3.17% (21/661 in males. Antibodies against the Rh system were the most frequent with 27 of 44 alloantibodies (61.36%. The most common alloantibody identified was anti-E (11/44 cases, 25%, followed by anti-C (6/44 cases, 13.63%. Conclusion: Our findings suggest that alloimmunization rate is affected by underlying disease. Provision of Rh and Kell phenotype-matched blood can significantly reduce alloimmunization.

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

    Institute of Scientific and Technical Information of China (English)

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

    2014-01-01

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

  2. Aloimunização Alloimmunization

    Directory of Open Access Journals (Sweden)

    Eduardo Baiochi

    2009-06-01

    Full Text Available Aloimunização é a formação de anticorpos quando há a exposição do indivíduo a antígenos não próprios, como ocorre, por exemplo, na transfusão de sangue incompatível e nas gestantes, cujos fetos expressam em suas células sanguíneas antígenos exclusivamente de origem paterna. Este artigo se restringe à aloimunização contra antígenos eritrocitários em pacientes obstétricas. Quase todos os anticorpos antieritrocitários podem ser enquadrados em um dos 29 sistemas de grupos sanguíneos já reconhecidos, sendo os mais comumente implicados na doença hemolítica perinatal o anti-D, anti-c e anti-Kell, seguidos por anti-C, anti-E, anti e, anti-Fyª e anti-Jkª. A pesquisa de anticorpos irregulares permite o diagnóstico de indivíduos aloimunizados e modernas técnicas genéticas têm melhor caracterizado estas pacientes para a profilaxia e segmento pré-natal. O tradicional acompanhamento das gestações de risco para a doença hemolítica perinatal, com a espectrofotometria do líquido amniótico e a transfusão intraperitoneal, vem rapidamente sendo substituído pela doplervelocimetria na artéria cerebral média e a transfusão intravascular guiada por ultrassonografia em tempo real. É possível também citar como avanços melhorias nos materiais e na qualidade do sangue transfundido, que, em conjunto, têm elevado a sobrevivência de fetos acometidos. Indubitavelmente, a correta aplicação da profilaxia com uso do anti-D é exitosa com potencial para reduzir os casos de aloimunização.Alloimmunization is the formation of antibodies when there is an exposition of the individual to non-self antigens, as it occurs, for example, in the transfusion of incompatible blood and pregnancies, in whom the fetus express in its sanguineous cells antigens exclusively of paternal origin. This article is restricted to the alloimmunization against erythrocytes antigens in obstetric patients. Almost all the anti-erythrocytes antibodies can

  3. [Treatment of chronic refractory idiopathic thrombocytopenia purpura. 10 years experience at the Salvador Zubiran National Institute of Nutrition].

    Science.gov (United States)

    Pita-Ramírez, L; Hurtado-Monroy, R; Labardini-Méndez, J

    1992-01-01

    A total of 126 patients with chronic idiopathic thrombocytopenic purpura were diagnosed from January 1980 to January 1990 in our institute. In this group of patients, 21 were refractory to prednisone therapy, splenectomy or both, or had had a relapse after a good response with these treatments. They were given other therapies. There was enough information for evaluation in 16 of the 21 patients. The treatment responses were classified according to the post-therapy platelet counts: complete response (CR) = > 150 x 10(9)/L for more than three months; partial response (PR) = 50-150 x 10(9)/L for more than three months; any response (AR) = CR + PR; no response (NR) = < 50 x 10(9)/L. There were 15 women and one male. The median age was 41 years (range 11 to 65). 6-mercaptopurine was given in all patients with CR = 31.2%, PR = 18.8%, AR = 50% and NR = 50%. Seven patients received cyclophosphamide with CR = 28.6%, PR = 14.3%, AR = 42.9% and NR = 57%. Vincristine was given in four patients with only one PR. Interferon alpha 2B was given in four patients with two transitory PR. One patient received colchicine and vitamin C without response. It is concluded that 6-mercaptopurine and cyclophosphamide are useful drugs in refractory thrombocytopenic purpura.

  4. 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.

  5. [Splenectomy in chronic idiopathic thrombopenic purpura in adults. Apropos of 49 cases].

    Science.gov (United States)

    Melki, J; Dauce, J P; Kunlin, A; Tilly, H; Julien, J P; Monconduit, M; Piguet, H

    1989-01-01

    The authors reviewed the case files of 49 adult patients undergoing splenectomy for chronic idiopathic thrombocytopenic purpura at the Centre Henri Becquerel between 1970 and 1987. Although the postoperative course was straightforward in 83.7% of cases, one reoperation for subphrenic abscess was necessary and there was one postoperative death. Remission from thrombocytopenia was obtained in 87.5% of the patients, but only transiently in 8.5% of them. No preoperative predictive factors could be demonstrated. An early postoperative rise in the platelet count to more than 500 G/litre appears to ensure a good subsequent result. Secondary infectious complications are not exceptional and can be fatal (one death in our series); they require prophylaxis by anti-pneumococcal vaccination. The place of prophylactic antibiotic therapy has yet to be defined.

  6. Crystallographic structure of the human leukocyte antigen DRA, DRB3*0101: models of a directional alloimmune response and autoimmunity.

    Science.gov (United States)

    Parry, Christian S; Gorski, Jack; Stern, Lawrence J

    2007-08-10

    We describe structural studies of the human leukocyte antigen DR52a, HLA-DRA/DRB3*0101, in complex with an N-terminal human platelet integrin alphaII(B)betaIII 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 alphaII(B)betaIII 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 A. There are two alphabeta heterodimers to the asymmetric unit in space group P4(1)2(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 beta57 Asp-->Val substitution abrogates the salt-bridge to alpha76 Arg and along with a hydrophobic beta37 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.

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

    Energy Technology Data Exchange (ETDEWEB)

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

    2007-01-01

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

  8. Shiga toxin-associated hemolytic uremic syndrome and thrombotic thrombocytopenic purpura: distinct mechanisms of pathogenesis.

    Science.gov (United States)

    Tarr, Phillip I

    2009-02-01

    The hemolytic uremic syndrome (HUS) of childhood is, in its most common form, a non-immune microangiopathic hemolytic anemia. HUS typically follows an enteric infection with a Shiga toxin-producing Escherichia coli, usually belonging to serotype O157:H7. The antecedent infection is almost always manifested as non-bloody diarrhea. In about 80% of cases, the diarrhea becomes bloody between one and five days after the onset of diarrhea. The courses of acute gastrointestinal infections, and of HUS, in adults and children are similar. There are no convincing data that this easily recognizable form of microangiopathy is related to any inborn or acquired deficiency of ADAMTS13, and plasma therapies are not justified on either theoretical or empirical grounds. Similarly, there are no realistic animal data to support use of plasma exchange or infusion in children or adults with, or at risk for, HUS secondary to gastrointestinal infection with E. coli O157:H7. Best clinical practices involve rapid and accurate clinical and microbiological identification of infected patients, volume expansion, and support of the intestinal and extraintestinal complications that can ensue during acute enteric infection and associated HUS. Clinical clues include a sequence of events where the stool becomes bloody after a several-day interval of bloody diarrhea, considerable abdominal pain, five or more stools in the 24 h before presentation, pain on defection, and absence of fever at the time of presentation. Diagnosis should rely primarily on sorbitol MacConkey agar culture. Shiga toxin testing should not be used as the only screen to identify infected patients.

  9. 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

    with a dose regimen of 375 mg/m2 i.v. approximately once weekly for 4 consecutive weeks. Six patients received a fixed dose of 500 mg disregarding their weight supplemented by 100 mg of methylprednisone i.v. or 50-100 mg of Pred given as premedication together with an antihistamine just before infusion...

  10. 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

    Upshaw-Schulman syndrome (USS) is due to severe congenital deficiency of von Willebrand factor (VWF)-cleaving protease ADAMTS13 (a disintegrin and metalloprotease with thrombospondin type 1 domains, nr 13) activity resulting in the presence of unusually large forms of VWF in the circulation...... 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....

  11. 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

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

    Institute of Scientific and Technical Information of China (English)

    Saad Usmani; Joel Appel; Zainab Shahid; Husain Saleh

    2008-01-01

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

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

    Energy Technology Data Exchange (ETDEWEB)

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

    1999-07-01

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

  14. Capnocytophaga canimorsus infection presenting with complete splenic infarction and thrombotic thrombocytopenic purpura: a case report

    Directory of Open Access Journals (Sweden)

    Brichacek Michal

    2012-12-01

    Full Text Available Abstract Background Animal bites are typically harmless, but in rare cases infections introduced by such bites can be fatal. Capnocytophaga canimorsus, found in the normal oral flora of dogs, has the potential to cause conditions ranging from minor cellulitis to fatal sepsis. The tendency of C. canimorsus infections to present with varied symptoms, the organism’s fastidious nature, and difficulty of culturing make this a challenging diagnosis. Rarely, bacterial cytotoxins such as those produced by C. canimorsus may act as causative agents of TTP, further complicating the diagnosis. Early recognition is crucial for survival, and the variability of presentation must be appreciated. We present the first known case of C. canimorsus infection resulting in TTP that initially presented as splenic infarction. Case presentation 72-year-old Caucasian male presented with a four-day history of abdominal pain, nausea, vomiting, diarrhea, and intermittent confusion. On presentation, vital signs were stable and the patient was afebrile. Physical examination was unremarkable apart from petechiae on the inner left thigh, and extreme diffuse abdominal pain to palpation and percussion along with positive rebound tenderness. Initial investigations revealed leukocytosis with left shift and thrombocytopenia, but normal liver enzymes, cardiac enzymes, lipase, INR and PTT. Abdominal CT demonstrated a non-enhancing spleen and hemoperitoneum, suggesting complete splenic infarction. Although the patient remained afebrile, he continued deteriorating over the next two days with worsening thrombocytopenia. After becoming febrile, he developed microangiopathic hemolytic anemia and hemodynamic instability, and soon after was intubated due to hypoxic respiratory failure and decreased consciousness. Plasma exchange was initiated but subsequently stopped when positive blood cultures grew a gram-negative organism. The patient progressively improved following therapy with piperacillin-tazobactam, which was switched to imipenem, then meropenem when Capnocytophaga was identified. Conclusions There is a common misconception amongst practitioners that the presence of systemic infection excludes the possibility of TTP and vice versa. This case emphasizes that TTP may occur secondary to a systemic infection, thereby allowing the two processes to coexist. It is important to maintain a wide differential when considering the diagnosis of either TTP or C. canimorsus infection since delays in treatment may have fatal consequences.

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

    Energy Technology Data Exchange (ETDEWEB)

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

    1997-04-14

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

  16. Medical image of the week: purpura fulminans

    Directory of Open Access Journals (Sweden)

    Mosier J

    2013-06-01

    Full Text Available Figure 1: Purpura fulminans, a cutaneous manifestation of disseminated intravascular coagulation, due to Group A streptococcal septic shock (A, which improved with antibiotics, resuscitation, IVIG infusion, and topical nitroglycerin (B.

  17. 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.

  18. An improbable and unusual case of thrombotic thrombocytopenia purpura

    Directory of Open Access Journals (Sweden)

    Jaymon Patel

    2016-09-01

    Full Text Available Thrombotic thrombocytopenic purpura (TTP is a life-threatening medical emergency which may be difficult to recognize given the wide spectrum in which it presents. A delay in treatment may be catastrophic as untreated cases of TTP have a mortality rate exceeding 90%. Given the high fatality rate of untreated TTP and its range of presenting symptoms, we present our unusual case of TTP in a post-splenectomy patient with early treatment and positive outcome. This case describes a 54-year-old female who presented with hematuria and gingival bleeding, followed by the development of a bilateral lower extremity petechial rash. Her past medical history was significant for multiple episodes of TTP, the last of which resulted in a splenectomy and a 20-year history of remission thereafter. On exam, she was alert, well appearing, and neurologically intact. Her only significant finding was a bilateral lower extremity petechial rash. Laboratory studies revealed mild anemia and thrombocytopenia, an elevated lactate dehydrogenase, and a decreased haptoglobin. Peripheral smear showed poikilocytosis, helmet cells, and schistocytes. Corticosteroid therapy was promptly initiated, her platelets were monitored closely, and she underwent urgent therapeutic plasma exchange. Due to the risk of significant morbidity and mortality that may result from delayed treatment of TTP as well as the significant variations of presentation, TTP requires a consistently high index of suspicion. Our patient suffered multiple relapses of TTP within a 30-year span, underwent splenectomy in early adulthood, and presented with atypical symptoms during her most recent relapse illustrating how persistent TTP can be as well as how unusually it may present. Providers should be aware of the vast spectrum of presentation and remember that TTP may recur following splenectomy despite prolonged remission.

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

    Science.gov (United States)

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

    2016-05-01

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

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

    DEFF Research Database (Denmark)

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

    2007-01-01

    OBJECTIVES: To estimate the costs and health consequences of three different screening strategies for neonatal alloimmune thrombocytopenia (NAIT). DESIGN: Cost-utility analysis on the basis of a decision tree that incorporates the relevant strategies and outcomes. SETTING: Three health regions...... in Norway encompassing a 2.78 million population. POPULATION: Pregnant women (n = 100,448) screened for human platelet antigen (HPA) 1a and anti-HPA 1a antibodies, and their babies. METHOD: Decision tree analysis. In three branches of the decision tree, pregnant women entered a programme while in one...

  1. Red blood cell alloimmunization in patients with sickle cell disease in Turkey: a single center retrospective cohort study

    Directory of Open Access Journals (Sweden)

    Soner Solmaz

    2016-12-01

    Results: Of 216 SCD patients included in the study. Alloimmunization was detected in 67 (31.0% out of 216 patients who underwent transfusion, and in 17 (30.4% out of 56 patients in Group 1 and in 50 (31.3% out of 160 patients in Group 2. When the patients were analyzed according to alloimmunization development, our study revealed that neither SCD complications are a risk factor for alloimmunization nor alloimmunization increases mortality rates. Conclusion: High alloimmunization frequency found in our study suggests the insufficient adherence of alloimmunization-prevention policies in RBC transfusions performed except experienced institutions. Therefore alloimmunization may be reduced or prevented through performing extended red cell typing among SCD patients. [Cukurova Med J 2016; 41(4.000: 622-627

  2. The Association of CD81 Polymorphisms with Alloimmunization in Sickle Cell Disease

    Directory of Open Access Journals (Sweden)

    Zohreh Tatari-Calderone

    2013-01-01

    Full Text Available The goal of the present work was to identify the candidate genetic markers predictive of alloimmunization in sickle cell disease (SCD. Red blood cell (RBC transfusion is indicated for acute treatment, prevention, and abrogation of some complications of SCD. A well-known consequence of multiple RBC transfusions is alloimmunization. Given that a subset of SCD patients develop multiple RBC allo-/autoantibodies, while others do not in a similar multiple transfusional setting, we investigated a possible genetic basis for alloimmunization. Biomarker(s which predicts (predict susceptibility to alloimmunization could identify patients at risk before the onset of a transfusion program and thus may have important implications for clinical management. In addition, such markers could shed light on the mechanism(s underlying alloimmunization. We genotyped 27 single nucleotide polymorphisms (SNPs in the CD81, CHRNA10, and ARHG genes in two groups of SCD patients. One group (35 of patients developed alloantibodies, and another (40 had no alloantibodies despite having received multiple transfusions. Two SNPs in the CD81 gene, that encodes molecule involved in the signal modulation of B lymphocytes, show a strong association with alloimmunization. If confirmed in prospective studies with larger cohorts, the two SNPs identified in this retrospective study could serve as predictive biomarkers for alloimmunization.

  3. Henoch-Schönlein Purpura

    Science.gov (United States)

    ... Kidney Disease (CKD) What Is Chronic Kidney Disease? Causes of CKD Tests & Diagnosis Managing CKD Eating Right Preventing CKD ... adults, HSP can lead to chronic kidney disease (CKD) and kidney failure, described as ... are the causes of HSP? Henoch-Schönlein purpura is caused by ...

  4. Fatal alloimmune thrombocytopenia due to anti-HLA alloimmunization in a twin pregnancy: A very infrequent complication of assisted reproduction.

    Science.gov (United States)

    Meler, Eva; Porta, Roser; Canals, Carme; Serra, Bernat; Lozano, Miguel

    2016-11-02

    The most frequently involved antigen in severe fetal and neonatal alloimmune thrombocytopenia (FNAIT) is the human platelet antigen 1a. Platelets express the HLA-A and B antigens on their membrane and some studies report that maternal anti-HLA class I antibody can also cause FNAIT. We report here a very unusual case of a first twin pregnancy produced in vitro by oocyte and semen donation where the mother developed markedly elevated HLA antibodies, in the absence of anti-platelet or anti-neutrophil antibodies, that provoked in one of the twins a profound thrombocytopenia and intracranial hemorrhage and a mild thrombocytopenia and neutropenia in the second twin lasting until the fourth month of life. In addition, anti-D alloimmunization provoked hemolytic disease of the newborn with intrauterus anemia detected in the first twin and post-natal anemia in the second twin that required red blood cell transfusion and phototherapy. We hypothesize that the complete HLA-incompatible twin pregnancy due to the oocyte donation might have contributed to the severity of the clinical manifestations.

  5. Role of heat shock protein 70 in innate alloimmunity

    Directory of Open Access Journals (Sweden)

    Walter G. eLand

    2012-01-01

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

  6. Fetal/neonatal allo-immune thrombocytopenia (FNAIT): past, present, and future.

    NARCIS (Netherlands)

    Serrarens-Janssen, V.M.; Semmekrot, B.A.; Novotny, V.M.J.; Porcelijn, L.; Lotgering, F.K.; Delemarre, F.M.C.; Steegers, E.A.P.

    2008-01-01

    We reviewed the English, American, and German literature for articles describing the prevalence, clinical presentation, outcome, therapeutic options, and screening possibilities for fetal/neonatal allo-immune thrombocytopenia (FNAIT), published between January 1950 and March 2007. The reported preva

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

    DEFF Research Database (Denmark)

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

    2011-01-01

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

  8. 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...

  9. 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.

  10. Red blood cell alloimmunization is influenced by recipient inflammatory state at time of transfusion in patients with sickle cell disease.

    Science.gov (United States)

    Fasano, Ross M; Booth, Garrett S; Miles, Megan; Du, Liping; Koyama, Tatsuki; Meier, Emily Riehm; Luban, Naomi L C

    2015-01-01

    Sickle cell disease (SCD) patients are at increased risk of red blood cell (RBC) alloimmunization. Recipient inflammatory state at time of transfusion has been shown to regulate alloimmunization in murine models, but evidence is lacking in SCD patients. We retrospectively studied a cohort of alloimmunized SCD patients to determine the influence of pro-inflammatory SCD-related complications at time of transfusion on alloimmunization. For each transfusion, the presence of pro-inflammatory state, degree of RBC antigen matching, unit age, storage solution and alloantibody detection date were ascertained. Transfusion-associated pro-inflammatory events were compared between transfusions resulting and not resulting in new alloantibodies. Univariate analysis and multivariate logistic regression were performed. Fifty-two patients received 3166 pre-storage leuco-reduced transfusions of which 128 resulted in alloantibodies. Transfusions during inflammatory events were associated with increased alloantibody risk on univariate and multivariate analysis; acute chest syndrome and vaso-occlusive crisis showed strongest associations with alloimmunization. Increased antigen matching demonstrated a protective effect on alloimmunization (univariate and multivariate analysis). Although an association was seen between citrate-phosphate-dextrose (adenine) stored units and alloimmunization on univariate analysis, no effect was found on multivariate analysis. Identifying recipient pro-inflammatory states at time of transfusion that promote alloimmunization can impact RBC unit selection decisions for SCD patients at risk for alloimmunization.

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

    Science.gov (United States)

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

    2015-01-01

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

  12. Hyperglobulinemic purpura in the course of multiple myeloma.

    Science.gov (United States)

    Shalit, M; Bar-Sela, S; Leviatan, A; Naparstek, Y

    1980-01-01

    Secondary hyperglobulinemic purpura of Waldenström is characterized by polyclonal gammopathy associated mainly with autoimmune diseases. Its occurrence with multiple myeloma is very rare. We described a patient who developed characteristic lesions of hyperglobulinemic purpura in the course of IgA myeloma. Skin biopsy revealed deposition of IgA in the blood vessels.

  13. Detection of alloimmunization to ensure safer transfusion practice

    Directory of Open Access Journals (Sweden)

    Rashmi Sood

    2013-01-01

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

  14. Study of establishing disease-syndrome combined with animal model for immune thrombocytopenic purpura without additional conditions

    Directory of Open Access Journals (Sweden)

    Haiyan Lang

    2016-07-01

    Conclusion: According to the syndrome differentiation criteria for disease-syndrome combined model of ITP, the APS-injected animal model of ITP replicated through the passive immune modeling method without additional conditions possesses the characteristics of disease-syndrome combined model. It provides an ideal tool for the development of traditional Chinese medicine pharmacology experiment.

  15. Megakaryocytic dysfunction in myelodysplastic syndromes and idiopathic thrombocytopenic purpura is in part due to different forms of cell death

    NARCIS (Netherlands)

    Houwerzijl, E. J.; Blom, N. R.; van der Want, J. J. L.; Vellenga, E.; de Wolf, J. T. M.

    2006-01-01

    Platelet production requires compartmentalized caspase activation within megakaryocytes. This eventually results in platelet release in conjunction with apoptosis of the remaining megakaryocyte. Recent studies have indicated that in low-risk myelodysplastic syndromes (MDS) and idiopathic thrombocyto

  16. 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

  17. [Duodenal complications of rheumatoid purpura. Endoscopic aspects].

    Science.gov (United States)

    Chapoy, P; Guidon, M J; Louchet, E

    1984-01-01

    The aim of this work was to describe the endoscopic features and clinical outcome of the duodenal complications in anaphylactoid purpura. Over a 3-year period, 20 patients were hospitalized in our unit because of purpura rheumatica. Duodenal complications occurred in 5 cases warranting endoscopic assessment. All patients had bilious vomiting and epigastric pain, constantly associated with low-grade purpuric rash. Plasma factor XIII concentrations were always decreased. The duodenal complication was suspected radiologically in 2 cases when "thumbprint" impressions were seen. Petechiae, oedema and intramural hematoma with superficial erosions were present endoscopically in 3 cases. The lesions were severe and extensive, involving the entire duodenum in 3 cases and the jejunum in one case. In one patient, there was a stricture of the upper part of the second duodenum. Treatment consisted of parenteral nutrition (using a central catheter: 3 cases, or a peripheral vein: 2 cases) and cimetidine (30 mg/kg.bw). The clinical outcome was favorable in 4 patients; the symptoms vanished and the endoscopic lesions were reversible (including the stricture) with restitutio ad integrum after 10 days. The last patient died the 8th day of treatment, 3 days after digestive improvement; the cause of death was probably iatrogenic and related to accidental migration of the central catheter. These results suggest that endoscopic examination should be performed in all patients with anaphylactoid purpura presenting with bilious vomiting. Endoscopy seems to be of great value in deciding if parenteral nutrition is indicated--or not--and perhaps in order to contraindicate the use of steroid therapy in the case of ulcerated hematomas.

  18. 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.

  19. Henoch Schönlein purpura associated with pulmonary adenocarcinoma

    Directory of Open Access Journals (Sweden)

    Tetsuka Takafumi

    2011-06-01

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

  20. Medical image of the week: purpura fulminans

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    Power EP

    2016-12-01

    Full Text Available No abstract available. Article truncated at 150 words. A 54-year-old man with coronary artery disease, fibromyalgia and chronic sacral ulcers was brought to the emergency department due to acute changes in mentation and hypotension. He suffered a cardiac arrest shortly after arrival to the emergency department during emergent airway management. After successful resuscitation, he was admitted to the medical intensive care unit and treated for septic shock with fluid resuscitation, vasopressors and broad spectrum antibiotics. Laboratory results were significant for disseminated intravascular coagulopathy (DIC- thrombocytopenia, decreased fibrinogen and elevated PT, PTT and D-dimer levels. Profound metabolic acidosis and lactate elevation was also seen. Blood Cultures later revealed a multi-drug resistant E. coli bacteremia. Images of the lower extremities (Figure 1 were obtained at initial assessment and are consistent with purpura fulminans. He did not survive the stay. Purpura fulminans, also referred to as skin mottling, is an evolving skin condition which is characterized by an acutely worsening reticular …

  1. [Schoenlein-Henoch purpura with intestinal involvement].

    Science.gov (United States)

    Blöchinger, M; Schmitt, W; Beer, M; Seib, H J

    1997-11-01

    A 51-year-old male patient admitted to the hospital because of colic-like abdominal pain, paralytic ileus, anal bleeding and microhaematuria with proteinuria, developed an intestinal ischemia with a serum lactate level of 6.3 mmol/l. An occlusion of the large vessels was excluded angiographically. Perfusion disorders were detected both endoscopically and histologically in the upper gastrointestinal tract and in the terminal ileum. When after two days a palpable purpura appeared on the anterior of both feet, a vasculitis type Schoenlein-Henoch was suspected and treated with high doses of steroids, resulting in decreasing symptoms. From the point of admittance, a nephritic urinary sediment had been apparent, and the renal affliction developed into a nephrotic syndrome without notable reduction in the glomerular filtration rate. On the 13th day of treatment the patient-being on a reduced dose of steroids-suffered from a severe relapse; however, this responded favorably to an increase of the dosage. The kidneys required approximately one year for complete recovery. Based on this case, the Schoenlein-Henoch purpura syndrome and its differential diagnosis are presented, particularly with respect to gastrointestinal symptoms and in view of the pertinent literature.

  2. Henoch-Schonlein purpura on the legs (image)

    Science.gov (United States)

    ... children than adults and often occurs after an upper respiratory infection. It causes skin rashes that bleed into the skin (petechiae and purpura). Bleeding may also occur from the gastrointestinal tract and kidneys.

  3. Prenatal testing for hemolytic disease of the newborn and fetal neonatal alloimmune thrombocytopenia - current status.

    Science.gov (United States)

    Avent, Neil D

    2014-12-01

    Incompatibility of red cell and platelet antigens can lead to maternal alloimmunization causing hemolytic disease of the fetus & newborn and fetal neonatal alloimmune thrombocytopenia respectively. As the molecular background of these polymorphisms emerged, prenatal testing using initially fetal DNA obtained from invasively obtained amniotic fluid or chorionic villus was implemented. This evolved into testing using maternal plasma as source of fetal DNA, and this is in routine use as a safe non-invasive diagnostic that has no risk to the fetus of alloimmunization or spontaneous miscarriage. These tests were initially applied to high risk pregnancies, but has been applied on a mass scale, to screen fetuses in D-negative pregnant populations as national screening programs. Fetal neonatal alloimmune thrombocytopenia management has had comparatively small take up in non-invasive testing for causative fetal platelet alleles (e.g., HPA-1A), but mass scale genotyping of mothers to identify at risk HPA-1b1b pregnancies and their treatment with prophylactic anti-HPA-1A is being considered in at least one country (Norway).

  4. Purpura Fulminans Secondary to Streptococcus pneumoniae Meningitis

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    Erick F. Alvarez

    2012-01-01

    Full Text Available Purpura fulminans (PF is a rare skin disorder with extensive areas of blueblack hemorrhagic necrosis. Patients manifest typical laboratory signs of disseminated intravascular coagulation (DIC. Our case describes a 37-year-old previously healthy man who presented with 3 days of generalized malaise, headache, vomiting, photophobia, and an ecchymotic skin rash. Initial laboratory workup revealed DIC without obvious infectious trigger including unremarkable cerebrospinal fluid (CSF biochemical analysis. There was further progression of the skin ecchymosis and multiorgan damage consistent with PF. Final CSF cultures revealed Streptococcus pneumoniae. Despite normal initial CSF biochemical analysis, bacterial meningitis should always be considered in patients with otherwise unexplained DIC as this may be an early manifestation of infection. PF is a clinical diagnosis that requires early recognition and prompt empirical treatment, especially, in patients with progressive altered mental status, ecchymotic skin rash, and DIC.

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

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    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

  6. Alloimmunization in Patients with Sickle Cell Disease and Thalassemia: Experience of a Single Centre in Oman

    Science.gov (United States)

    Alkindi, Salam; AlMahrooqi, Saba; AlHinai, Sumaiya; AlMarhoobi, Ali; Al-Hosni, Saif; Daar, Shahina; Fawaz, Naglaa; Pathare, Anil

    2017-01-01

    Background Blood transfusion is an integral part of the supportive care for patients with sickle cell disease (SCD) and thalassaemia. The hazard of red cell alloimmunization, however, is one of the main complications of this therapy. Objectives The aim of this study was to evaluate the prevalence of red cell alloimmunization in Omani patients with sickle cell anaemia and thalassemia. Methods This study included 262 patients whose historical transfusion records were available. One hundred and twenty-nine patients with thalassaemia who were attending the day care unit for regular transfusions, and 133 SCD patients admitted at our hospital were included in this study. The Diamed® gel system was used for the screening and identification of atypical antibodies. Results The rate of alloimmunization in SCD patients was 31.6% (n=42, 95%CI, 24.87–40.66), whereas in patients with thalassaemia it was 20% (n=26; 95%CI, 13.9–27.6). Antibodies to E, e, C, c, D, K, S, Fyª, Kpª, Jkª and Cw were observed; 85% of the patients were also immunised with Rh and Kell antigens. Considering the two groups together, 8 developed nonspecific antibodies and 12 developed more than one antibody. Conclusions Red cell transfusions were associated with a significant risk of alloimmunization. It is, therefore, imperative to perform an initial extended red cell phenotyping for both donors and recipients, and carefully select ABO, Rh and Kell matched donors. The higher incidence of alloimmunization in SCD patients is related to the inherent SCD-specific inflammatory state. PMID:28293401

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

    Science.gov (United States)

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

    2008-01-01

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

  8. Treatments for hematologic malignancies in contrast to those for solid cancers are associated with reduced red cell alloimmunization

    Science.gov (United States)

    Evers, Dorothea; Zwaginga, Jaap Jan; Tijmensen, Janneke; Middelburg, Rutger A.; de Haas, Masja; de Vooght, Karen M.K.; van de Kerkhof, Daan; Visser, Otto; Péquériaux, Nathalie C.V.; Hudig, Francisca; van der Bom, Johanna G.

    2017-01-01

    Red cell alloimmunization may induce severe hemolytic side effects. Identification of risk-modifying conditions will help tailor preventative strategies. This study aims to quantify the associations of hematologic malignancies and solid cancers with red cell alloimmunization in patients receiving red cell transfusions. We performed a nested multicenter case-control study in a source population of 24,063 patients receiving their first and subsequent red cell transfusions during an 8-year follow-up period. Cases (n=505), defined as patients developing a first transfusion-induced red cell alloantibody, were each compared with 2 non-alloimmunized controls (n=1010) who received a similar number of red cell units. Using multivariate logistic regression analyses, we evaluated the association of various malignancies and treatment regimens with alloimmunization during a delineated 5-week risk period. The incidence of alloimmunization among patients with acute (myeloid or lymphoid) leukemia and mature (B- or T-cell) lymphoma was significantly reduced compared to patients without these malignancies: adjusted relative risks (RR) with 95% confidence interval (CI) 0.36 (range 0.19–0.68) and 0.30 (range 0.12–0.81). Associations were primarily explained by immunosuppressive treatments [RR for (any type of) chemotherapy combined with immunotherapy 0.27 (95%CI: 0.09–0.83)]. Alloimmunization risks were similarly diminished in allogeneic or autologous stem cell transplanted patients (RR 0.34, 95%CI: 0.16–0.74), at least during the six months post transplant. Alloimmunization risks of patients with other hematologic diseases or solid cancers, and their associated treatment regimens were similar to risks in the general transfused population. Our findings suggest that, in contrast to malignancies in general, hemato-oncological patients treated with dose-intensive regimens have strongly diminished risk of red cell alloimmunization. PMID:27634204

  9. Pregnancy outcome in women with antiphospholipid syndrome and alloimmunity: a case report

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    Serguei Abel Castañeda Ospina

    Full Text Available CONTEXT: Patients with antiphospholipid syndrome and alloimmunity have poor pregnancy outcomes. Several diagnostic and therapeutic options exist for these disorders, although there is no consensus as to the best treatment. CASE REPORT: We present here the clinical course and treatment of a woman with a history of two miscarriages who joined our program 10 years ago and has been followed up ever since. After antiphospholipid syndrome and alloimmune failure were diagnosed, she was given preconceptional treatment using unfractionated heparin, aspirin, prednisone and lymphocyte immunizations. She delivered two premature babies in the following two pregnancies. At present both children are healthy and are attending school. The fifth pregnancy was unsuccessful, in spite of having undergone a similar but postconceptional therapeutic scheme. We discuss this case focusing on the pathogenic mechanisms and the therapeutic aspects of these disorders.

  10. 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

    2015-08-01

    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.

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

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    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.

  12. Brucellosis mimicking Henoch-Schönlein purpura.

    Science.gov (United States)

    Massasso, David; Gibson, Kathryn

    2007-06-04

    A young male immigrant from Syria with a vasculitic-appearing leg rash, asymmetrical polyarthritis, microscopic haematuria, and raised inflammatory markers was provisionally diagnosed with Henoch-Schönlein purpura. Skin biopsy showed leukocytoclastic vasculitis. Low-grade fevers persisted despite non-steroidal anti-inflammatory therapy, and Brucella sp. was subsequently grown from both blood and synovial fluid aspirates. Further tests gave positive results for B. abortus, and triple antibiotic therapy produced a rapid clinical response. Cutaneous vasculitis has rarely been described in brucellosis, and this is the first report in the English medical literature of brucellosis mimicking Henoch-Schönlein purpura.

  13. 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

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

    Science.gov (United States)

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

    2016-02-01

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

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

    NARCIS (Netherlands)

    J.C. Davin

    2011-01-01

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

  16. 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.

  17. Septic Shock with Purpura in Children: an experimental and clinical approach

    NARCIS (Netherlands)

    J.A. Hazelzet (Jan)

    1998-01-01

    textabstractSeptic shock with purpura is a rapidly evolving clinical picture characterized by sepsis (tachycardia, tachypnea, fever), shock (hypotension or signs of end-organ failure) and a spectrum of coagulation disorders (ranging from petechiae, purpura to ecchymoses). It is mainly (80 %) caused

  18. Strong association between respiratory viral infection early after hematopoietic stem cell transplantation and the development of life-threatening acute and chronic alloimmune lung syndromes

    NARCIS (Netherlands)

    Versluys, A. Birgitta; Rossen, John W. A.; van Ewijk, Bart; Schuurman, Rob; Bierings, Marc B.; Boelens, Jaap J.

    2010-01-01

    Alloimmune lung syndromes (allo-LS), including idiopathic pneumonia syndrome, bronchiolitis obliterans syndrome, and bronchiolitis obliterans organizing pneumonia, are severe complications after hematopoietic stem cell transplantation (HSCT). In our cohort of 110 pediatric patients, 30 had allo-LS (

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

    Science.gov (United States)

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

    2015-05-17

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

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

    Science.gov (United States)

    Curtis, Brian R

    2015-12-01

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

  1. 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).

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

    Energy Technology Data Exchange (ETDEWEB)

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

    1987-09-01

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

  3. Synchronous Occurrence of Diffuse Large B-cell Lymphoma of the Duodenum and Gastrointestinal Stromal Tumor of the Ileum in a Patient with Immune Thrombocytopenic Purpura

    Science.gov (United States)

    Takahashi, Tohru; Maruyama, Yumiko; Saitoh, Mayuko; Itoh, Hideto; Yoshimoto, Mitsuru; Tsujisaki, Masayuki; Nakayama, Masato

    2016-01-01

    A 64 year-old woman with steroid-dependent immune thrombocytopenia developed anemia. Esophagogastroduodenoscopy revealed the presence of a tumor, which was diagnosed to be diffuse large B-cell lymphoma, in the second portion of the duodenum. 18F-fluorodeoxy glucose positron emission tomography showed an increased uptake mass in the pelvic cavity as well as in the duodenum. Though the duodenal tumor disappeared after 4 cycles of chemotherapy, the pelvic mass did not shrink in size. As a result, laparoscopic resection of the pelvic tumor was performed and the tumor was histologically diagnosed to be a gastrointestinal stromal tumor. Subsequently, the patient was treated with 2 more cycles of the chemotherapy. Eventually, thrombocytopenia completely resolved. PMID:27746431

  4. 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

  5. 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.

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

    DEFF Research Database (Denmark)

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

    2014-01-01

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

  7. 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......We report 2 cases of Capnocytophaga canimorsus-induced septicaemia complicated by purpura fulminans in previously healthy individuals, both of whom had been exposed to dog saliva prior to disease. They both presented with purpuric skin lesions, as well as the tetrad of abdominal symptoms...... fulminans in the unresolved critically ill patient with a history of dog exposure....

  8. 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.

  9. Resolution of alloimmunization and refractory autoimmune hemolytic anemia in a multi-transfused beta-thalassemia major patient

    Directory of Open Access Journals (Sweden)

    Joseph Philip

    2014-01-01

    Full Text Available Beta-thalassemia is one of the most prevalent autosomal disorders, which affect more than 400,000 newborn per year worldwide. In India, the carrier rate of beta-thalassemia varies from 3-17%. The overall rate of alloimmunization in thalassemia patients has been reported to be 5-30% in the world, which is mostly contributed by the alloimmunization to minor blood group antigen. Among Asians, the incidence of red cell alloimmunization is 22%. The recommended treatment for beta-thalassemia major is regular blood transfusion every 3 to 4 weeks. The development of anti-red cell antibodies (alloantibodies and/or autoantibodies can significantly complicate transfusion therapy. Alloantibodies are commonly associated with red cell hemolysis. Red cell autoantibodies appear less frequently, but they can result in clinical hemolysis called autoimmune hemolytic anemia (AIHA, and in difficulty in cross-matching blood. Patients with autoantibodies may have a higher transfusion rate and often require immunosuppressive drugs or alternative treatments including intravenous immunoglobulin (IVIg and rituximab (anti-CD20 monoclonal antibody.

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

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    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.

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

    Institute of Scientific and Technical Information of China (English)

    ZHENG; Tian-wen

    2001-01-01

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

  12. Pregabalin- and azithromycin-induced rhabdomyolysis with purpura: An unrecognized interaction: A case report

    Directory of Open Access Journals (Sweden)

    Kazuya Kato, MD

    2016-01-01

    Conclusion: We report an extremely rare case of rhabdomyolysis with purpura caused by a drug interaction between pregabalin and azithromycin. However, the mechanisms of the interactions between azithromycin on the pregabalin are still unknown.

  13. Henoch-Schönlein purpura with c-ANCA antibody in an adult*

    Science.gov (United States)

    Torraca, Pedro de Freitas Silva; de Castro, Bruna Corrêa; Hans Filho, Günter

    2016-01-01

    The Henoch-Schönlein purpura is the vasculitis associated with deposits of immunoglobulin A in small vessels. Its association with cytoplasmic antineutrophil cytoplasmic antibodies is possible, but rare. This vasculitis is uncommon in adults and the main clinic manifestations are purpuric lesions in lower limbs with gastrointestinal symptoms and renal involvement. The present work describes a rare case of Henoch-Schönlein purpura in an adult with cytoplasmic antineutrophil cytoplasmic antibodies. PMID:27828648

  14. Prognostic factors of severe infectious purpura in children.

    Science.gov (United States)

    Leclerc, F; Beuscart, R; Guillois, B; Diependaele, J F; Krim, G; Devictor, D; Bompard, Y; van Albada, T

    1985-01-01

    The French Club of Pediatric Intensive Care has prospectively studied 90 cases of infectious purpura which were hospitalized in 1981; the purpose of this study was to determine prognostic factors. The statistical study (X2 test) of all these cases is in agreement with data in the literature and shows that the mortality is significantly higher when there is: shock (p less than 0.001), coma (p less than 0.05), ecchymotic or necrotic purpura (p less than 0.01), temperature less than 36 degrees C (p less than 0.05), no clinical meningism (p less than 0.001), white cell count less than 10,000/mm3 (p less than 0.05), thrombocytopenia less than 100,000 (p less than 0.01), fibrinogen less than 1.5 g/l (p less than 0.001), kalemia greater than 5 mEq/l (p less than 0.01), spinal fluid cell count less than 20/mm3 (p less than 0.01). Because shock is one of the main prognostic factors (23 deaths in 55 shocked patients, versus 2 in 35 non-shocked) we have performed another statistical study (with the Benzecri method) to determine a prognostic index for patients in shock. For its determination, five initial parameters are used: age, kalemia, white cell count, clinical meningism, platelet count. The predictive value for survival is 91%. The predictive value for death is 87%. The score was applied on the patients hospitalized in shock in 1982: the predictive value for survival is 75%, the predictive value for death is 61%.

  15. Red blood cell alloimmunization is influenced by the delay between Toll-like receptor agonist injection and transfusion.

    Science.gov (United States)

    Elayeb, Rahma; Tamagne, Marie; Bierling, Philippe; Noizat-Pirenne, France; Vingert, Benoît

    2016-02-01

    Murine models of red blood cell transfusion show that inflammation associated with viruses or methylated DNA promotes red blood cell alloimmunization. In vaccination studies, the intensity of antigen-specific responses depends on the delay between antigen and adjuvant administration, with a short delay limiting immune responses. In mouse models of alloimmunization, the delay between the injection of Toll-like receptor agonists and transfusion is usually short. In this study, we hypothesized that the timing of Toll-like receptor 3 agonist administration affects red blood cell alloimmunization. Poly(I:C), a Toll-like receptor 3 agonist, was administered to B10BR mice at various time points before the transfusion of HEL-expressing red blood cells. For each time point, we measured the activation of splenic HEL-presenting dendritic cells, HEL-specific CD4(+) T cells and anti-HEL antibodies in serum. The phenotype of activated immune cells depended on the delay between transfusion and Toll-like receptor-dependent inflammation. The production of anti-HEL antibodies was highest when transfusion occurred 7 days after agonist injection. The proportion of HEL-presenting CD8α(+) dendritic cells producing interleukin-12 was highest in mice injected with poly(I:C) 3 days before transfusion. Although the number of early-induced HEL-specific CD4(+) T cells was similar between groups, a high proportion of these cells expressed CD134, CD40 and CD44 in mice injected with poly(I:C) 7 days before transfusion. This study clearly shows that the delay between transfusion and Toll-like receptor-induced inflammation influences the immune response to transfused red blood cells.

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

    Science.gov (United States)

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

    1993-06-15

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

  17. Fas ligand enhances hematopoietic cell engraftment through abrogation of alloimmune responses and nonimmunogenic interactions.

    Science.gov (United States)

    Pearl-Yafe, Michal; Yolcu, Esma S; Stein, Jerry; Kaplan, Ofer; Yaniv, Isaac; Shirwan, Haval; Askenasy, Nadir

    2007-06-01

    Early after transplantation, donor lineage-negative bone marrow cells (lin(-) BMC) constitutively upregulated their expression of Fas ligand (FasL), suggesting an involvement of the Fas/FasL axis in engraftment. Following the observation of impaired engraftment in the presence of a dysfunctional Fas/FasL axis in FasL-defective (gld) donors or Fas-defective (lpr) recipients, we expressed a noncleavable FasL chimeric protein on the surface of donor lin(-) BMC. Despite a short life span of the protein in vivo, expression of FasL on the surface of all the donor lin(-) BMC improved the efficiency of engraftment twofold. The FasL-coated donor cells efficiently blunted the host alloimmune responses in primary recipients and retained their hematopoietic reconstituting potential in secondary transplants. Surprisingly, FasL protein improved the efficiency of engraftment in syngeneic transplants. The deficient engraftment in lpr recipients was not reversed in chimeric mice with Fas(-) stroma and Fas(+) BMC, demonstrating that the host marrow stroma was also a target of donor cell FasL. Hematopoietic stem and progenitor cells are insensitive to Fas-mediated apoptosis and thus can exploit the constitutive expression of FasL to exert potent veto activities in the early stages of engraftment. Manipulation of the donor cells using ectopic FasL protein accentuated the immunogenic and nonimmunogenic interactions between the donor cells and the host, alleviating the requirement for a megadose of transplanted cells to achieve a potent veto effect. Disclosure of potential conflicts of interest is found at the end of this article.

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

    Science.gov (United States)

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

    1999-01-01

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

  19. Platelets and platelet alloantigens: Lessons from human patients and animal models of fetal and neonatal alloimmune thrombocytopenia

    Science.gov (United States)

    Vadasz, Brian; Chen, Pingguo; Yougbaré, Issaka; Zdravic, Darko; Li, June; Li, Conglei; Carrim, Naadiya; Ni, Heyu

    2017-01-01

    Platelets play critical roles in hemostasis and thrombosis. Emerging evidence indicates that they are versatile cells and also involved in many other physiological processes and disease states. Fetal and neonatal alloimmune thrombocytopenia (FNAIT) is a life threatening bleeding disorder caused by fetal platelet destruction by maternal alloantibodies developed during pregnancy. Gene polymorphisms cause platelet surface protein incompatibilities between mother and fetus, and ultimately lead to maternal alloimmunization. FNAIT is the most common cause of intracranial hemorrhage in full-term infants and can also lead to intrauterine growth retardation and miscarriage. Proper diagnosis, prevention and treatment of FNAIT is challenging due to insufficient knowledge of the disease and a lack of routine screening as well as its frequent occurrence in first pregnancies. Given the ethical difficulties in performing basic research on human fetuses and neonates, animal models are essential to improve our understanding of the pathogenesis and treatment of FNAIT. The aim of this review is to provide an overview on platelets, hemostasis and thrombocytopenia with a focus on the advancements made in FNAIT by utilizing animal models.

  20. RECURRENT HENOCH-SCHÖNLEIN PURPURA IN FAMILIAL MEDITERRANEAN FEVER

    Directory of Open Access Journals (Sweden)

    A Nickavar

    2008-08-01

    Full Text Available "nFamilial Mediterranean fever (FMF is a relatively rare disorder, characterized by recurrent self-limited attacks of fever and polyserositis. Diagnosis is made by clinical features, gene identification on chromosome 16 and clinical response to specific treatment. Different types of vasculitis have been reported in FMF. Henoch-Schönlein purpura (HSP is one of them, usually with a benign clinical course. Repeated attacks of HSP have been rarely reported in FMF. This is the report of a 7-year-old girl who presented initially with recurrent fever and abdominal pain. After the primary diagnosis of FMF and appropriate treatment, she experienced two documented repeated attacks of HSP with severe renal involvement (crescentric glomerulonephritis and protracted abdominal pain in the second one. Glomerulonephritis was controlled by methyl-prednisolone pulse therapy plus oral corticosteroid and azathioprine, but abdominal pain was resistant to steroids and revealed completely by intravenous immunoglobuline (IVIg administration. In conclusion, it is suggested to consider the recurrence of HSP in cases with FMF to prevent irreversible renal complications. IVIg seems to be a good choice for the management of intractable abdominal pain of HSP.

  1. An unusual case of cryoglobulinemic purpura in elderly patient

    Directory of Open Access Journals (Sweden)

    Mauro Turrin

    2015-03-01

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

  2. Bartonella seropositivity in children with Henoch-Schonlein purpura

    Directory of Open Access Journals (Sweden)

    McColl Dorothy

    2005-04-01

    Full Text Available Abstract Background An association between Henoch-Schonlein purpura (HSP and seropositivity for Bartonella henselae (BH has been described. The objective of this study was to see if such an association exists in northern Alberta. Methods Immunofluorescent antibody testing utilizing an antigen prepared from B. henselae was undertaken on sera from six children with current HSP, 22 children with remote HSP, and 28 controls that were matched for age. Blood from the six children with current HSP was analysed by polymerase chain reaction (PCR assay with primers derived from the citrate synthase (gltA gene for the detection of Bartonella DNA. Results The seropositivity rate for BH was 61% in cases versus 21% in controls (p Conclusion There is an increased seropositivity rate for BH in children with HSP. However, it is not clear if infection with B. henselae or a related Bartonella species can result in HSP, or if the increased seropositivity is from non-specific or cross-reacting antibodies.

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

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    Ting He

    2014-01-01

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

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

    Directory of Open Access Journals (Sweden)

    Oliveira Raimundo Antônio Gomes

    2003-01-01

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

  5. Severe myalgia of the lower extremities as the first clinical feature of meningococcal purpura fulminans.

    Science.gov (United States)

    de Souza, Alexandre Leite; Sztajnbok, Jaques; Salgado, Maristela Marques; Romano, Carla C; Alkmin, Maria das Graças Adelino; Duarte, Alberto J S; Seguro, Antonio Carlos

    2007-10-01

    In patients with meningococcal infection, devastating presentations, such as purpura fulminans, which can progress to extensive tissue necrosis of the limbs and digits, have a significant social impact. The case presented herein illustrates such a phenomenon in a patient who developed bilateral necrosis of the lower extremities as a result of infection with Neisseria meningitis. We emphasize that severe myalgia was the first clinical manifestation of meningococcal purpura fulminans in our case. However, myalgia has typically been overlooked and undervalued as an early clinical feature of meningococcal sepsis. Early recognition and prompt initial antibiotic therapy continue to be the cornerstones of the successful management of this dramatic disease, reducing morbidity and mortality.

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

    Institute of Scientific and Technical Information of China (English)

    Parameswaran; Prathiba; Rajalakshmi; Kalyanasundaram; Srinivasan

    2015-01-01

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

  7. 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.

  8. 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.

  9. Ibuprofen-induced Henoch–Schönlein purpura nephritis: First reported case

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    Christopher Lim Thiam Seong

    2016-01-01

    Full Text Available Ibuprofen is a nonsteroidal anti-inflammatory drug that is used widely in treating pain, fever, and inflammation. Its side effects are mainly due to acute renal impairment and gastric discomfort. We hereby report a rare case of Henoch–Schönlein purpura nephritis secondary to ibuprofen consumption which has not been reported in literature before.

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

    DEFF Research Database (Denmark)

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

    1978-01-01

    A case of post-transfusion purpura in a 61-year-old, multiparous female with a platelet alloantibody (anti-Zwa) in her serum is reported. The patient was successfully treated with plasma exchange by means of a Haemonetics 30 cell separator and corticosteroids. Compared with other therapeutic meas...

  11. [Peripheral microthrombotic purpura associated with acute promyelocytic leukemia in pregnancy. A light and electron microscopic study].

    Science.gov (United States)

    Beller, F K; Wagner, H; Büchner, T

    1978-06-15

    A case is presented of a pregnant patient in the 28th week of gestation with promyelocytic leukemia and an unusual thrombohemorrhagic skin lesion. Ultrastructural examination revealed a microthrombotic purpura. Reduced coagulation factors increased during heparin treatment. The exacerbation of disseminated intravascular coagulation is explained by a hypercoagulable state in pregnancy in association with the as yet unknown etiology of "promyelocytic fibrinopathic leukemia".

  12. Aquaporin-4 positive neuromyelitis optica spectrum disorders secondary to thrombopenic purpura

    Science.gov (United States)

    Wang, Ying; Gong, Qiaoyun; Zhu, Mingqin; Lu, Chao; Sun, Li; Feng, Jiachun; Zhang, Hongliang

    2017-01-01

    Abstract Rationale: Neuromyelitis optica spectrum disorders (NMOSD) is considered as an immune-mediated disorder in the central nervous system (CNS). Numerous autoimmune diseases are frequently complicated with NMOSD and distinct clinical characteristics are noted in NMOSD patients with other autoimmune diseases. However, to our best knowledge, co-occurrence of NMOSD and thrombopenic purpura is rarely identified. Patient concerns: We presented a rare case of a 72-year-old female with 6-year history of thrombopenic purpura, and 1-month history of blurred vision as well as chest zonethesia. Anti-aquaporin-4 (AQP4) antibodies was positive in the serum of the patient. Diagnoses: With the addition of laboratory findings, iconography findings and physical examination results, the diagnosis of NMOSD was established according to the most recent diagnostic criteria. Interventions and outcomes: With the treatment of intravenous immunoglobulin (IVIg), the patient felt better at discharge without changing of expanded disability status scale (EDSS) score. Lessons: The case indicates that NMOSD could co-occur with thrombopenic purpura. The disturbance of immune system balance may explain this overlap. Further studies are warranted to reveal the mechanism and to explore whether patients with NMOSD with and without thrombopenic purpura have distinct clinical feature, drug responsiveness or prognosis. PMID:28079804

  13. A case of fulminant varicella infection with purpura fulminans, hepatitis, and rhabdomyolysis

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    A S Karadag

    2012-01-01

    Full Text Available Varicella zoster virus causes varicella which is a common disease. Generally it is self-limiting, and treatment is often unnecessary, but severe or life-threatening complications are rarely seen. We report a case of fulminant varicella complicating with purpura fulminans, hepatitis, and probable rhabdomyolysis in a previously healthy child.

  14. A Case of Fulminant Varicella Infection with Purpura Fulminans, Hepatitis, and Rhabdomyolysis

    Science.gov (United States)

    Karadag, A S; Bilgili, S G; Calka, O; Çeçen, İ; Akbayram, S

    2012-01-01

    Varicella zoster virus causes varicella which is a common disease. Generally it is self-limiting, and treatment is often unnecessary, but severe or life-threatening complications are rarely seen. We report a case of fulminant varicella complicating with purpura fulminans, hepatitis, and probable rhabdomyolysis in a previously healthy child. PMID:23248376

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

    Institute of Scientific and Technical Information of China (English)

    宣萍

    2014-01-01

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

  16. Henoch-Schönlein purpura with intracerebral haemorrhage in an adult patient: a case report

    Directory of Open Access Journals (Sweden)

    Karamadoukis Lazarus

    2008-06-01

    Full Text Available Abstract Introduction Henoch-Schönlein purpura is a small vessel vasculitis that affects mainly the skin, joints, gastrointestinal tract and kidneys. The central nervous system is also occasionally affected, although the majority of patients experience only mild symptoms such as headaches and behavioural changes. Intracerebral haemorrhage is a rare complication of Henoch-Schönlein purpura that so far has mainly been described in children and young adolescence. Case presentation We describe a 42-year-old man with Henoch-Schönlein purpura who developed an acute intracerebral haemorrhage that coincided with a reactivation of his vasculitis and the development of renal failure following discontinuation of steroids. In this patient, both the Henoch-Schönlein purpura and his neurological symptoms were successfully treated with intravenous cyclophosphamide and methylprednisolone, followed by a short course of oral cyclophosphamide and long-term oral prednisolone. His renal function also recovered sufficiently not to require renal replacement therapy. Conclusion The management of Henoch-Schönlein nephritis remains unclear, especially in the presence of severe complications such as intracerebral haemorrhage. We describe a successful outcome in such a patient.

  17. Recombinant HPA-1a antibody therapy for treatment of fetomaternal alloimmune thrombocytopenia: proof of principle in human volunteers.

    Science.gov (United States)

    Ghevaert, Cedric; Herbert, Nina; Hawkins, Louise; Grehan, Nicola; Cookson, Philip; Garner, Steve F; Crisp-Hihn, Abigail; Lloyd-Evans, Paul; Evans, Amanda; Balan, Kottekkattu; Ouwehand, Willem H; Armour, Kathryn L; Clark, Mike R; Williamson, Lorna M

    2013-07-18

    Fetomaternal alloimmune thrombocytopenia, caused by the maternal generation of antibodies against fetal human platelet antigen-1a (HPA-1a), can result in intracranial hemorrhage and intrauterine death. We have developed a therapeutic human recombinant high-affinity HPA-1a antibody (B2G1Δnab) that competes for binding to the HPA-1a epitope but carries a modified constant region that does not bind to Fcγ receptors. In vitro studies with a range of clinical anti-HPA-1a sera have shown that B2G1Δnab blocks monocyte chemiluminescence by >75%. In this first-in-man study, we demonstrate that HPA-1a1b autologous platelets (matching fetal phenotype) sensitized with B2G1Δnab have the same intravascular survival as unsensitized platelets (190 hours), while platelets sensitized with a destructive immunoglobulin G1 version of the antibody (B2G1) are cleared from the circulation in 2 hours. Mimicking the situation in fetuses receiving B2G1Δnab as therapy, we show that platelets sensitized with a combination of B2G1 (representing destructive HPA-1a antibody) and B2G1Δnab survive 3 times as long in circulation compared with platelets sensitized with B2G1 alone. This confirms the therapeutic potential of B2G1Δnab. The efficient clearance of platelets sensitized with B2G1 also opens up the opportunity to carry out studies of prophylaxis to prevent alloimmunization in HPA-1a-negative mothers.

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

    Science.gov (United States)

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

    2016-01-01

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

  19. [Purpura pigmentosa progressiva in type III cryoglobulinemia and tartrazine intolerance. A follow-up over 20 years].

    Science.gov (United States)

    Kalinke, D U; Wüthrich, B

    1999-01-01

    A 58 year old patient with hepatitis virus C (HCV) infection had a secondary polyclonal IgG-IgM cryoglobulinemia with a benign 20 year course. Clinically the patient suffered from progressive pigmented purpura (PPP). Histologic evaluation revealed a lymphocytic vasculitis. Food containing tartrazine triggered flares of the PPP, as demonstrated with controlled oral provocation testing. In most of the previously described cases of HCV and type III cryoglobulinemia, the typical cutaneous finding was palpable purpura with leukocytoclastic vasculitis.

  20. 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.

  1. 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 (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.

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

    Science.gov (United States)

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

    2016-01-01

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

  3. Extensive Necrotic Purpura in Levamisole-Adulterated Cocaine Abuse - A Case Report.

    Science.gov (United States)

    Le Garff, Erwan; Tournel, Gilles; Becquart, Coralie; Cottencin, Olivier; Dupin, Nicolas; Delaporte, Emmanuel; Hedouin, Valéry

    2016-11-01

    Levamisole, which is used as an adulterated compound of cocaine, is currently being seen year after year in cocaine intoxication. For a few cases in the last decade, necrotic purpura and neutropenia after levamisole/cocaine intoxication have been described in the medical community. Herein, we present an original case of levamisole intoxication of a 40-year-old woman who smoked heroin and cocaine few during a month. She rapidly presented an extensive necrotic purpura of the nose, cheeks and extremities (lower and upper), and immunologic reactions (positive anti-MPO and anti-HNE). Levamisole was detected on hairs with ultra-high performance liquid chromatography and tandem mass spectrometry. The case reports also a probable cocaine supplier deceit, which bring pure drug for hospital investigation after the intoxication of his client. The intoxicated woman had survived with several skin and chronic pain complications. That case recalls the knowledge about levamisole with a short review of the forensic literature.

  4. Korean Herbal Medicine for Treating Henoch-Schonlein Purpura with Yin Deficiency: Five Case Reports

    Directory of Open Access Journals (Sweden)

    Sung-Kyung Kim

    2014-12-01

    Full Text Available Objectives: The purpose of this study is to report the clinical effect of Korean medicine (KM treatment for Henoch-Schonlein purpura (HSP. Methods: Five HSP patients who demonstrated a Yin deficiency and who had a history of a previous upper respiratory tract infection were included in this study. Four patients had arthritis and three had severe stomachache. One of them appeared to have proteinuria and hematuria before starting KM treatment. Results: All patients were improved with only herbal medicine, Jarotang (JRT. Purpura in the lower extremities and abdominal pain, which were not treated by using a corticosteroid, disappeared and had not recurred after 6 months. Conclusion: These cases indicate that JRT may be effective in treating HSP in patients who demonstrate Yin deficiency, even though the number of cases was limited to five.

  5. Acute compartment syndrome of the hand in Henoch-Schonlein Purpura

    Directory of Open Access Journals (Sweden)

    Luis Guntur E

    2007-03-01

    Full Text Available Abstract An eight year old boy with Henoch-Schonlein Purpura (HSP presented with acute compartment syndrome (ACS of his left hand following arterial cannulation of his radial artery in intensive care unit. Emergency decompression and fasciotomy were performed. The authors report this first case in literature and discuss how HSP can be complicated by ACS and ways to prevent the latter from happening.

  6. Research on return visit and investigation of the relapse rate of children allergic purpura after treatment.

    Science.gov (United States)

    Yang, Weihong; Zhou, Yudong; Zhang, Sisen; Yang, Fang; Zhuang, Tanyue; Wu, Xinyan

    2015-01-01

    This paper aimed to research on return visit and investigation of the relapse rate of children allergic purpura after treatment. Children with allergic purpura were divided into two groups. The treatment group was treated with the adrenocorticotrophic hormone while the control group did not. We tracked and observed two groups of discharged children in the first month and the second month. It can be found that, at the first month, 5 cases recurred in the treatment group with 20 cases, the relapse rate was 25%, 1 case recurred in control group, the relapse rate was 5%; at the second month, 2 cases recurred in treatment group, the relapse rate was 10%, no case recurred in the control group. There were 8 cases recurred in the past two months, and there were no replace after the second time treatment. In contrast, the children, who treated with adrenocorticotrophic hormone, had higher relapse rate, while the control group had lower relapse rate. Then we can get the conclusion that, the application of adrenocorticotrophic hormone may be one of the main reasons to induce the allergic purpura and we should notice and discuss this conclusion in the clinical practice.

  7. Spontaneous Bacterial Peritonitis and Henoch-Schönlein Purpura in a Patient with Liver Cirrhosis

    Directory of Open Access Journals (Sweden)

    Neil Gupta

    2015-01-01

    Full Text Available Henoch-Schönlein purpura (HSP is a small vessel systemic vasculitis, predominantly affecting children, characterized by a tetrad of manifestations, specifically palpable purpura, arthralgia, abdominal pain, and renal disease. HSP in the adult population is rare, and no case has been described of HSP in liver cirrhosis with spontaneous bacterial peritonitis (SBP. We present the case of a 58-year-old male with liver cirrhosis, who was subsequently diagnosed with SBP and later HSP. In this patient, the diagnosis of HSP was demonstrated clinically by his palpable purpura, diarrhea, hematuria, and abdominal pain and confirmed pathologically by his renal and skin biopsies demonstrating leukocytoclastic vasculitis and IgA complexes. We believe that this is an example of altered IgA processing in cirrhosis leading to the development of IgA immune complexes and ultimately HSP. The patient additionally had SBP, which may have increased his risk for developing HSP given antigen processing by mucosa-associated lymphoid tissues leading to immune complex deposition, which may not have been effectively cleared in the context of his liver disease. The patient unfortunately died of gastrointestinal hemorrhage, which is unclear to be due to his underlying cirrhosis or a gastrointestinal manifestation of HSP itself.

  8. Mechanisms of Alloimmunization and Subsequent Bone Marrow Transplantation Rejection Induced by Platelet Transfusion in a Murine Model

    Science.gov (United States)

    Patel, Seema R; Smith, Nicole H; Kapp, Linda; Zimring, James C

    2015-01-01

    For many non-malignant hematological disorders, HLA-matched bone marrow transplantation (BMT) is curative. However, due to lack of neoplasia, the toxicity of stringent conditioning regimens is difficult to justify, and reduced-intensity conditioning is used. Unfortunately, current reduced-intensity regimens have high rates of BMT rejection. We have recently reported in a murine model that mHAs on transfused platelet products induce subsequent BMT rejection. Most non-malignant hematological disorders require transfusion support prior to BMT and the rate of BMT rejection in humans correlates to the number of transfusions given. Herein, we perform a mechanistic analysis of platelet transfusion induced BMT rejection and report that unlike exposure to alloantigens during transplantation, platelet transfusion primes alloimmunity but does not stimulate full effector function. Subsequent BMT is itself an additional and distinct immunizing event, which does not induce rejection without antecedent priming from transfusion. Both CD4+ and CD8+ T cells are required for priming during platelet transfusion, but only CD8+ T cells are required for BMT rejection. In neither case are antibodies required for rejection to occur. PMID:22300526

  9. High multi-cytokine levels are not a predictive marker of alloimmunization in transfused sickle cell disease patients.

    Science.gov (United States)

    Tatari-Calderone, Zohreh; Fasano, Ross M; Miles, Megan R; Pinto, Ligia A; Luban, Naomi L C; Vukmanovic, Stanislav

    2014-07-01

    Patients with sickle cell disease (SCD) receive multiple red blood cell (RBC) transfusions for both prevention of and therapy for disease-related complications. In some patients, transfusion results in development of both allo- and auto-antibodies to RBC antigens. What precipitates the antibody formation is currently unclear. It has been hypothesized that a pro-inflammatory state preceding the therapeutic transfusion may be a predisposing factor. Plasma levels of ten cytokines were evaluated upon recruitment to the study of 83 children with SCD undergoing therapeutic RBC transfusions. The levels of cytokines were correlated with development of anti-RBC antibodies prior, or during seven years post recruitment. Twelve subjects displayed significantly higher levels of all cytokines examined, with pro-, as well as anti-inflammatory properties. Surprisingly, the elevated levels of cytokines were preferentially found in patients without anti-RBC allo- and/or auto-antibodies. Further, presence of high cytokine levels was not predictive of anti-RBC antibody development during the subsequent seven year follow up. These data suggest that the increased concentration of multiple cytokines is not a biomarker of either the presence of or susceptibility to the development of RBC alloimmunization.

  10. 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

  11. Red cell alloimmunization and infectious marker status (human immunodeficiency virus, hepatitis B virus and hepatitis C virus in multiply transfused thalassemia patients of North India

    Directory of Open Access Journals (Sweden)

    Raj Nath Makroo

    2013-01-01

    Full Text Available Background: Patients with thalassemia major are largely transfusion dependent and are thus exposed to a variety of risks such as transmission of infectious diseases, iron overload and alloimmunization. This study was performed to determine the prevalence of human immune deficiency virus (HIV, hepatitis B virus (HBV, hepatitis C virus (HCV and red cell antibodies among multiple-transfused thalassemic patients in and around the national capital region. Materials and Methods: The Department of Transfusion Medicine, Indraprastha Apollo Hospitals, conducted this study in collaboration with the National Thalassemia Welfare Society over a period of 1 year starting February2011. Blood samples from the patients were tested for blood group, red cell alloantibody/ies, anti-HIV, anti-HCV and hepatitis B surface antigen (HBsAg by ELISA and for the respective viral ribonucleic acid (RNA or deoxyribonucleic acid (DNA by nucleic acid testing (NAT. Results: A total of 462 thalassemics which consists of 290 males and 172 females were tested. The overall alloimmunization rate was 4.1% and anti-Kell was the most common antibody identified. Thirteen cases (2.8% were positive for HBsAg by ELISA, 107 (23.1% were reactive for anti HCV and 11 (2.38% for anti HIV antibodies. Further screening and discriminatory assays by NAT confirmed the presence of HBV DNA in 11 cases, HIV RNA in 7 cases and HCV RNA in 48 cases. Conclusion: In spite of advances in Immunohematology and infectious marker testing in recent years, the rates of alloimmunization and infectious marker positivity remains high among multiply transfused patients like thalassemics. Provision of safe and adequate blood supply to these patients is a key to improving their quality-of-life and longevity.

  12. Sirolimus for Autoimmune Disease of Blood Cells

    Science.gov (United States)

    2017-03-16

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

  13. Acquired platelet function defect

    Science.gov (United States)

    Acquired qualitative platelet disorders; Acquired disorders of platelet function ... blood clotting. Disorders that can cause problems in platelet function include: Idiopathic thrombocytopenic purpura Chronic myelogenous leukemia Multiple ...

  14. 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.

  15. Diagnostic value of polymerase chain reaction analysis of skin biopsies in purpura fulminans.

    Science.gov (United States)

    Beau, Caroline; Vlassova, Natalia; Sarlangue, Jean; Brissaud, Olivier; Léauté-Labrèze, Christine; Boralevi, Franck

    2013-01-01

    Even though prompt diagnosis and treatment of purpura fulminans (PF) is essential to reduce mortality, early administration of antibiotics may preclude identification of the causative agent by standard bacterial cultures and thus render definitive diagnosis impossible. Here we present a case of an infant with PF and negative bacterial cultures for whom polymerase chain reaction (PCR) analysis of a cutaneous biopsy specimen obtained 4 days after initiation of antibiotics identified the genomic sequence of Neisseria meningitidis genogroup C. When bacterial cultures fail to provide useful information, PCR of skin biopsy specimens can be a valuable diagnostic tool in PF.

  16. 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.

  17. 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...

  18. 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.

  19. 小儿特发性血小板减少性紫癜外周血T、B淋巴细胞观察%The observation of T and B lymphocyte in peripheral of infantile idiopathic thrombocytopenic purpura

    Institute of Scientific and Technical Information of China (English)

    白文杰; 翟凤兰

    2000-01-01

    Objective: To detect the ratio changes of subgroup T lymphocyte and B lymphocyte in infants with ITP. Methods:Manufacturing single nucleus cell with lymphocyte, separate liquid, adding antibody after thermal cultivation and calculatingthe percentage of positive cells using inmuno-fluorescence method. Results: There are no statistie differences between the ITPpatients' T3、T4 and the healthy contral' s there are statistie differences between the ITP patients' T8、 B groups and the healthycontral' s. Conclusion: The patients of infantile ITP may have humoral and cellular immunity factional disorder.%目的:检测ITP患儿T淋巴细胞亚群及B细胞的比值变化。方法:采用荧光免疫法用淋巴细胞分离液配制单个细胞,温育后加入抗体,计算阳性百分率。结果:患儿组T3、T4与健康组差异不显著,T8及B细胞患儿组与健康组差异有显著性。结论:ITP患儿可出现细胞免疫及体液免疫功能紊乱。

  20. 选择素在脾切除治疗特发性血小板减少性紫癜中的意义%Sificance of selectin in treatment of idiopathic thrombocytopenic purpura with splenectomy

    Institute of Scientific and Technical Information of China (English)

    秦锡虎; 陈易人; 华铮; 黄伯华; 邱国强; 曹苇

    2001-01-01

    目的研究特发性血小板减少性紫癜(ITP)时脾脏血管内皮细胞的活化程度,了解脾切除后选择素(selectin)水平的变化,探讨脾切除治疗的ITP的指征.方法A组为12例激素疗效不佳,术前sE-selectin、sP-selectin/Plt水平明显高于正常的ITP病例;B组为8例肝炎后性肝硬化继发脾机能亢进病人.二组病人均行脾切除,术前、术后检测血小板(Plt)、选择素(P-selectin、L-selectin)、可溶性选择素(sE-selectin、sL-selectin、sP-selectin).结果A组术后获随访11例,10例反应极好,1例反应良好.术前sE-selectin水平A、B两组均高于正常,术后以近似幅度逐步下降;A组术前P-selectin明显高于B组(16.8%vs5.2%,P<0.05),术后快速下降;A组术前sP-selectin/Plt远高于B组(7.55vs2.03fg/plt,P=0.02),术后1个月两组均下降至正常水平;A组sE-selectin、P-selectin、sP-selectin/Plt在术后6个月仍维持低水平.A、B两组术前和术后L-selectin、sL-selectin水平无明显差异.结论E-selectin、P-selectin参与了ITP血小板破坏的病理过程.sE-selectin、sP-selectin/Plt水平可作为术前预测ITP脾切除疗效的参考指标,高者可能手术效果较好.

  1. 选择素在特发性血小板减少性紫癜中的表达%The selectin expression in patients with idiopathic thrombocytopenic purpura

    Institute of Scientific and Technical Information of China (English)

    钱珂; 秦锡虎; 邱国强

    2006-01-01

    目的研究选择素的表达在特发性血小板减少性紫癜(ITP)发生中的作用.方法激素治疗ITP23例中,13例疗效不良者为A组,10例疗效良好者为B组;另8例肝硬化脾亢者为C组,15名健康献血者为D组.抽全血用ELISA法检测可溶性E-选择素(E-selectin)、可溶性L-选择素(sL-selectin)、可溶性P-选择素(sP-selectin);通过流式细胞仪检测P-选择素(P-selectin)及L-选择素(L-selectin).结果血小板计数,A组为34.2×109/L,B组35.6×109/L,C组56.3×109/L,D组166×109/L.A组sE-selectin水平为53.1ng/ml,明显高于D组(28.3ng/ml,P<0.05);B组为21.2ng/ml;C组61.8ng/ml,也高于D组(P<0.05).A组sP-selectin水平上升(15.3ng/ml),高于B组(3.2ng/ml,P<0.01)和D组(10.9ng/ml);A组sP-selectin/Plt显著高于D组(6.9 vs 0.6fg/platelet、P<0.01);B、C组P-selectin低于D组,但sP-selectin/Plt却高于D组.A、B、C 3组sL-selectin、L-selectin水平与D组无明显差异.结论 P-selectin、E-selectin参与了ITP时血小板破坏的病理过程,sE-selectin、sP-selectin/Plt明显上升的ITP患者激素治疗效果可能较差,白细胞的活化与否和ITP无关.

  2. Nursing Care for One Patient Suffering from Thrombotic Thrombocytopenic Purpura and Hemolytic Uremic Syndrome After Caesarean Birth%1例剖宫产术后并发TTP-HUS患者的护理

    Institute of Scientific and Technical Information of China (English)

    刘波; 张鹏; 刘丽云

    2009-01-01

    对1例剖宫产术后并发血栓性血小板减少性紫癜-溶血性尿毒综合征(TTP-HUS)患者予激素、血浆置换、抗感染、对症支持等治疗,患者好转出院.提出严密观察病情变化,加强心理、基础、饮食、药物不良反应、血浆置换的护理及健康教育,可促进患者康复.

  3. Treatment for Serious Immune Thrombocytopenic Purpura%重症免疫性血小板减少性紫癜紧急救治方案的探讨

    Institute of Scientific and Technical Information of China (English)

    王静; 孙慧; 马杰

    2010-01-01

    为了探讨重症免疫性血小板减少性紫癜(ITP)的治疗方案,回顾性分析118例接受激素(92例)或丙种球蛋白(16例)或rhTPO(10例)治疗患者的临床资料,分析疗效及不良反应.结果显示:(1)激素组,丙种球蛋白组和rhTPO组总有效率分别为76.1%、81.3%和70%,组间差异无统计学意义.复发组有效率(67.3%)明显低于初治组(83.3%,P<0.05),PAIgG伴PAIgM升高组有效率明显低于不伴PAIgM升高组(P<0.05);(2)血小板达峰时间:激素组为(5.70±3.68)d,丙种球蛋白组为(4.31±0.75)d,rhTPO组为(5.00±1.63)d,激素组与丙种球蛋白组间差异有统计学意义(P<0.05);(3)不良反应:激素组均出现类库欣综合征表现,丙种球蛋白组未见不良反应,1例(10%)rhTPO组患者出现轻度转氨酶增高.总之,丙种球蛋白和rhTPO治疗成人重症ITP疗效好,不良反应轻微.

  4. Posterior reversible encephalopathy syndrome as a complication of Henoch-Schönlein purpura in a seven-year-old girl.

    Science.gov (United States)

    Dos Santos, Daiane; Langer, Felipe Welter; Dos Santos, Tatiane; Rafael Tronco Alves, Giordano; Feiten, Marisa; Teixeira de Paula Neto, Walter

    2017-02-01

    Introduction Henoch-Schönlein purpura is a multisystem small vessel vasculitis. Neurologic manifestations are uncommon. Posterior reversible encephalopathy syndrome is a rare complication of Henoch-Schönlein purpura with typical clinical and neuroimaging findings that occurs most commonly in the setting of severe hypertension and renal injury. Case presentation A seven-year-old girl was admitted to our institution presenting with clinical and laboratory findings suggestive of Henoch-Schönlein purpura. Glucocorticoid therapy was initiated, but five days following her admission, she developed altered consciousness, seizures, arterial hypertension, and cortical blindness. Brain MRI scan revealed areas of vasogenic oedema in parieto-occipital lobes, consistent with posterior reversible encephalopathy syndrome. She was immediately initiated on antihypertensives and antiepileptics, which successfully improved her neurologic symptoms. Further laboratory work-up disclosed a rapidly progressive glomerulonephritis secondary to Henoch-Schönlein purpura that was the likely cause of her sudden blood pressure elevation. Immunosuppressive therapy was undertaken, and at one-year follow-up, the patient exhibited complete renal and neurologic recovery. Conclusion Posterior reversible encephalopathy syndrome is a severe complication of Henoch-Schönlein purpura. If promptly diagnosed and treated, children with Henoch-Schönlein purpura presenting with posterior reversible encephalopathy syndrome usually have a good prognosis. Clinicians should be familiar with the characteristic presentation of posterior reversible encephalopathy syndrome and be aware that hypertension and renal injury may predispose Henoch-Schönlein purpura patients to developing this complication.

  5. The Development of Severe Neonatal Alloimmune Thrombocytopenia due to Anti-HPA-1a Antibodies Is Correlated to Maternal ABO Genotypes

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    Maria Therese Ahlen

    2012-01-01

    Full Text Available Background. Maternal alloantibodies against HPA-1a can cross placenta, opsonize foetal platelets, and induce neonatal alloimmune thrombocytopenia (NAIT. In a study of 100, 448 pregnant women in Norway during 1995–2004, 10.6% of HPA-1a negative women had detectable anti-HPA-1a antibodies. Design and Methods. A possible correlation between the maternal ABO blood group phenotype, or underlying genotype, and severe thrombocytopenia in the newborn was investigated. Results. We observed that immunized women with blood group O had a lower risk of having a child with severe NAIT than women with group A; 20% with blood group O gave birth to children with severe NAIT, compared to 47% among the blood group A mothers (relative risk 0.43; 95% CI 0.25–0.75. Conclusion. The risk of severe neonatal alloimmune thrombocytopenia due to anti-HPA-1a antibodies is correlated to maternal ABO types, and this study indicates that the observation is due to genetic properties on the maternal side.

  6. T-cell responses associated with neonatal alloimmune thrombocytopenia: isolation of HPA-1a-specific, HLA-DRB3*0101-restricted CD4+ T cells.

    Science.gov (United States)

    Ahlen, Maria Therese; Husebekk, Anne; Killie, Mette Kjaer; Skogen, Bjørn; Stuge, Tor B

    2009-04-16

    T-cell responses have been implicated in the development of HPA-1a-induced neonatal alloimmune thrombocytopenia (NAIT). However, HPA-1a-specific T cells have neither been isolated nor characterized. Here, we aimed to determine whether HPA-1a-specific T cells could be isolated from HPA-1a-immunized women. In the present study, peripheral blood mononuclear cells (PBMCs) from an HPA-1a-alloimmunized woman were cultured for weeks in the presence of HPA-1a peptide, labeled with CFSE, and assayed for antigen-specific proliferation. Individual proliferating cells were isolated by fluorescence-activated cell sorting and expanded in culture. Antigen specificity and HLA restriction were determined by cytokine secretion (enzyme-linked immunospot [ELISPOT]) and proliferation assays. Several CD3(+)CD4(+) T-cell clones were isolated that proliferated and secreted cytokines in response to HPA-1a peptide. Two of these clones have been established in long-term culture in our laboratory. Both of these recognize synthetic as well as naturally processed HPA-1a antigen, and the recognition is restricted by the MHC molecule HLA-DRB3*0101 that is strongly associated with NAIT. These HPA-1a-specific T-cell clones represent unambiguous evidence for the association of T-cell responses with NAIT, and they will serve as unique tools to elucidate the cellular immune response that may result in NAIT.

  7. Association of pseudomembranous colitis with Henoch-Schönlein purpura.

    Science.gov (United States)

    Hayakawa, Takehiko; Imaeda, Hiroyuki; Nakamura, Mitsuyasu; Komoto, Shunsuke; Maruta, Kazuo; Shiozu, Hidetoshi; Ogata, Haruhiko; Iwao, Yasushi; Ishii, Hiromasa; Hibi, Toshifumi

    2005-06-01

    A 79-year-old man was admitted because of cholecystitis that occurred about 40 days after sigmoidectomy had been performed for colonic cancer. Though antibiotics improved his condition, the patient had hematochezia, diarrhea, and left lower abdominal pain. Colonoscopic findings showed multiple ring-like areas of redness and petechiae in the rectosigmoid colon and marked edema from the descending to the transverse colon. The patient then developed purpura on the extensor surfaces of the legs and bilateral gonalgia, and exacerbation of the hematochezia. A second colonoscopy (CS) showed multiple ring-like areas of redness and ecchymosis throughout the colon. The patient was diagnosed with Henoch-Schönlein purpura (HSP), and the symptoms were attenuated after the administration of prednisolone. However, diarrhea recurred in about a week; stool culture confirmed Clostridium difficile, and a third CS revealed pseudomembranes throughout the colon. The patient was diagnosed with pseudomembranous colitis (PMC), and the administration of vancomycin attenuated the symptoms. In conclusion, we have reported a rare adult case of PMC that occurred during prednisolone treatment for HSP. The PMC may have been caused by changes in the intestinal bacterial flora after the sigmoidectomy and by the intestinal lesions of HSP, as well as by the administration of antibiotics after the sigmoidectomy and for the treatment of cholecystitis, and by the use of prednisolone for the treatment of the HSP.

  8. Central Nervous System Involvement in Henoch-Schonlein Purpura in Children and Adolescents

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    Iliyana H. Pacheva

    2017-01-01

    Full Text Available Central nervous system (CNS involvement in Henoch-Schonlein purpura (HSP is rare but poses diagnostic difficulties. The aim of the study was to establish the frequency of CNS involvement in HSP, to analyze its clinical characteristics and do a literature review. Medical files of patients with HSP admitted at the Department of Pediatrics, Plovdiv, were studied retrospectively for a five-year period (2009–2013. Diagnosis was based on the American College of Rheumatology criteria. Out of 112 children with HSP 1 case (0.9% had CNS involvement presenting as Posterior Reversible Encephalopathy Syndrome (PRES, which may be a result of CNS vasculitis or arterial hypertension. It was an 8-year-old girl with atypical HSP which started with abdominal pain requiring surgery. On the third day after the operation a transient macular rash and arterial hypertension appeared, followed by visual disturbances, hemiconvulsive epileptic seizures, postictal hemiparesis, and confusion. Head CT showed occipital hypodense lesions and MRT-T2 hyperintense lesion in the left occipital lobe. The patient experienced a second similar episode after 2 weeks when palpable purpura had also appeared. Neurological symptoms and MRI resolved completely. HSP can be an etiological factor for PRES in childhood. Although PRES is a rare complication of HSP, clinicians must be aware of it and avoid diagnostic and therapeutic delays.

  9. Henoch-Schönlein purpura associated with Strongyloides stercoralis infection

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    Janković Sveta

    2016-01-01

    Full Text Available Introduction. Henoch-Schönlein purpura (HSP is a small blood vessel vasculitis, which usually manifests during childhood. The exact cause of the disease is unknown. Case report. We reported a 14-year-old girl who had been admitted to our clinic due to the appearance of red macules on her extremities and face, vomiting, and pain in the abdomen and joints. The patient was initially diagnosed with Henoch- Schönlein purpura. At the end of the fourth week of illness, larvae of Strongyloides stercoralis were detected in stool samples. The patient was therefore treated with mebendazole, after which all symptoms permanently withdrew. About a month later laboratory examinations were repeated demonstrating increasing signs of renal damage. Kidney biopsy was performed, showing mesangioproliferative glomerulonephritis with crescents and IgA and C3 positive staining in the mesangium. Upon reviewing the clinical presentation, biochemically demonstrated progressive renal damage and biopsy results, the patient was diagnosed with HSP nephritis. Conclusion. The time course of the disease and present knowledge concerning the pathogenic mechanisms of HSP suggest that Strongyloides stercoralis infection could have caused HSP in the presented patient, which was complicated by nephritis.

  10. HENOCH SCHONLEIN PURPURA ASSOCIATED WITH ACUTE POSTSTREPTOCOCCAL GLOMERULONEPHRITIS: A CASE REPORT

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    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]  

  11. Chylothorax in Henoch-Schonlein purpura: a case report and review of the literature.

    Science.gov (United States)

    Cogar, Bryan D; Groshong, Ted D; Turpin, Brian K; Guajardo, Jesus R

    2005-06-01

    Henoch-Schonlein purpura (HSP) is the most common acute vasculitis in the pediatric population, with an incidence of 10-14 per 100,000. The classic presentation of this disorder includes erythematous papules followed by palpable purpura in the lower extremities, trunk, and face, arthralgia or arthritis, abdominal pain, gastrointestinal bleeding, and nephritis. While renal abnormalities in HSP are common, the classic pulmonary manifestations, such as hemorrhage and pneumonitis, are thought to be infrequent. Subclinical pulmonary manifestations, including diffusion defects and radiographic anomalies, seem to be quite frequent in patients with HSP but are not commonly reported. Other respiratory manifestations include pleural effusion and chylothorax, but these are rarely mentioned in the literature. Chylothorax was only reported once in an adult patient with HSP in whom the mechanism of formation was demonstrated to be secondary to transdiaphragmatic passage of chylous fluid from the peritoneal cavity. Here we describe an 8-year-old girl with HSP, nephrotic syndrome, and chylothorax, and we report the results of a review of the literature regarding respiratory complications in HSP. The present case is the first pediatric patient reported with HSP and chylothorax. The therapeutic measures utilized were effective in resolving her edema, ascites, and chylothorax, and we advocate the use of these measures as first-line therapy in future patients with similar complications from HSP.

  12. Central Nervous System Involvement in Henoch-Schonlein Purpura in Children and Adolescents

    Science.gov (United States)

    Ivanov, Ivan S.; Stefanova, Krastina; Chepisheva, Elena; Chochkova, Lyubov; Grozeva, Dafina; Stoyanova, Angelina; Milenkov, Stojan; Stefanova, Penka; Petrova, Anna

    2017-01-01

    Central nervous system (CNS) involvement in Henoch-Schonlein purpura (HSP) is rare but poses diagnostic difficulties. The aim of the study was to establish the frequency of CNS involvement in HSP, to analyze its clinical characteristics and do a literature review. Medical files of patients with HSP admitted at the Department of Pediatrics, Plovdiv, were studied retrospectively for a five-year period (2009–2013). Diagnosis was based on the American College of Rheumatology criteria. Out of 112 children with HSP 1 case (0.9%) had CNS involvement presenting as Posterior Reversible Encephalopathy Syndrome (PRES), which may be a result of CNS vasculitis or arterial hypertension. It was an 8-year-old girl with atypical HSP which started with abdominal pain requiring surgery. On the third day after the operation a transient macular rash and arterial hypertension appeared, followed by visual disturbances, hemiconvulsive epileptic seizures, postictal hemiparesis, and confusion. Head CT showed occipital hypodense lesions and MRT-T2 hyperintense lesion in the left occipital lobe. The patient experienced a second similar episode after 2 weeks when palpable purpura had also appeared. Neurological symptoms and MRI resolved completely. HSP can be an etiological factor for PRES in childhood. Although PRES is a rare complication of HSP, clinicians must be aware of it and avoid diagnostic and therapeutic delays.

  13. Bilateral cystoid macular oedema and cotton wool spots associated with Henoch-Schonlein purpura

    Science.gov (United States)

    Nicholson, Luke; Mathews, Divya

    2013-01-01

    We report a case of a male patient having bilateral cystoid macular oedema and cotton wool spots associated with Henoch-Schonlein purpura. He presented with decreased vision bilaterally measuring 0.2 (6/9.5) on the right and 0.5 (6/19) on the left. He has chronic hypertension with blood pressure averaging 150/90. His blood pressure was raised at 180/110 and with treatment reduced to 164/99. There were no other signs of malignant hypertensive retinopathy. He was treated with oral prednisolone and noticed a significant improvement the following day. An objective measurement performed 2 days later was −0.10 (6/4.8) bilaterally with resolution of macular oedema. PMID:23933864

  14. 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.

  15. Henoch-Schnlein purpura complicating adalimumab therapy for Crohn’s disease

    Institute of Scientific and Technical Information of China (English)

    Farooq; Z; Rahman; Gagandeep; K; Takhar; Ovishek; Roy; Anna; Shepherd; Stuart; L; Bloom; Sara; A; McCartney

    2010-01-01

    Anti-tumour necrosis factor-α(TNF) therapy has revolutionised the management of chronic inflammatory conditions.With ever increasing numbers of patients being treated with these agents,uncommon adverse reactions will inevitably occur more frequently.Cutaneous manifestations are associated with many of these chronic conditions and can complicate anti-TNF therapy in about 20% of cases.Vasculitic complications are rarely associated with anti-TNF therapy.Henoch-Schnlein purpura(HSP),a small vessel vasculitis,has been described following infliximab and etanercept therapy but never with adalimumab,a fully humanized TNF antibody.The risk of such immune-mediated reactions is theoretically less with adalimumab compared to infliximab but can still occur.Here we report the f irst case in the literature of HSP that can be attributed to the use of adalimumab in a 19-year-old male with recalcitrant Crohn’s disease.

  16. Strongyloides stercoralis hyperinfection in patient with autoimmune hepatitis and purpura fulminans

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    Neha Rathor

    2016-01-01

    Full Text Available Strongyloidiosis is usually an asymptomatic chronic nematodal disease. The term hyperinfection is used to denote autoinfection, a phenomenon in which the number of worms increases enormously. Development or exacerbation of gastrointestinal and pulmonary symptoms is seen, (A and the detection of increased numbers of larvae in stool and or sputum is the hallmark. It is known to occur with a change in immune status of the host; this can occur due to immunosuppressants. Cytomegalovirus (CMV is also known to suppress host immunity. Due to the nonspecific presentation, the diagnosis is frequently missed, and the outcome remains poor with 15-87% mortality despite therapy. We report here a case of Strongyloides stercoralis hyperinfection following immunosuppressive therapy for autoimmune hepatitis and concomitant CMV infection with purpura fulminance and frank sepsis, with fatal outcome.

  17. Pneumococcal Sepsis Complicated by Splenic Abscesses and Purpura Fulminans in a 15-Month-Old Child

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    Scott Pangonis MD

    2016-02-01

    Full Text Available Streptococcus pneumoniae is an invasive organism that causes a wide range of common diseases, including sinusitis, acute otitis media, and pneumonia. Splenic abscesses and purpura fulminans (PF are rare complications of pneumococcal disease. Splenic abscesses caused by S pneumoniae have only been reported in the adult literature. PF has been described in the pediatric population as a rare complication in patients with invasive pneumococcal disease (IPD with and without underlying immunological disorders such as asplenia. Here, we report a patient with IPD complicated by splenic abscesses and PF. Our patient initially presented with bacteremia, septic shock, and disseminated intravascular coagulation. She subsequently developed PF and splenic abscesses. She survived her illness after receiving a total of 8 weeks of antibiotic therapy. This case highlights 2 rare complications of IPD and demonstrates the need to keep pneumococcal disease in the differential diagnosis even in children whose vaccination status is up to date.

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

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    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.

  19. Characterization of a human platelet antigen-1a-specific monoclonal antibody derived from a B cell from a woman alloimmunized in pregnancy.

    Science.gov (United States)

    Eksteen, Mariana; Tiller, Heidi; Averina, Maria; Heide, Gøril; Kjaer, Mette; Ghevaert, Cedric; Michaelsen, Terje E; Ihle, Øistein; Husebekk, Anne; Skogen, Bjørn; Stuge, Tor B

    2015-06-15

    Human platelet Ag (HPA)-1a, located on integrin β3, is the main target for alloantibodies responsible for fetal and neonatal alloimmune thrombocytopenia (FNAIT) in the white population. There are ongoing efforts to develop an Ab prophylaxis and therapy to prevent or treat FNAIT. In this study, an mAb specific for HPA-1a, named 26.4, was derived from an immortalized B cell from an alloimmunized woman who had an infant affected by FNAIT. It is the only HPA-1a-specific human mAb with naturally paired H and L chains. Specific binding of mAb 26.4, both native and recombinant forms, to platelets and to purified integrins αIIbβ3 (from platelets) and αVβ3 (from trophoblasts) from HPA-1a(+) donors was demonstrated by flow cytometry and surface plasmon resonance technology, respectively. No binding to HPA-1a(-) platelets or integrins was detected. Moreover, the Ab binds with higher affinity to integrin αVβ3 compared with a second HPA-1a-specific human mAb, B2G1. Further in vitro experimentation demonstrated that mAb 26.4 can opsonize HPA-1a(+) platelets for enhanced phagocytosis by monocytes, inhibit binding of maternal polyclonal anti-HPA-1a Abs, and weakly inhibit aggregation of HPA-1a-heterozygous platelets, the latter with no predicted clinical relevance. Thus, mAb 26.4 is highly specific for HPA-1a and could potentially be explored for use as a prophylactic or therapeutic reagent for FNAIT intervention and as a phenotyping reagent to identify women at risk for immunization.

  20. Genetic homogeneity but IgG subclass-dependent clinical variability of alloimmune membranous nephropathy with anti-neutral endopeptidase antibodies.

    Science.gov (United States)

    Vivarelli, Marina; Emma, Francesco; Pellé, Thimothée; Gerken, Christopher; Pedicelli, Stefania; Diomedi-Camassei, Francesca; Klaus, Günter; Waldegger, Siegfried; Ronco, Pierre; Debiec, Hanna

    2015-03-01

    Alloimmune antenatal membranous nephropathy (MN) during pregnancy results from antibodies produced by a neutral endopeptidase (NEP)-deficient mother. Here we report two recent cases that provide clues to the severity of renal disease. Mothers of the two children had circulating antibodies against NEP showing the characteristic species-dependent pattern by immunofluorescence on kidney slices. A German mother produced predominantly anti-NEP IgG4 accompanied by a low amount of IgG1. Her child recovered renal function within a few weeks. In sharp contrast, an Italian mother mainly produced complement-fixing anti-NEP IgG1, which also inhibits NEP enzymatic activity, whereas anti-NEP IgG4 has a weak inhibitory potency. Her child was dialyzed for several weeks. A kidney biopsy performed at 12 days of age showed MN, ischemic glomeruli, and arteriolar and tubular lesions. A second biopsy performed at 12 weeks of age showed aggravation with an increased number of collapsed capillary tufts. Both mothers were homozygous for the truncating deletion mutation 466delC and were thus NEP deficient. The 466delC mutation, identified in three previously described families, suggests a founder effect. Because of the potential severity of alloimmune antenatal MN, it is essential to identify families at risk by the detection of anti-NEP antibodies and NEP antigen in urine. On the basis of the five families identified to date, we propose an algorithm for the diagnosis of the disease and the prevention of complications.

  1. Diagnosis of Henoch-Schönlein purpura: renal or skin biopsy?

    Science.gov (United States)

    Davin, Jean-Claude; Weening, Jan J

    2003-12-01

    Henoch-Schönlein purpura (HSP) is a form of systemic vasculitis characterized by vascular wall deposits of predominantly IgA, typically involving small vessels in skin, gut, and glomeruli and associated with purpura, intestinal colic, hematuria, and arthralgia or arthritis. HSP nephritis leads to chronic renal failure in up to 20% of pediatric patients after 20 years of follow-up in selected series. The risk is related to the initial clinical presentation and is maximal (more than 50%) when initial signs are a combination of nephrotic and nephritic syndromes. Although less important, the risk persists for mild renal symptoms or when the patient has apparently completely recovered from the renal disease. Other types of non-IgA-related leukocytoclastic vasculitis may be difficult to discriminate from HSP, thus confounding the diagnosis. The clinical picture of HSP is often incomplete and renal signs can become manifest years after initial signs. When based on clinical signs only, the diagnosis of HSP can therefore be missed, and some patients risk developing silent chronic renal failure after decades without appropriate treatment. Patients can also be overdiagnosed as HSP and thus submitted to unnecessary follow-up. It is therefore important that HSP should be correctly diagnosed from the initial signs. As the finding of IgA deposits in vessel walls associated with the characteristic signs of small-vessel vasculitis is a sine qua non in the diagnosis, a skin biopsy should be performed for histological and immunofluorescence studies in cases of clinical suspicion of HSP. The systematic diagnostic use of a cutaneous biopsy should not only improve the follow-up of patients with HSP but will also allow a reliable epidemiological study of vasculitis in children and a better knowledge of the disease.

  2. Henoch schonlein purpura--a 5-year review and proposed pathway.

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    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.

  3. Terminal Ileitis as a Feature of Henoch-Schönlein Purpura Masquerading as Crohn Disease in Adults.

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    Sampat, Hemal N; McAllister, Brian P; Gaines, Darryl D; Ostrov, Barbara

    2016-03-01

    Henoch-Schönlein purpura (HSP), more recently termed immunoglobulin A (IgA) vasculitis, is a systemic small-vessel vasculitis characterized by perivascular IgA deposition. This disease manifests clinically as palpable purpura, arthralgia, gastrointestinal symptoms, and renal dysfunction. Although ileitis can be seen in HSP, terminal ileitis is virtually pathognomonic for Crohn disease. We present a comprehensive review of the literature on this association, including 2 cases of our own, to demonstrate the importance of considering HSP in the differential diagnosis of ileitis suggestive of Crohn disease. We review the growing body of literature suggesting a pathophysiologic link between the conditions, possibly through an IgA-mediated mechanism.

  4. Comparison of platelet counts by sysmex XE 2100 and LH-750 with the international flow reference method in thrombocytopenic patients

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    Tina Dadu

    2013-01-01

    Full Text Available Background: There are several methods for counting platelets, of which the international flow reference method (IRM is considered to be the gold standard. We compared the platelet count given by this method to the count given by automated analyzers using other methods, such as optical fluorescence and impedance. Aims: The aim of this study is to compare the platelet counts obtained by Sysmex XE 2100 by Impedance (Sysmex-I, optical florescence (Sysmex-O and reported (Sysmex-R based on the switching algorithm and LH-750 by Impedance (LH-750 with the IRM in thrombocytopenic blood samples. To calculate the sensitivity, specificity, positive predictive value (PPV and negative predictive value (NPV of various technologies at the clinically relevant transfusion thresholds of 10 × 10 9 /l and 20 × 10 9 /l. Materials and Methods: A total of 118 blood samples with platelet count of <50 × 10 9 /l were selected for the study. Platelet counts of all samples were analyzed by all methods using the Sysmex analyzer, LH-750 and IRM in parallel within 6 h of collection. Statistical Analysis Used: Pearson correlation, bland Altman analysis, sensitivity and specificity, PPV and NPV. Results and Conclusions: Sysmex-R had the least Bias and 95% limits of agreement (95%LA range and thus correlated best with IRM values. LH-750 had a higher Bias compared to Sysmex-O and Sysmex-R, but a strikingly similar 95% LA ensures similar results in all three methods. In fact, in the oncology subset, it had the narrowest 95% LA, which made it the best performer in this subgroup. Of the three Sysmex results, Sysmex-I had the highest bias, widest 95% LA and highest potential risk of over transfusion. Hence, Sysmex-R and LH-750 were found to be reliable tools for estimation of platelet count in thrombocytopenic patients.

  5. Characteristic purpura of the ears, vasculitis, and neutropeniae–a potential public health epidemic associated with levamisole-adulterated cocaine

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    Chung, Catherine; Tumeh, Paul C.; Birnbaum, Ron; Tan, Belinda H.; Sharp, Linda; McCoy, Erin; Mercurio, Mary Gail; Craft, Noah

    2013-01-01

    Background Dermatologists at the University of California, San Francisco recently reported two patients in the online Journal of the American Academy of Dermatology with purpura presumably induced by levamisole in contaminated cocaine. Levamisole-induced vasculitis and neutropenia has been reported elsewhere in the United States and Canada. Up to 70% of cocaine in the United States could be contaminated. Objective We sought to describe similar cases of vasculitis associated with cocaine use. Methods This is a retrospective case series. Results We report 6 remarkably similar patients seen over just the past few months with retiform purpura on the body and tender purpuric eruptions, necrosis, and eschars of the ears after cocaine use in New York and California. All of these patients had positive perinuclear antineutrophil cytoplasmic antibody values and 3 of the 6 also had an associated neutropenia. Direct immunofluorescence studies suggested an immune complex–mediated vasculitis. Limitations This case series is descriptive in nature and, because testing is not easily performed, we did not test for levamisole in the serum or blood to prove this is the causative agent. Conclusion It appears the use of cocaine is associated with the peculiar clinical findings of ear purpura, retiform purpura of the trunk, and neutropenia. We believe this case series may represent the tip of the iceberg as a looming public health problem caused by levamisole. Although the direct causal relationship may be difficult to establish, the astute dermatologist or primary care physician should be able to recognize the characteristic skin lesions and should be wary of the potential development of agranulocytosis. (J Am Acad Dermatol 2011;65:722-5.) PMID:21658797

  6. 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

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    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

  7. A perspective on the measurement of ADAMTS13 in thrombotic thrombocytopaenic purpura.

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    Pimanda, John E; Chesterman, Colin N; Hogg, Philip J

    2003-04-01

    The recent discovery of the von Willebrand Factor (vWF) cleaving protease (ADAMTS13) and the association of its deficiency with thrombotic thrombocytopaenic purpura (TTP) has generated both enormous interest and considerable confusion. Ultra large von Willebrand Factor (UL vWF) multimers are present in the plasmas of patients with chronic relapsing TTP in remission but disappear during an attack. This observation led to the recognition that UL vWF multimers precipitate the thrombotic occlusion of arterioles, a feature that characterizes TTP. Multiple mutations in ADAMTS13 are associated with congenital TTP and neutralizing autoantibodies have been demonstrated in the acquired TTP syndrome. Although a number of functional assays for this enzyme have been described, the more rigorously evaluated assays are difficult to perform outside a research laboratory. There is also an enduring uncertainty about the specificity of ADAMTS13 deficiency for the diagnosis of acquired TTP and a perception that the result does not alter patient management. The cloning of the ADAMTS13 gene has also raised the prospect of recombinant enzyme therapy for the treatment of TTP, and this has heightened the need for a simple assay. In this review, we evaluate the value of measuring this enzyme in the management of TTP.

  8. [A case of disseminated nontuberculous mycobacteriosis during purpura associated with hypergammaglobulinemia and hepatitis type C].

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    Ueno, Shiro; Miyauchi, Shunichi; Umekita, Kunihiko; Kusumoto, Norio; Takajo, Ichiro; Kuroki, Masayuki; Kai, Yasufumi; Nagatomo, Yasuhiro; Okayama, Akihiko

    2008-11-01

    A 74-year-old woman with hepatitis due to hepatitis C virus followed up using oral predonisolone (3 mg/day) for two years because of hypergammaglobulinemia-associated purpura reported fever and lumbago in February 2005. Upon admission in June, she was found in chest-computed tomography to have atelectasia in the right middle lung lobe and a nodule with a cavity in the right lower lobe. She tested positive for tuberculous glycolipid antibody. Gallium scintigraphy showed an abnormal accumulation in the lower lumbar vertebra. Magnetic resonance imaging showed abnormal enhancement at L4, L5, and their intervertebral disc. Mycobacterium intracellulare (M. intracellulare) was detected in blood culture, bronchoalveolar lavage, and a biopsy specimen from the intervertebral disc, yielding a diagnosis of disseminated nontuberculous mycobacteriosis (NTM) due to M. intracellulare. She was treated with clarithromycin (CAM), ethambutol (EB), and rifampicin (RFP), but EB and RFP were discontinued due to of the fever they induced. She was then treated with a combination of CAM, levofloxacin, and streptomycin and followed up as an out patient. Based on case reports of disseminated NTM infection in Japan, the prognosis is poor and a protocol must be established for its treatment.

  9. Púrpura em paciente com estrongiloidíase disseminada Purpura in patient with disseminated strongiloidiasis

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    Luciano C. Ribeiro

    2005-05-01

    Full Text Available A infecção pelo Strongyloides stercoralis em associação com imunosupressão pode manifestar-se com lesões em múltiplos órgãos e sistemas, caracterizando a forma disseminada da doença. Lesões cutâneas não são freqüentemente relatadas e, se presentes, manifestam-se como rash e petéquias. Púrpuras bem definidas são pouco descritas. No presente trabalho é descrito um caso de estrongiloidíase disseminada, com acometimento cutâneo em forma de púrpura, que se desenvolveu em um paciente timectomizado e usuário crônico de corticosteróide devido à miastenia gravis.The association of systemic corticosteroid therapy and dissemination of Strongyloides stercoralis has been increasingly documented in the literature. Skin involvement in disseminated strongyloidiasis has been reported and the most commonly described cutaneous manifestations are rash and petechial eruptions. We present a case of an immunosuppressed man that developed disseminated strongyloidiasis with extensive purpura.

  10. Aloinmunización a un antígeno del sistema Rh de alta frecuencia Alloimmunization to a high frequency Rh antigen

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    Carlos Cotorruelo

    2006-02-01

    Full Text Available Describimos el caso de una embarazada sensibilizada con un aloanticuerpo anti-Rh17 de muy amplia reactividad. Los glóbulos rojos de la paciente presentaban una deleción parcial de los antígenos del sistema Rh, responsable de la aloinmunización encontrada. Debido a la dificultad de obtener sangre compatible se elaboró un plan de transfusión autóloga para cubrir las posibles demandas. El análisis molecular del locus RH demostró la presencia de un alelo híbrido RHCE-D(5-7-CE que generaba el fenotipo delecionado.We report the case of a pregnant woman sensitized with a panreactive anti-Rh17 alloantibody. Patient’s red blood cells showed a partial deletion of Rh antigens, which was responsible for the alloimmunization. An autotransfusion program was instrumented so as to cover possible demands. Molecular analysis of the RH locus showed the presence of a hybrid RHCE-D(5-7-CE allele that gave origin to the deleted phenotype.

  11. Hemolytic disease of the newborn associated with anti-Jra alloimmunization in a twin pregnancy: the first case report in Korea.

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    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.

  12. Calciphylaxis with peau d'orange induration and absence of classical features of purpura, livedo reticularis and ulcers.

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    Nahm, Walter K; Badiavas, Evangelos; Touma, Dany J; Wilkel, Caroline S; Falanga, Vincent

    2002-04-01

    Calciphylaxis is an ill-defined syndrome that is commonly associated with chronic renal failure. Its heterogeneous clinical features include painful livedo reticularis-like purpuric patches and plaques, vesicles, irregularly shaped ulcers, and black eschars. Despite demonstration of extensive vascular arteriolar calcification in this syndrome, its exact pathogenesis remains unknown. Here, we report a case of calciphylaxis presenting with indurated plaques without the usual clinical picture of livedo reticulate purpura, ulcers or necrotic eschars. This case provides an opportunity to review the clinical spectrum of calciphylaxis and to discuss the therapeutic approaches and pathogenesis of this syndrome from deep intra-wall vascular calcification to the resulting infarctions of adjacent tissues.

  13. Endothelin 1 levels in relation to clinical presentation and outcome of Henoch Schonlein purpura

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    Moustaki M

    2008-09-01

    Full Text Available Abstract Background Henoch Schonlein purpura (HSP is a common vasculitis of small vessels whereas endothelin-1 (ET-1 is usually reported elevated in vasculities and systematic inflammation. The aim of the present study was to investigate whether ET-1 levels are correlated with the clinical presentation and the outcome of HSP. Methods The study sample consisted of thirty consecutive patients with HSP. An equal number of healthy patients of similar age and the same gender were served as controls. The patients' age range was 2–12.6 years with a mean ± SD = 6.3 ± 3 years. All patients had a physical examination with a renal, and an overall clinical score. Blood and urinary biochemistry, immunology investigation, a skin biopsy and ET-1 measurements in blood and urine samples were made at presentation, 1 month later and 1 year after the appearance of HSP. The controls underwent the same investigation with the exception of skin biopsy. Results ET-1 levels in plasma and urine did not differ between patients and controls at three distinct time points. Furthermore the ET-1 were not correlated with the clinical score and renal involvement was independent from the ET-1 measurements. However, the urinary ET-1 levels were a significant predictor of the duration of the acute phase of HSP (HR = 0.98, p = 0.032, CI0.96–0.99. The ET-1 levels did not correlate with the duration of renal involvement. Conclusion Urinary ET-1 levels are a useful marker for the duration of the acute phase of HSP but not for the length of renal involvement.

  14. 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:

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

    Science.gov (United States)

    Veronese, F V; Dode, R S O; Friderichs, M; Thomé, G G; da Silva, D R; Schaefer, P G; Sebben, V C; Nicolella, A R; Barros, E J G

    2016-01-01

    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.

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

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    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.

  17. Significance of antineutrophil cytoplasmic antibody in adult patients with Henoch-Sch(o)nlein purpura presenting mainly with gastrointestinal symptoms

    Institute of Scientific and Technical Information of China (English)

    Yan Zhang; Yong-Kang Wu; Matthew A Ciorba; Qin Ouyang

    2008-01-01

    AIM: To test the clinical significance of antineutrophil cytoplasmic antibody (ANCA) in evaluation of adult Henoch-Schonlein purpura (HSP) patients presenting mainly with abdominal symptoms.METHODS: Twenty-eight consecutive HSP patients who presented predominantly with abdominal symptoms were enrolled in this study. Control subjects included 27 age-and sex-matched patients with peptic ulcer disease, colon cancer, acute gastroenteritis, irritable bowel syndrome and colonic polyps. ANCA was measured by indirect immunofluorescence (IIF) in all patients, and follow-up ELJSA was performed in patients with positive IIF tests.RESULTS: ANCA was detected in 9 HSP patients by IIF (2 were positive for c-ANCA and 7 were positive for p-ANCA). No ANCA was found in the control group. The sensitivity and specificity of a positive ANCA test (either c- or p-ANCA) were 32.1% and 100% respectively. Only one out of the 9 patients with positive ANCA by IIF had positive ANCA by ELISA and the antigen was myeloperoxidase (MPO). The patients positive for ANCA had higher HSP clinical scores, and were more likely to have renal function impairment. Patients with late purpura development were also associated with more severe clinical manifestations.CONCLUSION: A positive ANCA test is associated with more severe symptoms in HSP. After inflammatory bowel disease is excluded, a positive ANCA test provides a clue to the diagnosis of HSP presenting predominantly with abdominal symptoms.

  18. Co-stimulation with LPS or Poly I:C markedly enhances the anti-platelet immune response and severity of fetal and neonatal alloimmune thrombocytopenia.

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    Li, Conglei; Chen, Pingguo; Vadasz, Brian; Ma, Li; Zhou, Hui; Lang, Sean; Freedman, John; Ni, Heyu

    2013-12-01

    Fetal and neonatal alloimmune thrombocytopenia (FNAIT) is a life-threatening bleeding disorder caused by maternal antibodies against fetal/neonatal platelets. FNAIT is also linked with miscarriages, although the incidence and mechanisms of fetal death have not been well studied. IntegrinαIIbβ3 (GPIIbIIIa) and the GPIbα complex are major glycoproteins expressed on platelets and are also major antigens targeted in autoimmune thrombocytopenia (ITP), but reported cases of anti-GPIb-mediated FNAIT are rare. Bacterial and viral infections have been causally linked with the pathogenesis of immune-mediated thrombocytopenia (ITP); however, it is unknown whether these infections contribute to the severity of FNAIT. Here, immune responses against platelet antigens were examined by transfusing wild-type (WT) mouse platelets into β3-/- or GPIbα-/- mice. To mimic bacterial or viral infections, lipopolysaccharide (LPS) or polyinosinic:polycytidylic acid (Poly I:C) were injected intraperitoneally following platelet transfusions. The FNAIT model was established by breeding the immunised female mice with WT male mice. We demonstrated for the first time that the platelet GPIbα has lower immunogenicity compared to β3 integrin. Interestingly, co-stimulation with LPS or Poly I:C markedly enhanced the immune response against platelet GPIbα and caused severe pathology of FNAIT (i.e. miscarriages). LPS or Poly I:C also enhanced the immune response against platelet β3 integrin. Our data suggest that bacterial and viral infections facilitate the anti-platelet GPIbα response, which may lead to a severe non-classical FNAIT (i.e. miscarriage but not neonatal bleeding) that has not been adequately reported in humans.

  19. 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

  20. Experience of buffy coat pooling of platelets as a supportive care in thrombocytopenic dengue patients: A prospective study

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    Kabita Chatterjee

    2014-01-01

    Full Text Available Random donor platelet (RDP is not sufficient to improve the platelet count in most thrombocytopenic patients. Single donor platelet (SDP or buffy coat pooled platelet (BCPP are the two choices to provide a full therapeutic dose of platelets. However, there are constraints in the preparation of SDP due to stringent donor selection procedure, time required for procedure, and need of special expensive equipments and kits. BCPP is widely practiced, especially in the European countries, since 1995. In India, we decided to adopt the procedure of buffy coat pooling of platelets, especially for economically backward patients and for emergencies. This study was prospectively conducted from September 2009 to September 2010. A total of 129 units of BCPP [tested prior for viral markers by enzyme-linked immunosorbent assay (ELISA and individual donor nucleic acid amplification test (ID-NAT] were issued to 129 patients suffering from dengue and were included in this study. For comparison between efficacy of SDP and BCCP, patients were divided into two groups of 50 each. The post-transfusion platelet counts of the patients were noted after 2 hours of transfusion for each type of component. The platelet yield varied from 2.5 to 4.4 Χ 10ΉΉ in BCPP samples. The samples analyzed were sterile without any contamination. The different biochemical parameters were analyzed in detail. The observed post-transfusion platelet recovery and corrected count increment (CCI at 1 hour and 24 hours after BCPP transfusion were similar to that after SDP transfusion. Hence, we concluded that BCPP can be a low cost alternative to SDP in the times of emergencies like dengue and non-affordability by the patient for SDP.

  1. Henoch-Schonlein Purpura in Children: Retrospective evaluation of 53 cases

    Directory of Open Access Journals (Sweden)

    Samet Ozer

    2015-09-01

    Full Text Available Objective: Henoch-Schonlein Purpura (HSP is the most commonly observed vasculitis during childhood and is a systemic disease which may mainly affect skin, joint, kidney and gastrointestinal system. The aim of the study is to evaluate clinical and laboratory findings of followed patients with HSP diagnosis between the years 2010 and 2015. Materials and Methods: In the study, the reports of 53 patients were retrospectively evaluated who diagnosed with HSP and were treated and followed in the department of pediatrics in Medical Faculty of Tokat Gaziosmanpasa University during the years from 2010 to 2015. The diagnosis was realized provided that at least two criteria of HSP diagnosis, which were created by American College of Rheumatology in 1990, was present. Non-steroid anti-inflammatory and/or corticosteroid was used for the treatment. Results: Of 53 patients, 28 were female and 25 were male. The age range of the patients was 3 to 17 years and median age was 9,8 years. A clinical finding, skin rash was present in all patients. 24 patients (45.3% had arthritis/arthralgia, 33 patients (62.3% had stomachache and 6 patients (11.3% had headache. Proteinuria was observed in 22 patients (41%, hematuria in 9 (17%, occult blood positivity in stool in 20 (37.7%. 25 patients (47% had kidney involvement and 33 patients (62.3% had gastrointestinal system involvement. Steroid treatment was given to 20 patients (37.7% with gastrointestinal system involvement. 6 patients (11% with severe kidney findings were transferred to another health care facility with a child nephrology department. 15 patients (28.3% had recurrent skin rash six months after a complete recovery. Conclusion: HSP is a vasculitis that can involve mostly skin and joints and other organs and systems. It was observed that it has a good short-term prognosis and patients with the case might heal without any sequela. Moreover, the cases with HSP should be closely followed for complications such as

  2. EULAR/PRINTO/PRES criteria for Henoch-Schönlein purpura, childhood polyarteritis nodosa, childhood Wegener granulomatosis and childhood Takayasu arteritis: Ankara 2008. Part II: Final classification criteria

    DEFF Research Database (Denmark)

    Ozen, Seza; Pistorio, Angela; Iusan, Silvia M

    2010-01-01

    To validate the previously proposed classification criteria for Henoch-Schönlein purpura (HSP), childhood polyarteritis nodosa (c-PAN), c-Wegener granulomatosis (c-WG) and c-Takayasu arteritis (c-TA)....

  3. Risk Factors Associated with Renal Involvement in Childhood Henoch-Schönlein Purpura: A Meta-Analysis

    Science.gov (United States)

    Chan, Han; Tang, Yan-Ling; Lv, Xiao-Hang; Zhang, Gao-Fu; Wang, Mo; Yang, Hai-Ping; Li, Qiu

    2016-01-01

    Background and objective Henoch-Schönlein purpura (HSP) is an important cause of chronic kidney disease in children. This meta-analysis identified risk factors associated with renal involvement in childhood HSP. Methods PubMed, Embase, and Web of Science were searched. The quality of all eligible studies was assessed using the Newcastle-Ottawa scale criteria. An analysis of possible risk factors was conducted to report the odds ratio (OR) and weighted mean difference (WMD). Results Thirteen studies (2398 children) revealed 20 possible and 13 significant risk factors associated with renal involvement in HSP, with the following meta-analysis estimates of OR and WMD, with 95% confidence intervals: older age (0.90, 0.61–1.19); age > 10 y (3.13, 1.39–7.07); male gender (1.36, 1.07–1.74); abdominal pain (1.94,1.24–3.04); gastrointestinal bleeding (1.86, 1.30–2.65); severe bowel angina (3.38, 1.17–9.80); persistent purpura (4.02, 1.22–13.25); relapse (4.70, 2.42–9.14); WBC > 15 × 109/L (2.42, 1.39–4.22); platelets > 500 × 109/L (2.98, 1.22–7.25); elevated antistreptolysin O (ASO) (2.17, 1.29–3.64); and decreased complement component 3 (C3) (3.13, 1.62–6.05). Factors not significantly associated with renal involvement were: blood pressure; orchitis; elevated C-reactive protein; elevated erythrocyte sedimentation rate (ESR); and elevated serum IgA/IgE or IgG. Arthritis/arthralgia may be a risk factor according to the criteria of the American College of Rheumatology (1.41, 1.01–1.96). Conclusion The following are associated with renal involvement in pediatric HSP: male gender; > 10 y old; severe gastrointestinal symptoms (abdominal pain, gastrointestinal bleeding, and severe bowel angina); arthritis/arthralgia; persistent purpura or relapse; WBC > 15 × 109/L; platelets > 500 × 109/L; elevated ASO; and low C3. Relevant clinical interventions for these risk factors may exert positive effects on the prevention of kidney disease during the early

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

    Directory of Open Access Journals (Sweden)

    Risa Yamada

    2011-01-01

    Full Text Available Thrombotic thrombocytopenia purpura (TTP caused by a deficiency in ADAMTS-13 activity is considered to involve a subset of thrombotic microangiopathy (TMA. Although concept of TTP is included under the umbrella of TMA, discrimination of TTP from TMA is occasionally difficult in an autoimmune disorder. Herein, we report a case with TTP associated with systemic lupus erythematosus (SLE. In this case, it was difficult to discriminate TTP from TMA and the measurement of ADAMTS-13 activity was useful for obtaining an accurate diagnosis. SLE patients having thrombocytopenia in complication with anemia should be considered a monitoring of ADAMTS-13 activity even though the patients lacked symptoms of TTP related to the microvascular coagulation.

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

    Directory of Open Access Journals (Sweden)

    Howarth Charles B

    2011-08-01

    Full Text Available Abstract Introduction Henoch-Schönlein purpura is the most common systemic vasculitis in children. Typical presentations are palpable purpura, abdominal pain, arthritis, and hematuria. This vasculitic syndrome can present as an uncommon cause of rectal bleeding in older patients. We report a case of an older man with Henoch-Schönlein purpura. He presented with rectal bleeding and acute kidney injury secondary to IgA mesangioproliferative glomerulonephritis. Case presentation A 75-year-old Polish man with a history of diverticulosis presented with a five-day history of rectal bleeding. He had first noticed colicky left lower abdominal pain two months previously. At that time he was treated with a 10-day course of ciprofloxacin and metronidazole for possible diverticulitis. He subsequently presented with rectal bleeding to our emergency department. Physical examination revealed generalized palpable purpuric rash and tenderness on his left lower abdomen. Laboratory testing showed a mildly elevated serum creatinine of 1.3. Computed tomography of his abdomen revealed a diffusely edematous and thickened sigmoid colon. Flexible sigmoidoscopy showed severe petechiae throughout the colon. Colonic biopsy showed small vessel acute inflammation. Skin biopsy resulted in a diagnosis of leukocytoclastic vasculitis. Due to worsening kidney function, microscopic hematuria and new onset proteinuria, he underwent a kidney biopsy which demonstrated IgA mesangioproliferative glomerulonephritis. A diagnosis of Henoch-Schönlein purpura was made. Intravenous methylprednisolone was initially started and transitioned to prednisone tapering orally to complete six months of therapy. There was marked improvement of abdominal pain. Skin lesions gradually faded and gastrointestinal bleeding stopped. Acute kidney injury also improved. Conclusion Henoch-Schönlein purpura, an uncommon vasculitic syndrome in older patients, can present with lower gastrointestinal bleeding

  6. EB病毒感染对住院新生儿血小板减少的影响%Epstein-Barr Virus Infection of Newborn Thrombocytopenic

    Institute of Scientific and Technical Information of China (English)

    杨燕; 金晓希; 曾真

    2014-01-01

    Objective Inquired into EB virus infection of newborn thrombocytopenic.Methods A an-alysed 96 cases of newborn thrombocytopenic in 2 years,determine the IgM antibodies of EB virus capsid and IgG antibodies , than diagnostic the EBV DNA of EB virus.Their mothers was simultaneously tested as well.Results 96 cases of newborn with thrombocytopenia in 34 cases with EB virus infection.Among them, 30 cases (31.25%) were detected EBV DNA positive;10 cases (10.42%) were EBV-CA-IgM positive;19 cases (19.79%) of EBV-EA-D-IgG positive;mothers were confirmed to be infected in 23 cases, 20 of them were EBV DNA positive and seven of them were EBV-CA-IgM positive, ten cases were EBV-EA-D-IgG posi-tive.Conclusion EB virus infection of newborn thrombocytopenic show the symptoms such as critical condi -tion , various of complications and slow recovery.Better curative effect can be accomplished if it gets the diag-nosis and treatment on time.%目的:探讨EB病毒感染对住院新生儿血小板减少的影响。方法分析2年来本院收治的96例新生儿血小板减少患儿,均检测EB病毒衣壳Ig M抗体、早期抗原IgG抗体及检测EBV DNA测定EB病毒;并对其母亲进行同期检测。结果96例血小板减少患儿中检出EB病毒感染34例,其中30例(31.25%)检测出EBV DNA阳性,EBV-CA-IgM阳性10例(10.42%),EBV-EA-D-IgG阳性19例(19.79%);患儿母亲确证感染23例,其中EBV DNA阳性20例, EBV-CA-IgM阳性7例,EBV-EA-D-IgG阳性10例。结论 EB病毒感染引起住院新生儿血小板减少症患者程度重,合并症多且恢复慢,如早诊断并及时治疗可取得较好疗效。

  7. One case of Henoch-Schonlein purpura with the onset of orchitis%以睾丸炎起病的过敏性紫癜1例

    Institute of Scientific and Technical Information of China (English)

    高璐璐; 黄玉柱; 张开俊; 陈红; 高奎武

    2011-01-01

    @@ 过敏性紫癜(Henoch-Schonlein purpura,HSP)是一种以小血管炎为主要病变的血管炎综合征,多发生于学龄前期和学龄期儿童,男多于女[1].其发病率,临床症状多变性呈现逐年增高的趋势[2].

  8. The accuracy of platelet counting in thrombocytopenic blood samples distributed by the UK National External Quality Assessment Scheme for General Haematology.

    Science.gov (United States)

    De la Salle, Barbara J; McTaggart, Paul N; Briggs, Carol; Harrison, Paul; Doré, Caroline J; Longair, Ian; Machin, Samuel J; Hyde, Keith

    2012-01-01

    A knowledge of the limitations of automated platelet counting is essential for the effective care of thrombocytopenic patients and management of platelet stocks for transfusion. For this study, 29 external quality assessment specimen pools with platelet counts between 5 and 64 × 10(9)/L were distributed to more than 1,100 users of 23 different hematology analyzer models. The same specimen pools were analyzed by the international reference method (IRM) for platelet counting at 3 reference centers. The IRM values were on average lower than the all-methods median values returned by the automated analyzers. The majority (~67%) of the automated analyzer results overestimated the platelet count compared with the IRM, with significant differences in 16.5% of cases. Performance differed between analyzer models. The observed differences may depend in part on the nature of the survey material and analyzer technology, but the findings have implications for the interpretation of platelet counts at levels of clinical decision making.

  9. 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, ...

  10. Haematology and neurology

    Science.gov (United States)

    Austin, Steven; Cohen, Hannah; Losseff, Nick

    2007-01-01

    This review aims to update the reader on advances in the understanding of haematological conditions that may arise in neurological practice. Thrombophilia, antiphospholipid antibody syndrome, thrombotic thrombocytopenic purpura, sickle cell and clonal disorders associated with neuropathy are discussed. PMID:17369588

  11. Nye behandlingsmuligheder ved primær immun trombocytopeni

    DEFF Research Database (Denmark)

    Gudbrandsdottir, Sif; Frederiksen, Henrik; Birgens, Henrik Sverre

    2011-01-01

    Primary immune thrombocytopenia (ITP)--formerly known as idiopathic thrombocytopenic purpura--is an autoimmune disorder characterized by immune mediated thrombocytopenia. The aetiology of ITP remains unknown, but studies have shown that multiple immunological mechanisms are involved in the pathog...

  12. Changes of serum IL-6, IL-18 and IFN-γ in children with idiopathic thrombocytopenic purura%IL-6、IL-18和IFN-γ在特发性血小板减少性紫癜中的水平变化

    Institute of Scientific and Technical Information of China (English)

    路桂云

    2013-01-01

    目的 通过测定特发性血小板减少性紫癜(ITP)患儿血清中白细胞介素-6(IL-6)、白细胞介素-18(IL-18)和γ-干扰素(IFN-γ)的含量变化,探讨IL-6、IL-18和IFN-γ在ITP的发病机制中的作用.方法 选择ITP急性发作期患儿40例(急性组),经治疗后处于缓解期的患儿35例(缓解组),另选择30名健康儿童作为对照(对照组),采用酶联免疫法(ELISA)检测血清IL-6、IL-18和IFN-γ水平变化,并进行统计学分析比较.结果 ITP患儿血清IL-6、IL-18和IFN-γ水平急性期组明显高于缓解期组和对照组,缓解期组亦高于对照组,差异均有统计学意义(P均<0.05).结论 IL-6、IL-18和IFN-γ可能均参与了ITP的发病机制,通过测定血清IL-6、IL-18和IFN-γ水平可以用来判断ITP的病情和预后.%Objective To study the content changes of serum IL-6,IL-18 and IFN-γ in children with idiopathic thrombocytopenic purpura (ITP),and to investigate the role in the pathogenesis of IL-6,IL-18 and IFN-γin ITP.Methods Active group (n =40) and remission group (n =35) with ITR were picked up,and 30 healthy children served as normal controls.IL-6,IL-18 and IFN-γ levels were measured by ELISA,and the results were compared.Results The serum levels of IL-6,IL-18 and IFN-γ in children with active group were markedly higher than those in remission group and healthy controls,and the serum levels of IL-6,IL-18 and IFN-γin remission group were markedly higher than those in healthy controls (P < 0.05).Conclusions IL-6,IL-18 and IFN-γmaybe involved in the pathogenesis of ITP.Determination of IL-6,IL-18 and INF-γ levels can be used to judge the ITP' s condition and prognosis.

  13. 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

  14. 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.

  15. Analysis of dietary effect factors of childhood allergic purpura%儿童过敏性紫癜饮食影响因素分析

    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

    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 developing allergic purpura.%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.

  16. Integrative Treatment of Children with Allergic Purpura%中西医结合治疗小儿过敏性紫癜

    Institute of Scientific and Technical Information of China (English)

    陈淑荣; 王志刚

    2014-01-01

    Analysis of curative ef ect of combined traditional Chinese and Western medicine treatment of al ergic purpura,46 cases of al ergic purpura patients on 2010 ~2012 years were divided into control group, treatment group,In the two group were given bed rest, light, free of fish eggs and milk diet, while the control group was treated with oral cetirizine, static point of etamsylate, abdominal type purpura with glucocorticoid treatment, the treatment group was treated by Chinese herb on the basis of the control group, the treatment group 23 cases, ef ective in 15 cases, ef ective 7 cases, invalid 1 cases, the total ef iciency of 95. 6%. A control group of 23 cases, 13 cases markedly ef ective, ef ective 6 cases, invalid 4 cases, the total ef iciency of 82. 6%.%分析中西医结合治疗过敏性紫癜的疗效,对2010年~2012年收治的46例过敏性紫癜患者分成对照组、治疗组,两组均在给予卧床休息、清淡、免鱼虾蛋奶饮食的基础上,对照组予口服西替利嗪、静点酚磺乙胺、腹型紫癜加糖皮质激素治疗,治疗组在对照组的基础上予中药治疗,治疗组23例,显效15例,有效7例,无效1例,总有效率95.6%。对照组23例,显效13例,有效6例,无效4例,总有效率82.6%。

  17. The Thinking and Method of TCM in Treating Allergic Purpura%中医药治疗过敏性紫癜的思路与方法

    Institute of Scientific and Technical Information of China (English)

    夏小军; 段赟

    2016-01-01

    The causes and the pathogenesis of allergic purpura were explored from five aspects: innate defi-ciency, invasion of external pathogen, irregular diet, insect bites,Qideficiency failing to control, blood loss,Yin-de-ficiency and fire hyperactivity, fire burning blood vessels, blood stasis obstructing the collaterals, blood failing to stay in the meridians, allergic purpura were divided into four patterns: heat hurting blood vessels,Yin-deficiency and fire hyperactivity,Qifailing to control blood, and treated by selfmade purpura prescriptions series, simultaneously, the patterns were differentiated according to the causes′to clear heat, detoxify, dispel the wind, promote blood circu-lation and eliminate blood stasis, invigorate spleen-stomach.%从禀赋薄弱、感受外邪,饮食不节、昆虫叮咬,气虚不摄、血溢脉外,阴虚火旺、灼伤血络,瘀血阻络、血不归经5方面探讨过敏性紫癜的病因病机,并辨证为热伤血络、阴虚火旺、瘀血阻络、气不摄血4种分型,用自拟紫癜系列方加减治疗,并注重审证求因,清热解毒祛风,并活用活血化瘀方法,同时勿忘健运脾胃。

  18. 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.

  19. Guidelines on the use of intravenous immune globulin for hematologic conditions.

    Science.gov (United States)

    Anderson, David; Ali, Kaiser; Blanchette, Victor; Brouwers, Melissa; Couban, Stephen; Radmoor, Paula; Huebsch, Lothar; Hume, Heather; McLeod, Anne; Meyer, Ralph; Moltzan, Catherine; Nahirniak, Susan; Nantel, Stephen; Pineo, Graham; Rock, Gail

    2007-04-01

    Canada's per capita use of intravenous immune globulin (IVIG) grew by approximately 115% between 1998 and 2006, making Canada one of the world's highest per capita users of IVIG. It is believed that most of this growth is attributable to off-label usage. To help ensure IVIG use is in keeping with an evidence-based approach to the practice of medicine, the National Advisory Committee on Blood and Blood Products of Canada (NAC) and Canadian Blood Services convened a panel of national experts to develop an evidence-based practice guideline on the use of IVIG for hematologic conditions. The mandate of the expert panel was to review evidence regarding use of IVIG for 18 hematologic conditions and formulate recommendations on IVIG use for each. A panel of 13 clinical experts and 1 expert in practice guideline development met to review the evidence and reach consensus on the recommendations for the use of IVIG. The primary sources used by the panel were 3 recent evidence-based reviews. Recommendations were based on interpretation of the available evidence and where evidence was lacking, consensus of expert clinical opinion. A draft of the practice guideline was circulated to hematologists in Canada for feedback. The results of this process were reviewed by the expert panel, and modifications to the draft guideline were made where appropriate. This practice guideline will provide the NAC with a basis for making recommendations to provincial and territorial health ministries regarding IVIG use management. Specific recommendations for routine use of IVIG were made for 7 conditions including acquired red cell aplasia; acquired hypogammaglobulinemia (secondary to malignancy); fetal-neonatal alloimmune thrombocytopenia; hemolytic disease of the newborn; HIV-associated thrombocytopenia; idiopathic thrombocytopenic purpura; and posttransfusion purpura. Intravenous immune globulin was not recommended for use, except under certain life-threatening circumstances, for 8 conditions

  20. 过敏性紫癜患儿血清过敏原检测应用%Application of serum allergen detection for children with allergic purpura

    Institute of Scientific and Technical Information of China (English)

    曾子坤

    2016-01-01

    Objective To investigate clinical significance by serum allergen detection in diagnosis of children with allergic purpura. Methods A total of 120 children with allergic purpura received serum allergen detection of immune globulin E (IgE) antibody for allergen identification. Results The 120 cases had total positive rate of inhaled allergen as 54.17% (65 cases), with proportion as dermatophagoides farina > mould > tree pollen > house dust > hair and scurf of cat and dog > common ragweed artemisia, cockroach, mosquito. Their total positive rate of food allergen was 35.00% (42 cases), with proportion as peanut, soybean > shrimp, crab > pork, beef, mutton > egg > fish > milk. Conclusion Under certain circumstance, allergic purpura can be induced by various allergen. Therefore, both convention therapy and allergen detection are necessary in clinical treatment to identify allergen and restrain occurrence of allergic purpura.%目的:探究血清过敏原检测在过敏性紫癜患儿诊断中的临床意义。方法120例过敏性紫癜患儿,检测其血清中过敏原特异性免疫球蛋白 E(IgE)抗体等,并找出过敏原。结果120例患儿中,其中总吸入过敏原阳性率为54.17%(65例),过敏原占比由高到低排列为:尘粉螨>霉菌>树花粉>屋尘>猫狗毛发皮屑>普通豚草蒿属及蟑螂、蚊子;总食物过敏原阳性率为35.00%(42例),过敏原占比由高到低排列为:花生、大豆>虾、蟹>猪肉、牛肉、羊肉>蛋类>鱼>牛奶。结论多种过敏原在一定条件下均可诱发过敏性紫癜,因此,在临床治疗中既需要进行常规治疗,也应进行过敏原检测,以查找出过敏原,减少紫癜的发生。

  1. 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.

  2. 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

  3. Clinicopathological features and prognosis of membranoproliferative-like Henoch-Schönlein purpura nephritis in children

    Institute of Scientific and Technical Information of China (English)

    Yan-Jie Huang; Ying Ding; Xiao-Qing Yang; Wen-Sheng Zhai; Xian-Qing Ren; Qing-Yin Guo; Xia Zhang; Meng Yang; Tatsuo Yamamoto; Yuan Sun

    2015-01-01

    Background: The aim of this retrospective study was to defi ne the clinical manifestations, pathological features and prognosis of children with membranoproliferative-like Henoch-Schönlein purpura nephritis (HSPN), representing International Study of Kidney Disease in Children (ISKDC) grade VI. Methods: Among 245 patients with HSPN treated in our hospital between 2008 and 2010, nine patients (3.7%) were diagnosed with HSPN of ISKDC grade VI (males=5, females=4, age: 9.5±2.03 years, mean±SD). The clinical features, laboratory and pathologicalfi ndings, treatment and outcome of the 9 patients were retrospectively analyzed. Results: Of the 9 patients, 7 (78%) presented with hematuria and nephrotic syndrome, and were treated with steroids (oral prednisone or intravenous methylprednisolone pulse therapy) and immunosuppressants (oral tripterygium glycosides or intravenous cyclophosphamide pulse therapy). One (11%) patient had hematuria and nephrotic range proteinuria (>50 mg/kg per 24 hours) and was treated with oral prednisone and tripterygium glycosides. Another (11%) patient presented with hematuria and moderate proteinuria (25-50 mg/kg per 24 hours) and was treated with oral tripterygium glycoside only. Histopathological examination showed diffuse glomerular mesangial and endocapillary proliferation, mesangial interposition, double-contour formation, podocyte hypertrophy, shedding, and cytoplasmic absorption droplets. The percentages of glomeruli with small cellular crescents varied from 4%-25% in 6 of 9 patients. Follow-up for 2 to 4 years showed excellent recovery in all patients. Conclusions: The main clinical feature of ISKDC grade VI HSPN in children is a nephrotic syndrome with hematuria. The excellent prognosis of the disease was probably related to early diagnosis and treatment with steroids and/or immunosuppressants, and mild degree of glomerulosclerosis and tubulointerstitial damage.

  4. A Case of Henoch-Schonlein Purpura Associated with Rotavirus Infection in an Elderly Asian Male and Review of the Literature

    Science.gov (United States)

    Tang, Chen; Scaramangas-Plumley, Daphne; Nast, Cynthia C.; Mosenifar, Zab; Edelstein, Marc A.; Weisman, Michael

    2017-01-01

    Patient: Male, 73 Final Diagnosis: Henoch-Schönlein purpura (HSP) Symptoms: Abdominal pain • bloating • blood in stool • nausea • vomiting Medication: — Clinical Procedure: EGD • colonoscopy • kidney biopsy • skin biopsy • arthrocentesis Specialty: Rheumatology Objective: Unknown ethiology Background: Henoch-Schönlein purpura (HSP), a small vessel vasculitis mediated by deposition of immune-complexes containing IgA in the skin, gut, and glomeruli, often presents with abdominal pain, purpuric rash in the lower extremities and buttocks, joint pain, and hematuria. The disease most commonly targets children but can affect adults who tend to have a worse prognosis. Case Report: We discuss a case of HSP in an elderly Chinese male who presented with severe proximal bowel inflammation, vasculitic rash, and proteinuria; he was found to have positive stool rotavirus and giardia. He improved significantly with high dose steroids. We believe rotavirus may have been a triggering event in this patient. A brief review of the literature is also presented. Conclusions: This is the first case report describing a classic presentation of HSP in an adult following a rotavirus infection. HSP can cause significant morbidity and mortality in adult patients predominantly from progressive renal failure; therefore careful management and monitoring is important. GI infections seem to be a common trigger for HSP and this case report suggests that rotavirus may be part of the spectrum. PMID:28174414

  5. Palpable purpura complicated by streptococcal toxic shock syndrome resulting in limb necrosis and amputation: a case of levamisole and cocaine coingestion.

    Science.gov (United States)

    Freyer, Craig W; Peters, Michael

    2012-02-01

    Palpable purpura resulting from cocaine and levamisole coingestion has been reported with increasing frequency over the last several years as distribution of this drug combination becomes more universal. Toxicity from ingestion of this dangerous combination is difficult to diagnose due to the multitude of possible clinical presentations, variety of possible adulterants, and elusive nature of levamisole given its short half-life and limited availability of detection methods. Levamisole is a chemotherapeutic and immunomodulatory agent currently marketed as a veterinary anthelmintic. We describe the case of a 48-year-old woman admitted to our intensive care unit with a diagnosis of streptococcal toxic shock syndrome (STSS), confirmed from fluid taken from an elbow lesion that grew Streptococcus pyogenes. She was noted to have bullae of the elbow and diffuse purpura with necrotic centers covering a large portion of her body (trunk, legs, arms, back, toes, fingers, and tip of nose). On further evaluation, she was found to have ingested levamisole-tainted cocaine. The patient's complications related to either cocaine and levamisole coingestion or STSS included thrombocytopenia, acute renal failure, and limb necrosis. Thrombocytopenia gradually improved upon treatment with prednisone, and acute renal failure improved with intravenous fluid resuscitation; however, she subsequently required several appendage amputations due to severe gangrene. Clinicians must have high suspicion for ingestion of this drug combination and request prompt testing of urine samples for levamisole if a patient who admits to illicit drug use presents with purpuric or necrotic skin lesions.

  6. Henoch-Schönlein purpura (IgA vasculitis developing after postoperative wound infection by methicillin-resistant Staphylococcus aureus

    Directory of Open Access Journals (Sweden)

    Masataka Satoh

    2016-04-01

    Full Text Available Henoch-Schönlein purpura (HSP is an acute small-vessel leukocytoclastic vasculitis, affecting the skin, joints, gastrointestinal tract and kidneys. Its prognosis depends on the severity of nephritis. A wide variety of pathogens, drugs, and other environmental exposures have been associated with HSP. Although group A β-haemolytic streptococcus has been the most studied, the majority of cases showed no direct link to streptococcal infection. Here we report a case of methicillin-resistant Staphylococcus aureus (MRSA infection-associated HSP. A 68-year-old woman underwent a coronary artery bypass surgery. After the surgery, a postoperative chest wound was infected by MRSA and sternal osteomyelitis developed. Palpable purpura then appeared on the extremities, followed by hematuria, proteinuria and increased serum creatine. Treatments with antibiotics and debridement of the infected wound and sequestrum resulted in rapid improvement of skin symptoms. Renal function partially recovered, however mild hematuria and proteinuria remained. Published work review and the present case suggest that Staphylococcal infection-associated HSP frequently involves kidney disease and its prognosis is likely to be poor compared to a common type of HSP. Further studies are needed to establish an appropriate treatment strategy for Staphylococcal infection-associated HSP.

  7. 重组人瘦素对慢性特发性血小板减少性紫癜患者骨髓巨核细胞的影响%Effect of recombinant human leptin on megakaryocyte in patients with chronic idiopathic thrombocytopenic purpura

    Institute of Scientific and Technical Information of China (English)

    王晓燕; 蔡艳霞; 田文洪; 赵友恒; 杨仁池

    2014-01-01

    目的:探讨瘦素对 CITP 骨髓巨核细胞的影响。方法以急性巨核细胞白血病细胞株 M07e 和 CITP 骨髓 CD34+细胞经诱导分化的巨核细胞为研究对象。运用 RT-PCR 检测 M07e 细胞瘦素受体 mRNA 表达,运用 Brdu-ELISA 法检测瘦素对 M07e 细胞的增殖作用,运用 Annexin V/ PI 双标流式检测瘦素对 M07e 细胞的凋亡作用。经TPO、SCF 诱导磁珠分选后获得的 CITP 骨髓 CD34+细胞分化为巨核细胞,运用流式测定瘦素对其分化过程中CD41a +、CD61+的表达以及 CD41a +细胞的凋亡是否有影响。结果瘦素受体长型 Ob-RL 和短型 Ob-RS 在 M07e细胞均有 mRNA 表达。瘦素对 M07e 细胞的增殖有促进作用,并呈现一定的剂量依赖性,浓度为5 ng/ ml 即显示出促增殖效应(P =0.037),但浓度在5、10、25 ng/ ml 之间无明显统计学差异,浓度为100 ng/ ml 时其增殖效应最大。在时间依赖效应上,瘦素作用48 h,其细胞增殖明显高于24 h,而作用72 h 其细胞增殖介于两者之间(P =0.000)。瘦素对 M07e 细胞具有抑制凋亡作用(P =0.001),而且在作用72小时后凋亡率最高。瘦素对骨髓 CD34+细胞分化为巨核细胞过程中 CD41a +、CD61+表达的影响,与不加瘦素组相比无明显统计学差异(P >0.05)。对 CD41a +细胞凋亡的影响与不加瘦素组相比亦无明显统计学差异(P >0.05)。结论瘦素通过促进 M07e 细胞的增殖,抑制其凋亡来参与巨核细胞白血病的发生、发展。瘦素在 TPO + SCF 诱导 CITP 骨髓巨核细胞分化中无明显协同作用,对分化产生 CD41a +巨核细胞的凋亡亦无明显作用。%Objective:To explore whether leptin plays a role in megakaryocyte of CITP. Methods:Megakaryocytic leu-kemia cell line M07e,megakaryocyte differentiated from bone marrow CD34 + cells of CITP were used in this study. The mR-NA expression of leptin receptor(ob-R)in M07e was detected by RT-PCR. The proliferative effect of leptin on M07e was investigated with Brdu-ELISA assay and the apoptosis of cells was measured with flow cytometry after Annexin V labeling and PI staining . Bone marrow CD34 + cells were selected by magnetic cell sorting(MACS),and TPO,SCF were used in the expansion system of megakaryocyte progenitor. The influences of leptin on the expression of megakaryocytic specific mono-clonal antibodies CD41a ,CD61 and the apoptosis of megakaryocyte were measured with flow cytometry. Results:There was mRNA expression of ob-RL and ob-RS in M07e. Leptin promoted the proliferation of M07e in a dose and time dependent manner. The effect of proliferation was the most significant at the concentration of 100ng/ ml and after cultured for 48h. Lep-tin inhibited the apoptosis of M07e. Compared with the control group,leptin had no effect on the expression of CD41a , CD61 and the apoptosis of megakaryocyte during the period of megakaryocyte differentiation from bone marrow CD34 + cells of CITP. Conclusion:Leptin may play an important role in human megakaryocytic leukemia by promoting the proliferation and inhibiting the apoptosis of M07e. Leptin had no synergistic effect on megakaryocyte differentiation of ITP induced by TPO and SCF. There was also no significant influence on the apoptosis of CD41a + cells.

  8. Recombinant Human Thrombopoietin and Human Gamma Globulin Treatment of Refractory Thrombocytopenic Purpura%重组人血小板生成素与人血丙种球蛋白治疗难治性血小板减少性紫癜

    Institute of Scientific and Technical Information of China (English)

    席壮新

    2015-01-01

    目的:比较重组人血小板生成素(rhTPO)与人血丙种球蛋白治疗难治性血小板减少性紫癜的临床疗效.方法:选取难治性血小板减少性紫癜患者80例随机分为3组,rhTPO组皮下注射rhTPO,对照组予糖皮质激素治疗,人血丙球组在对照组治疗基础上给予人血丙种球蛋白;比较3组出血停止时间、血小板开始上升时间、血小板达正常时间、血小板升高达最高峰时间、停药后恢复到治疗前水平时间,观察3组患者疗效和不良反应.结果:rhTPO组、人血丙球组总有效率均高于对照组,差异有统计学意义(P<0.05),rhTPO组、人血丙球组总有效率差异元统计学意义(P>0.05);人血丙球组血小板开始上升时间、血小板达正常时间均短于rhTPO组,rhTPO组血小板升高达最高峰时间及停药后恢复到治疗前水平时间长于人血丙球组,差异具有统计学意义(P<0.05);rhTPO组与人血丙球组比较,不良反应发生率差异无统计学意义(P>0.05).结论:rhTPO与人血丙种球蛋白均可有效治疗难治性血小板减少性紫癜,丙球起效更为快速,但rhTPO疗效更持久.

  9. The Effects of Thrombopoietin and Interleukin-11 on Bone Marrow Megakaryocytic Progenitors in Patients with Chronic Idiopathic Thrombocytopenic Purpura in Vitro%血小板生成素和白细胞介素-11对慢性特发性血小板减少性紫癜巨核细胞的影响

    Institute of Scientific and Technical Information of China (English)

    廖小梅; 唐雪梅; 邓承祺; 牛挺; 孟文彤

    2001-01-01

    目的探讨重组人血小板生成素(rhTPO)及联用重组人白细胞介素-11(rhIL-11)对慢性特发性血小板减少性紫瘢(CITP)患者骨髓巨核祖细胞增殖和成熟的影响.方法采用血浆凝块法对21例CITP患者骨髓巨核祖细胞进行体外培养,培养体系中加入不同浓度的rhTPO,或同时加rhIL-11两者联用.培养14天后经SZ-21(GPⅢa)单抗和ABC试剂盒染色观察集落生长数,用MCDS-2010型超清晰度骨髓细胞分析系统进行巨核细胞的面积和直径测定.结果CITP组骨髓巨核细胞面积及直径明显低于对照组(P<0.05).rhTPO对CITP患者的巨核细胞集落形成单位(CFU-MK)总集落数、巨核细胞直径与面积均有促进作用,但无浓度依赖性.体外最适浓度为10ng/ml;rhTPO与rhIL-11联用组较单用组的CFU-MK、总集落数及面积、直径均显著增加.结论CITP患者骨髓巨核细胞存在成熟障碍,体外rhTPO单用及与rhIL-11联用均可促进CITP患者巨核祖细胞的增殖与成熟,其联用较单用促进作用更强.

  10. Therapeutic effects of rhTPO combining intravenous immunoglobulin on children with severe refractory idiopathic thrombocytopenic purpura%rhTPO联合静脉注射免疫球蛋白治疗儿童难治性重型特发性血小板减少性紫癜疗效观察

    Institute of Scientific and Technical Information of China (English)

    马平

    2016-01-01

    目的 评价重组人血小板生成素(rhTPO)联合静脉注射免疫球蛋白治疗儿童难治性ITP的临床疗效.方法 对18例儿童难治性重型ITP进行rhTPO联合静脉注射免疫球蛋白治疗,rhTPO给予每次300U/kg,皮下注射,隔日一次,共7次,同时联合静脉注射免疫球蛋白每次5.0g,每周一次共4次,于rhTPO应用的第1天开始应用.结果 18例儿童难治性重型ITP有15例有效,其中6例完全缓解(随访中位时间6个月,有4例呈持续缓解),9例部分缓解(其中4例维持3~4个月后血小板再次下降至≤20×109/L),余3例患儿无效或血小板短暂性升高后快速下降至较低值.结论 rhTPO联合静脉注射免疫球蛋白可使部分难治性重型ITP患儿得到缓解,无明显毒副作用,可成为治疗儿童重型难治性ITP的一种方法.

  11. Corticosteroids Combined with Recombinant Human Thrombopoietin Treatment of Idiopathic Thrombocytopenic Purpura%糖皮质激素联合重组人促血小板生成素治疗特发性血小板减少性紫癜

    Institute of Scientific and Technical Information of China (English)

    杜以萍; 张充力; 毕珍宁

    2014-01-01

    目的 评价糖皮质激素联合重组人促血小板生成素(rhTPO)对特发性血小板减少性紫癜(ITP)患者的疗效和安全性.方法 治疗组21例ITP患者常规应用糖皮质激素同时皮下注射rhTPO 15 000 u/d;对照组20例常规应用糖皮质激素治疗,动态监测血小板生长情况,并比较两组治疗效果.结果 治疗组较对照组有效率明显提高(P<0.05);且治疗组血小板上升时间明显短于对照组(P<0.01);降低了ITP患者出血的危险性,且不良反应少.结论 糖皮质激素联合rhTPO治疗ITP疗效确切,用药安全.

  12. A clinical comparative study on treatment of idiopathic thrombocytopenic purpura with recombinant human thrombopoietin%重组人血小板生成素治疗特发性血小板减少性紫癜的临床对照试验

    Institute of Scientific and Technical Information of China (English)

    赵娟; 邹萍; 罗毅; 王晓聪; 洪梅

    2009-01-01

    目的:评价重组人血小板生成素(rhTPO)对特发性血小板减少性紫癜(ITP)的疗效和安全性.方法:21例ITP患者采用随机区组,分成试验组和对照组2组.试验组皮下注射rhTPO 1.0 μg/kg,1次/d,疗程14 d.对照组在服用达那唑14 d后如血小板仍≤20×109/L,加用rhTPO皮下注射,1.0 μg/kg,疗程14 d.2组在整个试验阶段均服用达那唑,0.2 g,每日3次.结果:试验组用药前血小板计数的中位数为8.0(4.0~15.0)×109/L,用rhTPO后血小板最高值为100.0(49.0~118.5) ×109/L,与用药前相比P<0.01.停药后血小板计数逐渐回落,至开始治疗后28 d,血小板计数降至39.0(22.0~68.5)×109/L.对照组用药前血小板计数的中位数为6.0(4.0~10.0)×109/L,第1阶段(1~14 d)仅用达那唑治疗血小板最高值为33.0(23.0~72.0)×109/L.第2阶段(14~28 d)加用rhTPO,血小板计数最高值为111.5(60.0~152.0)×109/L,与用药前及第1阶段最高值相比均P<0.01.停药后血小板计数逐渐下降,至42 d降至33.0(22.5~46.0)×109/L.近期有效率84.2%,其中显效47.4%,良效36.8%.不良反应轻微,仅2例出现头痛,未发现有肝肾功能、凝血功能的影响.结论:rhTPO治疗ITP短期疗效显著,不良反应轻微.

  13. Nursing for patients with refractory idiopathic thrombocytopenic purpura by combination treatment of small dose of rituximab and recombinant human thrombopoietin%小剂量美罗华联合重组人血小板生成素治疗难治性特发性血小板减少性紫癜的护理

    Institute of Scientific and Technical Information of China (English)

    2016-01-01

    目的 探讨小剂量美罗华联合重组人血小板生成素(rhTPO)治疗难治性特发性血小板减少性紫癜(ITP)的疗效及其护理.方法 对诊断为难治性ITP的4例患者采用小剂量美罗华联合rhTPO治疗,治疗过程中实施有效的心理护理,严格规范药液的保管、配置及输注,积极预防和处理药物不良反应,并对副作用进行严密观察和护理.结果 4例患者中,1例获完全反应(CR),3例获部分反应(PR),发热1例、肝功能异常1例,所有患者均顺利完成治疗.结论 小剂量美罗华联合rhTPO为难治性ITP的治疗提供了新途径,是一种安全有效的治疗方法,精心的护理能有效预防药物的毒副作用,保证治疗效果.

  14. 紫茜胶囊对慢性原发性血小板减少性紫癜患者IL-2、sIL-2R的影响%The Effect on IL - 2 and sIL - 2R of chronic Idiopathic Thrombocytopenic Purpura treated by Zi Qian capsule

    Institute of Scientific and Technical Information of China (English)

    孙凤; 朱云丽

    2007-01-01

    目的:探讨紫茜胶囊对慢性原发性血小板减少性紫癜(CIIp)患者血清IL-2及sIL-2R的影响及作用机理.方法:将64例CITP患者分为紫茜胶囊治疗组和升血小板胶囊对照组各32例,用ELISA方法检测CITp患者治疗前后血清IL-2、sIL-2R的含量变化.结果:CITP患者的IL-2水平明显低于正常对照组,sIL-2R则高于正常对照组,二者经治疗后均有明显改善,但治疗组优于对照组.结论:血清IL-2及sIL-2R水平变化与CITP的发病有关,紫茜胶囊可通过降低IL-2、升高sIL-2R改善CITP患者的细胞免疫功能.

  15. Therapeutic effect of cyclosp-ionrin A on chronic idiopathic thrombocytopenic purpura with diabetes mellitus%环胞素A治疗慢性原发性血小板减少性紫癜合并糖尿病的疗效观察

    Institute of Scientific and Technical Information of China (English)

    耿惠

    2001-01-01

    目的探讨环胞素A(CsA)治疗合并糖尿病(DM)的慢性原发性血小板减少性紫癜(CIIP)的疗效.方法对19例合并DM的CITP给予CsA治疗,用长春新碱(VCR)静脉滴注22例作为对照治疗,比较两组治疗总有效率及T细胞亚群各指标.结果两组治疗总有效率差异有显著性,治疗组优于对照组(P<0.05).CsA组治疗前后T细胞亚群中CD8+及CD4+/CD8+变化显著(分别为P<0.05,P<0.01).而对照组T细胞亚群各指标变化差异无显著性.结论CsA治疗合并DM的CITP较传统治疗有疗效高等特点,可作为合并较严重DM的CTTP治疗有效手段之一.

  16. Kampo medicines improved blood test and QOL in two vasculitis cases of Churg–Strauss syndrome and Henoch–Shönlein purpura after inadequate treatment with conventional therapies

    Directory of Open Access Journals (Sweden)

    Yasuyo Hijikata

    2010-02-01

    Full Text Available Yasuyo Hijikata1, Yumiko Ikemoto2, Urara Kohdera31Toyodo Hijikata Clinic, Osaka, Japan; 2St Mary’s Hospital, Himeji, Japan; 3Nakano Children’s Hospital, Osaka, JapanObjective: Based on the tenets of traditional Chinese medicine (TCM theory, Kampo medicines were selected and applied to two cases of Churg–Strauss syndrome and Henoch–Shönlein purpura. Two vasculitis syndrome patients exhibited persistent symptoms and abnormal blood tests after treatment with conventional therapies.Methods: As the two cases had “blood stagnation” and “damps and heat” and one had a “yang deficiency” in terms of TCM theory, we applied certain selected Kampo medicines.Results: In case 1, the patient presented with hypereosinophilia, venous thrombosis, pulmonary infarction, decreased platelet count, ulner nerve palsy and Raynaud’s phenomena, which led to a diminished quality of life. After starting the Kampo medicines, the patient improved quickly and recovered within 11 months. In case 2, persistent purpura, abdominal pain, and bloody feces quickly improved and disappeared after Kampo treatment. After starting the Kampo medicines, prednisolone was stopped at 21 days without any sign of relapse to date.Conclusion: Kampo medicines helped clear the persistent abnormal symptoms and laboratory findings of vasculitis syndromes, Churg–Strauss syndrome and Henoch–Shönlein purpura, which had responded inadequately to the conventional therapies administered.Keywords: Kampo medicine, vasculitis, Churg–Strauss syndrome, Henoch–Shönlein purpura

  17. Investigations of the capacity of synthesizing 3β-sterols in mollusca—VI. The biosynthesis and composition of 3β-sterols in the neogastropods Purpura lapillus and Murex brandaris

    NARCIS (Netherlands)

    Voogt, P.A.

    1972-01-01

    1. 1. It is shown that Purpura lapillus and Murex brandaris are capable of synthesizing sterols from mevalonate and acetate. The biosynthesis of sterols from acetate proceeds only at a slow rate. This phenomenon is compared with that observed in Natica cataena. 2. 2. The composition of the sterol m

  18. EULAR/PRINTO/PRES criteria for Henoch-Schönlein purpura, childhood polyarteritis nodosa, childhood Wegener granulomatosis and childhood Takayasu arteritis: Ankara 2008. Part I: Overall methodology and clinical characterisation

    DEFF Research Database (Denmark)

    Ruperto, Nicolino; Ozen, Seza; Pistorio, Angela;

    2010-01-01

    To report methodology and overall clinical, laboratory and radiographic characteristics for Henoch-Schönlein purpura (HSP), childhood polyarteritis nodosa (c-PAN), c-Wegener granulomatosis (c-WG) and c-Takayasu arteritis (c-TA) classification criteria....

  19. A case of neonatal alloimmune thrombocytopenia in the presence of both anti-HPA-4b and anti-HPA-5b antibody: clinical and serological analysis of the subsequent pregnancy.

    Science.gov (United States)

    Kiyokawa, Tomoko; Koh, Yangsook; Mimura, Kazuya; Nakayama, Kotarosumitomo; Hosokawa, Mika; Sakuragi, Mikiko; Morikawa, Tamayo; Nakao, Mayumi; Aochi, Hiroshi; Fukumori, Yasuo; Kanagawa, Takeshi; Nagamine, Keisuke; Kimura, Tadashi; Tomiyama, Yoshiaki

    2014-10-01

    Neonatal alloimmune thrombocytopenia (NAIT) is induced by maternal alloantibodies raised against fetal platelet antigens inherited from the paternal parent. In contrast to Caucasians, in Asians, predominantly in Japanese, most frequently detected antibodies in NAIT are anti-HPA-4b and anti-HPA-5b. In some NAIT cases multiple alloantibodies are detected. In such cases it is very difficult to determine which antibody is the dominant antibody in NAIT. In this case report, we describe a NAIT case (first sibling) with severe thrombocytopenia and cephalhematoma in the presence of both anti-HPA-4b and anti-HPA-5b antibodies in the maternal serum. We carefully examined titers of anti-HPA antibodies during the subsequent pregnancy with HPA-4b-positive and HPA-5b-negative fetus determined by amniocentesis at gestational week 16. We administered IVIG (1 g/kg/w) to the mother from gestational week 32 to 35. The mother subsequently delivered a second sibling with normal platelet count by cesarean section. Although we could not completely rule out the involvement of anti-HPA-4b, our findings suggested that anti-HPA-5b was implicated in the NAIT in the first sibling.

  20. 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.

  1. 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

  2. Human Immunoglobulin Combined Immune Regulation Effect of Platelet Transfusion for Idiopathic Thrombocytopenia Purpura%人免疫球蛋白联合血小板输注用于特发性血小板减少性紫癜的免疫调节作用

    Institute of Scientific and Technical Information of China (English)

    王明慧; 杨涛; 方春燕; 梁萍

    2015-01-01

    Objective To investigate the immune regulation effect of Platelet transfusion for idioPathic thrombocytoPenia PurPura. Methods 40 cases of children from January 2010 to October 2013 were selected and randomly divided into the control grouP and the observation grouP, 20 cases in each grouP. The two grouPs were given conventional treatment;on the basis of conventional theraPy, the observation grouP were given the Platelet transfusion 1 dose of theraPy with intravenous gamma globulin 0. 4 g/kg, and on the next day and the 3rd day received intravenous immunoglobulin 0. 4 g/ ( kg·d ) . Results After 1 course of treatment, the obvious effective rate of the ob-servation grouP was 65. 00%, and the total effective rate was 100. 00%, which were significantly higher than 25. 00% and 65. 00% of the control grouP ( P < 0. 05 );after 24 h of treatment, the Platelet count ( × 109/L ) of the observation grouP was better than that of the control grouP ( P < 0. 05 );CCI 24 ≥ 4. 5 × 109/L of the observation grouP was better than that of the control grouP ( P < 0. 05 );comPared with the control grouP, the time of hemostasis, Platelet count reaching normal, Platelet count reaching the Peak, and the Peak value of the observation grouP showed statistical differences ( P < 0. 05 );after treatment, the CD3 +, CD4 +, CD8 +, CD4 + /CD8 + of the ob-servation grouP were better than those of the control grouP ( P < 0. 05 ); the Platelet associated antibody ( PAIgG ) of the two grouPs after treatment had statistical difference comPared with before treatment ( P < 0. 05 );after treatment, the IL-4 of the observation grouP was ( 61. 19 ± 10. 42 ) ng/mL, which was lower than ( 72. 12 ± 11. 39 ) ng/mL of the control grouP ( t=4. 068, P < 0. 05 ) . Conclusion The Human Immunoglobulin combined Platelet transfusion can regulate the body's immune function of the idioPathic thrombocytoPenic PurPura Patients, and imProve the Prognosis of the Patients.%目的:观察人免疫球蛋白联合血小

  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. Henoch-Sch(o)nlein purpura combined with antiphospholipid syndrome: one case report and literature review%过敏性紫癜合并抗磷脂综合征1例报告并文献复习

    Institute of Scientific and Technical Information of China (English)

    张宏文; 钟旭辉; 黄建萍; 肖慧捷

    2012-01-01

    目的 探讨过敏性紫癜与抗磷脂综合征的关系.方法 通过对1例以间断皮疹、血尿、蛋白尿及上肢肿痛为主诉的患儿进行肾组织病理、抗磷脂抗体检查,随访观察治疗反应,并结合文献复习进行综合分析.结果 患儿临床表现为过敏性紫癜混合型,肾脏病理为紫癜性肾炎(Ⅳb型);同时有右上肢静脉血栓形成,狼疮抗凝血因子、抗心磷脂抗体IgM及抗β2糖蛋白-I抗体IgM阳性,诊断为过敏性紫癜合并抗磷脂综合征.给予泼尼松口服、环磷酰胺冲击以及抗凝治疗,患儿临床症状好转,尿蛋白和抗磷脂抗体转阴.结论 过敏性紫癜可以合并抗磷脂综合征,临床上应引起高度重视.%Objective To explore the relationship of Henoch-Sch(o)nlein purpura (HSP) and antiphospholipid syndrome (APS). Methods One patient presenting with purpura, hematuria, proteinuria and right extremity pain was followed up. The information about kidney tissue pathology, antiphospholipid antibody test, and response to treatment was collected and analyzed. The literatures were reviewed. Results The patient clinically manifested Henoch-Sch(o)nlein purpura. The pathologic diagnosis was Henoch-Sch(o)nlein purpura nephritis (type Ivb). The vein thrombosis was formed in right upper extremity veins. The anticoagulant, anticardiolipin antibodies and anti β2 glycoprotein I antibodies were positive. The diagnosis was Henoch-Sch(o)nlein purpura combined with antiphospholipid syndrome. After given oral pred-nisone, cyclophosphamide, and anticoagulant therapy, clinical symptoms of the patient were improved. The urine protein and antiphospholipid antibody were negative. Conclusions Henoch-Sch(o)nlein purpura could be combined with antiphospholipid syndrome. More attention should be paid to it in clinic.

  5. Inhibition of HPA-1a alloantibody-mediated platelet destruction by a deglycosylated anti-HPA-1a monoclonal antibody in mice: toward targeted treatment of fetal-alloimmune thrombocytopenia.

    Science.gov (United States)

    Bakchoul, Tamam; Greinacher, Andreas; Sachs, Ulrich J; Krautwurst, Annika; Renz, Harald; Harb, Habi; Bein, Gregor; Newman, Peter J; Santoso, Sentot

    2013-07-18

    Fetal/neonatal alloimmune thrombocytopenia (FNAIT) is often caused by maternal alloantibodies against the human platelet antigen (HPA)-1a, which opsonizes fetal platelets (PLTs). Subsequent PLT destruction is mediated via the Fc part of the alloantibodies. The monoclonal antibody (mAb) SZ21 binds to the HPA-1a epitope and inhibits the binding of maternal alloantibodies. However, it also promotes complement activation and phagocytosis. Deglycosylation of antibodies abrogates the Fc-related effector functions. We modified the N-glycan of SZ21 by endoglycosidase F. The in vivo transplacental transport of N-glycan-modified (NGM)-SZ21 was not impaired. When injected into pregnant mice, both native-SZ21 and NGM-SZ21 were transported equally into fetal circulation (8.9% vs 8.7%, respectively, P = .58). Neither the binding properties of NGM-SZ21 to HPA-1a in surface plasmon resonance, nor the inhibition of anti-HPA-1a-induced PLT phagocytosis, were affected by N-glycan modification. NGM-SZ21 prevented PLT destruction induced by maternal anti-HPA-1a antibodies in vivo in a mouse model (PLT clearance after 5 hours; 18% vs 62%, in the presence or absence of NGM-SZ21, respectively, P = .013). Deglycosylation of SZ21 abrogates Fc-effector functions without interfering with placental transport or the ability to block anti-HPA-1a binding. Humanized, deglycosylated anti-HPA-1a mAbs may represent a novel treatment strategy to prevent anti-HPA-1a-mediated PLT destruction in FNAIT.

  6. Clinical Effect of Cyclophosphamide and Prednisone inTreating Henoch Schonlein Purpura Nephritis%环磷酰胺联合泼尼松治疗紫癜性肾炎的临床疗效

    Institute of Scientific and Technical Information of China (English)

    刘婷婷; 王建

    2014-01-01

    In 23 cases of Henoch Schonlein purpura nephritis patients, 12 cases were given CTX combined with prednisone in shock treatment, 11 patients were treated with oral prednisone therapy,Comparison of CTX combined with prednisone in the treatment of Henoch Schonlein purpura nephritis has better clinical efficacy.%针对23例紫癜性肾炎患者,12例给予CTX联合泼尼松冲击治疗,11例给予口服泼尼松维持治疗,对比发现CTX联合泼尼松治疗紫癜性肾炎有着较好的临床疗效。

  7. Physiopathology of blood platelets and development of platelets substitutes. Progress report, August 1, 1976--October 31, 1977. [/sup 51/Cr

    Energy Technology Data Exchange (ETDEWEB)

    Baldini, M G

    1977-07-31

    Progress is reported on the following research projects: the effect of estrogen on platelet aggregability and thrombus formation; the antithrombotic effect of platelet inhibiting agents in a bench model of artificial kidney; the arrest of hemorrhage in severely alloimmunized thrombocytopenic patients; and in vivo elution of /sup 51/Cr from labeled platelets induced by antibody. (HLW)

  8. Progress in treatment of pediatric Henoch-Schönlein purpura nephritis%儿童紫癜性肾炎治疗进展

    Institute of Scientific and Technical Information of China (English)

    叶乐珍; 周江瑾(综述)

    2014-01-01

    Henoch-Schönlein purpura nephritis (HSPN) is the most common secondary glomerular disease in children. The clinical manifestations of HSPN vary from microscopic hematuria, microalbuminuria to renal dysfunction even end stage renal disease which is needed to rely on long-term renal replacement therapy, thus affecting the quality of children life seriously. In recent years, the incidence of Henoch-Schönlein purpura continues to increase, which should be paid more attention. There are many studies about the treatment of HSPN both at home and abroad, yet no certain conclusion is drawn because of the inconsistent results. We advocate stepped therapies, that is, the appropriate regimens are chosen based on the clinical manifestations and renal pathology in children. For severe HSPN, we recommend multi-drug intensive therapy combined with other methods such as plasma exchange to alleviate the symptoms.%紫癜性肾炎是最常见的儿童继发性肾小球疾病。其临床表现不一,轻者仅表现为镜下血尿、微量蛋白尿,重者可逐渐进展为肾功能不全,直至终末期肾脏病而需要依靠长期肾脏替代治疗,严重影响患儿生存质量。近年来过敏性紫癜发生率不断提高,需予以重视。关于紫癜性肾炎的治疗,目前国内外有较多的研究,但结果不尽一致,因此至今未有定论。我们提倡阶梯式疗法,即根据患儿临床表现、肾脏病理选择合适的方案,而对于重症紫癜性肾炎,我们推荐多药强化治疗并联合血浆置换等方法以缓解病情。

  9. 幽门螺杆菌感染与过敏性紫癜相关性研究%Study the allergic purpura association witll Helicobacter pylori infection

    Institute of Scientific and Technical Information of China (English)

    郭猛; 徐春华

    2010-01-01

    Objective To study the relationship between allergic purpura and Helicobacter pylori infection and direct therapy.Methods From December 2007 to December 2009 120 cases at the undergraduate treated of HSP patients had HP-IgG antibody been tested,and serum Hp-IgG-positive patients were randomly divided into two groups,A group on the basis of conventional therapy to give anti-Hp therapy,B group only conventional treatment,cured patients were followed up for 6 months.Results 120 case with The percentage of HpIgG-positive is 67.5% ,Hp-IgG-positive HSP patients given conventional therapy on the basis of anti-Hp therapy,there are high efficiency and low recurrence rate.Conclusion Helicobacter pylori infection in the pathogenesis of allergic purpura is one important factor,anti-HP therapy is conducive to the rehabilitation of HSP.%目的 研究幽门螺杆感染与过敏性紫癜的相关性并指导治疗.方法 对2007年12月至2009年12月在本科治疗的120例HSP患者进行了HP-IgG抗体检查、并将血Hp-IgG阳性的患者随机分为2组,A组在常规治疗的基础上给与抗Hp治疗,B组仅用常规治疗,对治愈患者随访6个月.结果 120例过敏性紫癜患者血Hp-IgG阳性81例(67.5%),Hp-IgG阳性的HSP患者在常规治疗的基础上给与抗Hp治疗,有效率高,且复发率低.结论 幽门螺杆菌感染是过敏性紫癜发病的重要因素之一,抗Hp治疗有利于HSP的康复.

  10. Elevated levels of antibodies against phosphatidylserine/prothrombin complex and/or cardiolipin associated with infection and recurrent purpura in a child: a forme fruste of antiphospholipid syndrome?

    Science.gov (United States)

    Kinoshita, Yuri; Mayumi, Nobuko; Inaba, Motoyuki; Igarashi, Touru; Katagiri, Ichigen; Kawana, Seiji

    2015-07-15

    Antiphospholipid syndrome is an autoimmune disorder characterized by the occurrence of venous and arterial thrombosis, as well as morbidity in pregnancy, in the presence of anti-phospholipid antibodies. The diagnosis of antiphospholipid syndrome is usually established based on clinical and laboratory findings by strictly following the 2006 Sapporo classification. However, the diagnosis remains challenging owing to the ongoing debates on the serological criteria. We report a case we describe as forme fruste antiphospholipid syndrome in which these criteria were not fulfilled. Purpura appeared repeatedly in a female infant starting from the age of 6 months and following episodes of upper respiratory infections and vaccinations. The levels of anti-cardiolipin IgG antibodies and anti-phosphatidylserine/prothrombin complex antibodies were elevated in accordance with these events. Histopathological evaluation revealed multiple small vessel thrombi in the dermis and adipose tissue. After 2 weeks of treatment with aspirin and heparin, the cutaneous symptoms subsided. Infection has long been associated with antiphospholipid syndrome, and anti-phosphatidylserine/prothrombin antibodies are considered a new marker for the diagnosis of antiphospholipid syndrome. Forme fruste antiphospholipid syndrome should be considered even if the antiphospholipid syndrome diagnostic criteria are not completely fulfilled, especially in the presence of elevated levels of anti-phosphatidylserine/prothrombin antibodies and known preceding infections.

  11. Early Treatment with Methylprednisolone Pulse Therapy Combined with Tonsillectomy for Heavy Proteinuric Henoch-Schönlein Purpura Nephritis in Children

    Directory of Open Access Journals (Sweden)

    Hiroaki Kanai

    2011-10-01

    Full Text Available Background: There is no clear consensus as to which patients with Henoch-Schönlein purpura nephritis (HSPN at risk of a poor outcome should be treated and what therapeutic regimen should be used. Methods: Nine children with heavy proteinuric HSPN received prompt initiation of methylprednisolone pulse therapy (MPT combined with tonsillectomy in a prospective study. Results: At presentation, the mean values for the patients’ urine protein excretion (early-morning urinary protein/creatinine ratio, serum IgA, activity index (AI, and chronicity index (CI were 5.0 ± 5.6 g/g Cr, 135.6 ± 56.5 mg/dl, 4.0 ± 0.7, and 1.7 ± 1.3, respectively. At the second biopsy, conducted approximately 24 months after initiation of therapy, the patients’ serum albumin had significantly increased (4.4 ± 0.2, p Conclusions: Early treatment with MPT combined with tonsillectomy is effective in ameliorating the histopathological progression and improving the clinical course of children with heavy proteinuric HSPN.

  12. Evaluation of the diagnostic performance of platelet-derived indices for the differential diagnosis of thrombocytopenia in pediatrics

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    Nelson Hernando Aponte-Barrios

    2014-10-01

    Full Text Available Background. Platelet-derived indices have a well-established correlation with the differential diagnosis of thrombocytopenia in adult-based research. These indices include mean platelet volume, platelet distribution width, and platelet-large cell ratio. Objective. To determine the values of platelet-derived indices in a pediatric population with diagnoses of thrombocytopenia and their etiologic correlation. Materials and methods. Analytic observational diagnostic-test study. The population for this analytical study was pediatric patients between 6 months and 18 years of age who had thrombocytopenia (<100x10(9/L. The study period was 18 months long. Results. Of 54 subjects, 18 (33.3% were diagnosed with idiopathic thrombocytopenic purpura, and 36 (66.7% were diagnosed with acute leukemia. Mean age was 7.4 years and 6.8 years for immune thrombocytopenic purpura and acute leukemia, respectively. Mean platelet distribution width values for immune thrombocytopenic purpura and acute leukemia were 15.08 fL and 10.73, respectively. Mean MPV for immune thrombocytopenic purpura and acute leukemia was 11.7 fL and 9.8 fL, respectively. Mean platelet-large cell ratio was 38.26% and 24.97% for idiopathic thrombocytopenic purpura and acute leukemia, respectively. Differences in these three distinct platelet indices between idiopathic thrombocytopenic purpura and acute leukemia were statistically significant (p=0.00. The area under the ROC curve for platelet-derived indices showed that they were adequate for defining the causes of thrombocytopenia. MPV and platelet-large cell ratio had an area under the curve of 0.89 and 0.88, respectively, while platelet size deviation width had an area under the curve of 0.903. Conclusions. Platelet-derived indices could be useful in the initial approach for the differential diagnosis of pediatric patients with thrombocytopenia.

  13. Red blood cell alloimmunization after blood transfusion

    NARCIS (Netherlands)

    Schonewille, Henk

    2008-01-01

    Current pretransfusion policy requires the patients’ serum to be tested for the presence of irregular red blood cell antibodies. In case of an antibody, red blood cells lacking the corresponding antigen are transfused after an antiglobulin crossmatch. The aim of the studies in this thesis is primari

  14. Clinical Therapentic Efficacy of Rituximab for Relapsed and Refractory Idiopathic Thromocytopenic Purpura%利妥昔单克隆抗体治疗难治复发性特发性血小板减少性紫癜临床疗效初步评估

    Institute of Scientific and Technical Information of China (English)

    周立宇; 张征; 宋陆茜; 张曦; 苏基滢; 李晓; 常春康

    2012-01-01

    The aim of this study was to evaluate the effect of rituximab treatments for refractory and relapsed idiopathic thrombocytopenic purpura (ITP). 18 patients with refractory and relapsed ITP who received 22 courses of rituximab treatments from January 2007 to December 2010 were analyzed retrospectively. Rituximab was given at a dose of 375 mg/m2 intravenously weekly for a continuous 4 weeks. The results indicated that responses were achieved in 15 of 22 (68% ) courses, out of which complete responses were achieved in 10 of 22 (45% ) courses, partial and minimal responses were achieved in 5 of 22 (23% ) courses, and no responses were achieved in 7 of 22 (32% ) courses. The median time of response was 3 weeks (1-17 weeks) from the start of treatment and median duration of response was 13 weeks (1 week -42 months). The responses were mostly short -sustained and follow-up median time was 20 months (1-52 months). The responses of 8 patients (36% ) sustained for over 6 months, 6 patients (27% ) sustained for over 1 year, and 4 patients also showed sustained response at last visit of follow-up. Previous splenectomy resulted in a poor response (P = 0. 037). Patients who failed in rituximab treatment and prior received multiple treatments including splenectomy, had a poor response to further therapies. It is concluded that rituximab is well tolerated by patients and is useful in some patients with relapsed and refractory ITP, however, only about 20% patients can achieve sustained remissions. The patients who failed in rituximab treatment has a poor response to further treatment.%本研究评价利妥昔单克隆抗体治疗难治复发性特发性血小板减少性紫癜(ITP)的临床疗效.选择我院2007年1月-2010年12月期间确诊为难治、复发性ITP患者18例,给予利妥昔单克隆抗体375mg/m2静脉滴注,每周1次,连用4周为1个疗程.结果表明,18例患者接受22个疗程的利妥昔单克隆抗体治疗,治疗有效率为68%,完全反应率为45

  15. Childhood Henoch-Sch(o)nlein Purpura Nephritis and IgA Nephropathy:One Disease Entity?--A Clinico-pathologically Comparative Study

    Institute of Scientific and Technical Information of China (English)

    2005-01-01

    In order to characterize their relationship through clinicopathological comparison between IgA nephropathy and Henoch-Schonlein purpura nephritis (HSPN), 31 children with IgA nephropathy aged between 3 to 15 years and 120 children with HSPN aged between 4 to 15 years were compared with each other in clinical manifestation, blood biochemistry, serum immunology and followup study. Renal pathological findings under light microscope, immunofluorescence and electronic microscope were analyzed and also compared between 31 children with IgA nephropathy and 32 biopsied children with HSPN. The results showed that the onset age was over 12 years in 25.8 %children with IgA nephropathy, but only 10 % in HSPN (P<0.05). The clinical patterns of IgA nephropathy and HSPN were similar, but extra-renal manifestations were more often in HSPN, all of them had skin purpura, 59 % had gastrointestinal symptoms and 47 % suffered from arthralgia,compared with only abdominal pain in 3.2 % children with IgA nephropathy. The renal pathological investigation showed global sclerosis in 35.5 % of IgA nephropathy and 3.1% of HSPN, mesangial sclerosis in 41.9 % of IgA nephropathy and 6.3 % of HSPN, but endothelial proliferation in 65.6% of HSPN and 29 % of IgA nephropathy (all P<0.01). Thin basement membrane nephropathy was only found in 6.5 % children with IgA nephropathy, no in HSPN. The electronic dense deposits in HSPN were sparse, loose and wildly spread in glomerular mesangium, subendothelial area and even intra basement membrane, but it was dense, lumpy and mostly limited in mesangium and paramesangium in IgA nephropathy. Predominant IgA deposits were found in 81.2 %of HSPN, and overwhelming IgG deposits in 12.5 % of HSPN with relatively weak IgA deposits,moreover 6.3 % of HSPN showed linear IgG deposits in glomerular capillary. Totally 71.9 % of HSPN had IgG deposits in glomeruli and only 19.4 % of IgA nephropathy showed glomerular IgG deposits (P<0.01). No IgG deposit was observed

  16. Effect of sanqizhixue capsule on allergic purpura%三七止血胶囊治疗过敏性紫癜临床疗效观察

    Institute of Scientific and Technical Information of China (English)

    龚启英; 黄朝; 黄建国

    2015-01-01

    Objective to study the effect of sanqizhixue capsuLe on aLLergic purpura. Methods aLL patients were randomLy divided into two groups. Group a( controL group)had 48 cases,and the patients were treated with oraL cetirizine hydrochLoride syrup,Loratadine dispersibLe tabLets. MethyLprednisoLone tabLets,compound ranitidine capsuLes were given for medium cases. Dexamethasoni injection and compound ranitidine capsuLes were given for severe cases or very severe cases,anisodamine injection was given for cases acccompanied with abdomen ache. Group b( treatment group)had 48 cases,the patients were treated with o-raL sanqizhixue capsuLe,cetirizine hydrochLoride syrup,Loratadine dispersibLe tabLets. MethyLprednisoLone tabLets,compound ran-itidine capsuLes were given for medium cases;Dexamethasoni injection and compound ranitidine capsuLes were given for severe cases or very severe cases,anisodamine injection was given for cases acccompanied with abdomen ache. Results the cure rate and effective rate of group a were 54. 17% and 95. 83% respectiveLy. the resuLts showed that the cure rate and effective rate of group b were 79. 17% and 97. 92% respectiveLy. the cure rate of group a was significant higher than that of group b(χ2 =6. 75,p<0. 01). Conclusion Sanqizhixue capsuLe is safe and effective for aLLergic purpura,so it is worthy to be popuLarized in cLinics.%目的:探讨三七止血胶囊治疗过敏性紫癜的疗效。方法将全部患者随机分成两组,a组(对照组)48例,给予西替利嗪糖浆,氯雷他啶分散片;中型患者加用甲泼尼龙片、复方雷尼替丁胶囊;重型、严重型则加用地塞米松针,复方雷尼替丁胶囊,腹痛者注射山莨菪碱针。b组(治疗组)48例,采用口服三七止血胶囊,西替利嗪糖浆,氯雷他啶分散片;中度患者加用甲泼尼龙片、复方雷尼替丁胶囊;重型、严重型则加用地塞米松针、复方雷尼替丁胶囊,腹痛者

  17. Association between RAS Gene Polymorphisms (ACE I/D, AGT M235T and Henoch-Schönlein Purpura in a Turkish Population

    Directory of Open Access Journals (Sweden)

    Sinem Nalbantoglu

    2013-01-01

    Full Text Available Henoch-Schönlein purpura (HSP is a small-vessel vasculitis of autoimmune hypersensitivity, and renin-angiotensin system (RAS regulates vascular homeostasis and inflammation with activation of cytokine release. Thus, we aimed to investigate the association between HSP and ACE I/D and AGT M235T polymorphisms. Genotyping was determined by allele specific PCR and PCR-RFLP. We obtained a significant difference in genotype distribution (p = 0.003 and allele frequencies (p 0.05 and allele frequencies (p > 0.05 of the AGT M235T polymorphism. Risk assessment showed significant risk for HSP in the subjects both with the ID + DD genotype (p = 0.019, OR: 2.288, 95% CI: 1.136–4.609 and D allele (OR: D vs. I: 2.0528, 95% CI: 1.3632–3.0912, p = 0.001 while no significant risk was obtained for HSP in the subjects both with the MT + TT genotype (p = 0.312, OR: 1.3905, 95% CI: 0.7326–2.6391 and T allele (OR: T vs. M: 1.065, 95% CI: 0.729–1.557, p = 0.743. Furthermore, when patients were stratified by the presence of certain systemic complications of HSP, no significant association was detected with ACE I/D, and AGT M235T polymorphisms. Our findings suggest that ACE I/D polymorphism is significantly associated with HSP susceptibility.

  18. Acupoint implantable drug-line treatment for allergic purpura%穴位埋植药线治疗过敏性紫癜效果观察

    Institute of Scientific and Technical Information of China (English)

    郑丽丽; 马卫东; 施文洁; 马萍萍

    2014-01-01

    目的:观察穴位埋植药线治疗过敏性紫癜的临床疗效。方法:将过敏性紫癜患儿200例随机分为观察组和对照组各100例。对照组采用传统治疗方法即对症治疗+肾上腺糖皮质激素应用,观察组采用双侧足三穴位埋植药线的方法。即患儿取坐位或仰卧位,在常规消毒和局麻下,用无菌手术缝合针引线,一侧足三里穴埋植卡介菌多糖浸泡的肠线,另一侧埋植核酸注射液浸泡肠线。双侧穴位埋植药线的深度均要达肌层,然后将露出表皮的线头用手术剪剪除,在用无菌敷料包扎固定。7d治疗1次,2次为1个疗程,连续治疗3个疗程。上述两组患者均治疗40d后评定整体疗效及典型症状消失的时间。数据进行统计学处理(x2检验,t检验)。结果:两组患者整体疗效比较,提示观察组优于对照组(P<0.01),具有非常显著性差异。其典型症状如皮肤紫癜、消化道症状、关节症状、肾损害症状比较,也具有非常显著性差异(P<0.01),观察组所用的时间均较对照组短。结论:采用足三里穴位埋植药线治疗过敏性紫癜效果好。%Objective:To observe the clinical efficacy of the the acupoint implants the drug line for allergic purpura. Methods:200 children were randomly divided into observation group and control group of 100 patients, control group took traditional treatment methods that symptomatic treatment +glucocorticoid. The observation group was treated with bilateral foot three acupuncture points implantable drug line. Children take sitting or supine position, routine disinfection and under local anesthesia, sterile surgical suture needle leads the side of the foot the the Zusanli implants card BCG polysaccharide soaked catgut, the other side of the implant nucleic acid injection soak catgut. The bilateral acupoint implantable drug line depth of myometrial, then the exposed epidermis thread cut off with

  19. Rituximab in the treatment of patients with systemic Iupus erythematosus-associated thrombotic thrombocytopenic purpura with normaI ADAMTS-13 activity:three cases and Iiterature review%利妥昔单抗治疗ADAMTS-13活性正常系统性红斑狼疮相关血栓性血小板减少性紫癜三例并文献复习

    Institute of Scientific and Technical Information of China (English)

    李嗣钊; 卢昕; 周丽静; 陈亚宁; 尚健; 姚合斌

    2016-01-01

    biopsy which revealed typeⅡ lupus nephritis (LN) and typeⅣ andⅤ LN with renal thrombotic microangiopathy respectively. All 3 patients received methylprednisolone pulse and intravenous immunoglobulin or in combination with mycophenolate mofetil therapy before SLE-associated TTP was diagnosed, however they got worse. As the diagnosis was considered, 2 patients received plasma exchange (PE) combined with glucocorticoids and/or mycophenolate mofetil (MMF), but there were still no improvement. Then 3 patients were rescued by RTX therapy for 2 to 4 episode, after that they achieved complete remission. With mean follow-up of 14 months they showed a sustained remission. 1 patient died from severe pneumonia at 6 months of follow-up. From literature review, a total of 6 cases including our cas suffered from SLE-associated TTP with normal ADAMTS-13 activity, some of them were treated by glucocorticoids combined with PE and/or immunosuppressive failure, then switched to RTX therapy, some of them received RTX in combination with glucocorticoids and PE as an initial treatment, they all got complete remission.ConcIusion SLE-associated TTP should be considered when SLE complicated with fever, anemia accompanied with thrombocytopenia and high LDH level. Increased peripheral broken red cells count and renal biopsy are necessary for the diagnosis of this disease. Patients with SLE-associated TTP with normal ADAMTS-13 activity usually perform high disease activity, severe nervous system and kidney damage. RTX combined with glucocorticoids and/or PE and immunosuppressive may significantly improve effectiveness of treatment and the prognosis of these patients.%目的:探讨利妥昔单抗(RTX)治疗血管性血友病因子裂解蛋白酶(ADAMTS-13)活性正常的系统性红斑狼疮(SLE)相关血栓性血小板减少性紫癜(TTP)的疗效。方法分析中国人民解放军海军总医院内分泌风湿免疫科先后应用 RTX 治疗3例ADAMTS-13活性正常的SLE相关 TTP 患者的临床特征,随访患者治疗后的转归,并总结相关文献。结果3例 SLE 相关 TTP患者均有不同程度的发热、血小板减少和微血管病性溶血性贫血,伴神经系统和肾损害,血乳酸脱氢酶(LDH)明显升高,外周血涂片可见破碎红细胞,而 ADAMTS-13活性正常。2例患者行肾活检显示1例为狼疮肾炎Ⅳ+Ⅴ型伴血栓性微血管病,1例为狼疮肾炎Ⅱ型。3例患者在确诊SLE相关TTP前均接受甲泼尼龙冲击联合静脉注射免疫球蛋白和吗替麦考酚酯(MMF)治疗,但病情加重,确诊后其中2例患者联合血浆置换治疗病情仍无改善,给予RTX治疗;1例患者在确诊后直接联合RTX治疗。经上述治疗后3例患者病情均达到完全缓解,平均随访14个月呈持续缓解,其中1例在随访6个月时因重症肺炎死亡。回顾文献结合本文系列报道,共6例应用RTX治疗ADAMTS-13活性正常的SLE相关TTP,显示所有患者均在糖皮质激素联合血浆置换和(或)联合免疫抑制剂治疗失败后加用RTX,或在初始治疗的同时即加用RTX,病情得到缓解。结论 SLE患者出现发热、贫血、血小板减少及LDH升高可能提示SLE相关TTP,而外周血涂片和肾活检检查有助于确诊。ADAMTS-13活性正常的SLE相关TTP患者神经系统和肾损害发生率高,病情重,对血浆置换联合糖皮质激素和(或)免疫抑制剂的治疗反应差,而RTX对这类患者有效,可改善患者预后。

  20. Clinical Effects of Kangfuxin Liquid in the Treatment of Children With Abdominal Type Allergic Purpura%康复新液辅治儿童腹型过敏性紫癜疗效观察

    Institute of Scientific and Technical Information of China (English)

    李辉; 杜海霞

    2015-01-01

    ObjectiveTo observe the clinical effects of Kangfuxin Liquid in treating children with Abdominal Type Allergic Purpura.Methods 60 patients were randomly divided into treatment group ( 30 cases), and control group ( 30 cases). Grouping the same clinical manifesta tions , there were two lower limbs purpura, abdominal pain, vomiting a few coffee sample and mild haematochezia performance. The two groups were given on the same treatment measures, the treatment group was given oral and enema of Kangfuxin in addition, the efifcacy of the two groups were compared.Results The abolominal pain felief time, hematemesis disappear time and fetal occult blood clearance time were shortened in treatment group compared with the controlgroup (P<0.05).Conclusion Oral and enema of Kangfuxin Liquid in children with Abdominal Type Allergic Purpura have a good role of adjuvant therapy.%目的:观察康复新液辅助治疗儿童腹型过敏性紫癜的疗效。方法将60例腹型过敏性紫癜患儿随机分为联合治疗组及对照组,各30例。分组时临床表现相同,都有双下肢紫癜、腹痛、呕少量咖啡样物及轻度便血等表现,具有可比性。在常规治疗基础上,联合治疗组予康复新液口服加灌肠,比较2组疗效。结果联合治疗组腹痛、呕血、大便隐血消失时间均短于对照组(P<0.05)。结论康复新液口服加灌肠对儿童腹型过敏性紫癜有很好的辅助治疗作用。

  1. Detection of Allergens in Children with Henoch-schonlein Purpura%过敏性紫癜患儿过敏原检测分析

    Institute of Scientific and Technical Information of China (English)

    刘静; 陆彪

    2013-01-01

    Objective To detect and analyze allergens with the common seasonal food and inhalant allergens in children with henoch - schonlein purpura ( HSP) . Methods 849 children with HSP admitted between November 2005 and November 2010 were recruited in this research. The biological resonance of the common food and inhalant allergens of 849 children were characterized using 2000 German BICOM allergy treatment system. Results ① The top five allergens based on positive rate of allergens for both foods and inhalants in children with HSP were dust mites, pod, cocoa, flowers and artemisia. ②The significant difference in food and inhalant allergens was found for both male and female ( P < 0. 05 ) . However, the female had more positive for food allergens (P =0.04)while the male had more positive for the inhalant allergens(P =0. 168). ③Food allergens had a significant higher positive rate(P <0.05) in aged 1-6 than that in aged 7-15. ④The food allergens had significantly higher positive rate in Hui ethnicity than that in Han ethnicity (P <0. 01 ). ⑤ The food and inhaled allergens shoewed the significantly seasonal difference (P <0. 01 ). The food allergens had the highest positive rate in winter while the inhalant allergens had the highest positive rate in summer. Conclusion In Ningxia, dust mites, pod, cocoa, flowers and Artemisia are the most significant allergens in children who have Henoch - Schonlein purpura. Gender, age, ethnicity, seasonal have impact on the distribution of food, and inhalant allergens.%目的 探讨宁夏地区过敏性紫癜患儿常见食物和吸入性过敏原的检出与分布特征,为过敏性紫癜的预防和治疗提供参考.方法 选取2005年11月-2010年11月我院儿科收治的过敏性紫癜患儿849例,应用生物共振治疗仪进行食物和吸入性过敏原检测.结果 ①过敏性紫癜患儿过敏原阳性率由高到低依次为粉尘螨、豆荚、可可饮料、花草混合物、艾蒿蒿属等.②男女性

  2. 过敏性紫癜患儿食物不耐受检测及其意义%Determination of serum specific IgG of intolerance food measurement in anaphylactoid purpura

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    彭丽; 常桂芬; 宋丽君; 王朝霞

    2011-01-01

    目的 探讨过敏性紫癜患儿食物不耐受血清特异性IgG水平对其病因诊断及饮食治疗的意义.方法 用食物不耐受IgG体外检测试剂盒检测30例过敏性紫癜患儿和20例健康对照儿童的血清特异性IgG水平.结果 实验组30例过敏性紫癜患者的血清总IgG阳性率为76.67%,其中对1种食物不耐受为3例,对2种以上食物不耐受者为20例.健康对照组20例血清总IgG阳性率为25%,对1种食物不耐受为2例,对2种以上食物不耐受者为3例,常见的引起不耐受的食物依次为牛奶、虾、鸡蛋清和鸡蛋黄、蟹、大豆、鳕鱼、大米、小麦、猪肉、牛肉等,实验组饮食上经避免不耐受食物及抗过敏治疗疗程缩短,疗效优于对照组.结论 血清特异性IgG水平检测对过敏性紫癜的病因诊断有参考意义,对患儿饮食治疗及营养状态的改善具有重要的指导意义.%Objective To analyse the significance of diagnosing the pathogenies for the serum specific IgG level and the food therapy in the anaphylactoid purpura patients .Methods Using the IgG food intolerance external assay ,the specific IgG level was detected in the serum of 30 cases of anaphylactoid purpura .Results Total positive rate of serum IgG in the ingestive group was 76.67% , in which 3 cases were sensitive to 1 kind of ingestants and 20 cases over 2 kinds of ingestants .In the control group ,total positive rate of serum IgG was 25% ,in which 2 cases were sensitive to 1 kind of ingestants and 3 cases over 2 kinds of ingestants .The common food inducing intolerance were milk ,shrimps ,egg white and yolk ,cab, soybeans ,fish ,rice ,wheat ,pork ,beef in tum .Avoiding the intolerance food and therapy with anti-allergen drugs can lessen the cause of the disease ,the effect was better than the control group .Conclusion :The serum specific IgG level has important reference significance for diagnosing the pathogenies in the anaphylac toid purpura and has instuction

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

    OpenAIRE

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

    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.

  4. Drug: D09348 [KEGG MEDICUS

    Lifescience Database Archive (English)

    Full Text Available D09348.gif Treatment of rheumatoid arthritis, immune thrombocytopenic purpura, and B-cell lymphoma spleen ty...D09348 Drug Fostamatinib disodium (USAN) C23H24FN6O9P. 6H2O. 2Na 732.1756 732.5148

  5. [Diagnosis and therapy of systemic necrotizing vasculitis].

    Science.gov (United States)

    Aksamija-Rizvić, B

    1992-01-01

    Through the presentation of the case of Leucocytoclastic vasculitis of a young man we enlighten the problems of diagnosis, differential diagnosis, ethiology, development of disease, and therapeutic approach. We presented the new knowledge in the patogenesis of changes in the vessel wall and possible correlation with thrombotic thrombocytopenic purpura.

  6. Lenalidomide-Associated ITP

    Directory of Open Access Journals (Sweden)

    Christina I. Herold

    2011-01-01

    Full Text Available Lenalidomide is a potent immunomodulatory agent being used increasingly for treatment of hematologic malignancies including multiple myeloma and myelodysplasia. The common toxicities of lenalidomide, including dose-limiting myelosuppression, are well described. However, the immunomodulatory properties of lenalidomide may give rise to unexpected autoimmune complications. Herein, we describe a case of immune thrombocytopenic purpura (ITP associated with use of lenalidomide.

  7. Rituximab treatment for symptomatic chronic ITP

    NARCIS (Netherlands)

    Tamminga, Rienk Y. J.; Bruin, Marrie C. A.

    2006-01-01

    About 20% of the children diagnosed with acute idiopathic thrombocytopenic purpura (ITP) will run a chronic course. Only in a minority of these, platelet-count-enhancing treatments are indicated. Most treatment options are directed at decreasing platelet destruction including corticosteroids, intrav

  8. How Do I Talk to My Family about Gaucher?

    Science.gov (United States)

    ... to healthcare providers. TALKING ABOUT GAUCHER 1 Gaucher Disease What is Gaucher? Gaucher (pronounced go-shay) is an inherited condition ... Multiple myeloma • Idiopathic thrombocytopenic purpura (ITP) • Neimann-Pick disease TALKING ABOUT GAUCHER 7 How are families affected? A diagnosis of ...

  9. Drug: D05777 [KEGG MEDICUS

    Lifescience Database Archive (English)

    Full Text Available D05777 Drug Ruplizumab (USAN/INN) Treatment of immune thrombocytopenic purpura and ...receptor signaling pathway Target-based classification of drugs [BR:br08310] Cyto...kines TNF family CD40L (CD154) [HSA:959] [KO:K03161] Ruplizumab D05777 Ruplizumab (USAN/INN) CAS: 220651-94-5 PubChem: 47207438 ...

  10. Autoimmune hemolytic anemia, as part of Evans' syndrome, caused by cold reactive IgG autoantibodies

    NARCIS (Netherlands)

    Jaarsma, AS; Muis, N; DeGraaf, SSN

    1996-01-01

    We describe a boy with Evans' syndrome, consisting of immune thrombocytopenic purpura at age 2 and autoimmune hemolytic anemia (AIHA) at age 4. AIHA was caused by cold Ige autoantibodies. This is unusual because AIHA is generally associated with either warm IgG antibodies or cold IgM antibodies. Tre

  11. 白细胞滤过的血小板预防血小板输注无效和人类白细胞抗原同种免疫反应的Meta分析%Prevention of Platelet Transfusion Refractoriness and HLA-alloimmunization by Leukocyte-filtered Platelet Transfusion: A Meta-analysis

    Institute of Scientific and Technical Information of China (English)

    袁强; 陈雪; 成岚; 周昌华; 傅雪梅; 李幼平; 王乃红; 王莉

    2011-01-01

    Objective To compare and assess the effectiveness of leukocyte-filtered platelet and standard platelet concentrates transfusion in preventing platelet transfusion refractoriness ( PTR) and human leukocyte antigen (HLA) -alloimmunization. Methods Randomized controlled trials (RCTs) or quasi-RCTs comparing leukocyte-filtered platelet with standard platelet concentrates transfusion ( up to December 31,2009) were searched and identified from Medline, EMBASE, The Cochrane Library, and CBM. A meta-analy-sis was conducted with Cochrane Collaboration's RevMan 5. 0. Results The search identified 558 citations in total, in which 7 articles in English were finally included in the meta-analysis. The analysis showed that compared with standard platelet concentrates transfusion, leukocyte-filtered platelet transfusion significantly decreased PTR [ RR = 0. 59, 95% CI (0.42, 0. 82), P = 0. 002 ] and HLA-alloimmunization [ RR = 0. 49, 95% CI (0. 33 , 0. 74) , P = 0. 0006 ]. Subgroup analysis showed that HLA-alloimmunization was significantly reduced by leukocyte-filtered platelet transfusion among the patients with acute myelocytic leukemia [ RR = 0. 42, 95% CI (0. 32, 0. 56 ) , P < 0. 00001 ], while no significant difference was detected in patients with acute lymphoblastic leukemia because of the limited sample size [ RR = 0. 50, 95% CI (0. 10, 2. 41) , P = 0. 39 ]. Conclusions The current evidence shows that leukocyte-filtered platelet transfusion can prevent PTR and HLA-alloimmunization more effectively than standard platelet transfusion. Well-designed large-scale RCTs are still needed to further confirm this finding.%目的 系统评价白细胞滤过的血小板与标准血小板输注预防血小板输注无效(PTR)和人类白细胞抗原(HLA)同种免疫反应的效果.方法 电子检索Medline数据库、EMBASE、The Cochrane Library和CBM收录的白细胞滤过血小板与标准血小板输注的随机或半随机对照试验(截至2009年12月31日),对预防PTR

  12. 儿童紫癜性肾炎诊治现状多中心回顾性调查分析%Multicenter investigation of diagnosis and treatment of Henoch-Schonlein purpura nephritis in childhood

    Institute of Scientific and Technical Information of China (English)

    全国儿童常见肾脏病诊治现状调研工作组

    2013-01-01

    目的 探讨我国住院儿童紫癜性肾炎的临床特点、病理表现及治疗现状.方法 回顾性分析全国40所医院2008年7月1日至2011年6月30日收治的新发过敏性紫癜、经临床或病理确诊为紫癜性肾炎并在调查单位接受治疗的紫癜性肾炎患儿的病历资料,并填写统一的调查表,收集相关资料,病例登记表经参研单位初步汇总后,由中华医学会儿科学分会肾脏学组最终汇总,由专门数据录入人员录入和核对,并随机抽样进行数据核实,最后对儿童紫癜性肾炎的临床特点、病理表现及治疗现状进行统计分析.结果 此次调研最终符合入选条件且病例完整可供分析者共4863例患儿,其中男2935例,女1928例,男女比例为1.52∶1,6~13岁为发病高峰.4702例(96.7%)患儿于过敏性紫癜起病6个月内确诊肾损害.临床分型最常见为血尿和蛋白尿型(2831例,58.2%).其中1625例患儿行肾活检治疗,最常见肾活检指征为蛋白尿合并镜下血尿(1149例,70.7%).1448例肾活检患儿提供病理分级,以Ⅲ级和Ⅱ级为多见.4863例患儿中接受激素及其他免疫抑制剂治疗者共3677例(75.6%).最常见的治疗方案为单用激素1655例(34.0%);单纯血尿型患儿(362例,56.2%)多未予特殊治疗,血尿和蛋白尿型患儿最常选择治疗方案仍为单用激素治疗.肾组织病理Ⅰ级和Ⅱ级以单用激素或联合雷公藤多甙治疗为主,病理Ⅲ级和Ⅳ级以激素+环磷酰胺+甲泼尼龙冲击治疗为主.结论 本研究调查分析了我国住院紫癜性肾炎患儿的临床诊治现状,目前多根据临床分型及参照病理分级选择治疗方案,但免疫抑制剂及非特异性治疗的选择和使用方式多样,仍有待进一步临床多中心研究结果验证并加以规范.%Objective To retrospectively investigate the current diagnosis and treatment of children with Henoch-Schonlein purpura nephritis in hospital,to survey the

  13. Detection of serum allergen specific IgE in the child with recurrent auergic purpura%过敏性紫癜患儿血清过敏原特异性IgE的检测及意义

    Institute of Scientific and Technical Information of China (English)

    夏秀娟

    2009-01-01

    Objective To investigate the role of allergen specific IgE in the child with recurrent auergic purpura,and to detect the relation between all kinds of allergen and the disease.Methods The serum specific IgE of allergen detected by ELISA in 100 cases of the child with recurrent auergic purpura.Results 81 cases of serum allergen-specific IgE were over than 50ku/L(81%).52 cases(52%)were positive reaction to more than two kinds of allergens and 29 cases(29%)were positive reaction to one kind of allergen.Several familiar aeroallergen mixed molds (19/100),dermatophagoides(12/100)and mugwort pollens(9/100).The most familiar food fish(28/100).shrimpand crab(22/100),milk(18/100).protein(11/100).Conclusion It suggests that aeroallergens,fish,shrimp and crab play an important role in the pathogenesis of repeating child anaphy lactic purpura.Mixed molds and protein foods are important pathogens to the disease.To detect the serum specific IgE could help screen allergens,and have important role to prevent disease.%目的 探讨反复发作性过敏性紫癜患儿血清过敏原特异性IgE的变化及与疾病发病的关系.方法 采用酶联免疫吸附试验检测100例反复发作性过敏性紫癜患儿的血清过敏原特异性IgE.结果 100例患儿中血清总IgE水平>50 ku/L者81例,阳性率81%,其中52例(52%)对两种以上过敏原呈阳性反应,29例(29%)对一种过敏原呈阳性反应;常见吸人性过敏原为霉菌(19/100)、尘螨(12/100)和蒿类花粉(9/100).常见的食人性过敏原为海鱼类(28/100)、虾蟹(22/100)、牛奶(18/100)、蛋白蛋黄类(11/100).结论 反复发作性过敏性紫癜的发生与吸人性和食人性过敏原都有一定的相关性,特异性IgE检测可帮助筛选过敏原,对疾病的预防具有积极意义.

  14. 以腹痛为首发症状的31例儿童过敏性紫癜临床分析%Clinical Analysis of Abdominal Pain as the First Symptom of 31 Children with Allergic Purpura

    Institute of Scientific and Technical Information of China (English)

    阮美江

    2015-01-01

    目的:探讨以腹痛为首发症状的儿童过敏性紫癜( HSP)的临床特征和实验室检查特点,为该病早期诊断提供依据。方法回顾性分析我院2011-08~2014-03间收治的31例以腹痛为首发症状的HSP患儿的临床资料,记录患儿临床表现、实验室检查及误诊情况。结果患儿多表现为全腹痛、持续性钝痛、无固定压痛点,实验室检查阳性者较少,且误诊率较高,容易与急性阑尾炎、十二指肠炎等疾病相混淆。结论遇到不明原因以全腹痛、持续性钝痛和无固定压痛点为主且腹部症状与体征不甚符合者,亦无皮疹出现时,应考虑HSP的可能。%Objective To discuss the clinical features and laboratory features of abdominal pain as the first symptom of children with allergic purpura ( HSP) , to provide the basis for early diagnosis of the disease.Methods A retrospective analysis of abdominal pain as the first symptom of 31 children with allergic purpura in the hospital from August, 2011 to March, 2014 was done, the clinical data, laboratory tests and misdiagnosis of 31 cases were recorded.Results Children had more performance of the whole abdominal pain, persistent dull pain, no fixed point of tenderness and less positive laboratory tests, and had the high rate of misdiagnosis with acute appendicitis and duode-nal disease.Conclusion Under unexplained full abdominal pain, persistent dull pain and tenderness without a fixed point-based and less abdominal symptoms and signs, and no rash appearance, possibility of HSP should be considered.

  15. 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

  16. IgA Nephropathy and Henoch-Schoenlein Purpura Nephritis: Aberrant Glycosylation of IgA1, Formation of IgA1-Containing Immune Complexes, and Activation of Mesangial Cells

    DEFF Research Database (Denmark)

    Novak, J.; Moldoveanu, Z.; Renfrow, M.B.;

    2007-01-01

    IgA1 in the circulation and glomerular deposits of patients with IgA nephropathy (IgAN) is aberrantly glycosylated; the hinge-region O-linked glycans are galactose-deficient. The circulating IgA1 of patients with Henoch-Schoenlein purpura nephritis (HSPN) has a similar defect. This aberrancy...... at specific sites. We sought to define the aberrant glycosylation of a galactose-deficient IgA1 myeloma protein and analyze the formation of the immune complexes and their biological activities. Supplementation of serum or cord-blood serum with this IgA1 protein resulted in formation of new IgA1 complexes...... determined the O-glycosylation sites in the hinge region of the IgA1 myeloma protein and IgA1 proteins from sera of IgAN patients. The IgA1 myeloma protein had galactose-deficient sites at residues 228 and/or 230 and 232. These sites reacted with IgG specific to galactose-deficient IgA1. IgA1 from the Ig...

  17. AMEGAKARYOCYTIC THROMBOCYTOPENIC PURPURA‎: A FIFTEEN YEAR EXPERIENCE

    Directory of Open Access Journals (Sweden)

    M. Bakhshi

    2006-07-01

    Full Text Available Totally implantable venous access devices (TIVAD or implantable catheter ports are devices which can be implanted subcutaneously. They enable prolonged and repeated access to the vascular system, into the peritoneal cavity or intravertebral space. This device is particularly useful for repeated medical injection, for blood sampling or transfusion of blood and blood derivatives and for total parenteral nutrition (TPN. Although many patients benefit from the insertion of TIVAD without any secondary effects, any surgical implantation can nevertheless lead to complications. ‎In this study, we investigated the advantages and disadvantages of TIVAD catheter in pediatric age group. A total of 94 cases, 2 to 14 years old, were included in our study. We implanted TIVAD in these patients for chemotherapy in 83 cases (88.29%, for prolonged TPN in 6 cases (6.38%, for corticosteroid and antibiotic therapy after ‎Kasai operation in 2 cases (2.12%, for intermittent IV therapy in 2 cases (2.12% and for need to partial parenteral nutrition in 1 case (1.06%. Out of 94 cases, 14 cases (15% had some kind of complications and 80 cases (85% had no complication. There was no mortality. Most patients and their parents (82 cases, 87.23% were satisfied from TIVAD. ‎It seems that TIVAD can be a useful device for many chronic patients who need an IV access for multiple injections.

  18. Alterações ultra-sonográficas na gravidez Rh negativo sensibilizada avaliada pela espectrofotometria do líquido amniótico e pela dopplervelocimetria da artéria cerebral média Ultrasound findings in Rh-alloimmunized pregnancies assessed by spectrophotometric analysis of amniotic fluid and Doppler velocimetry of fetal middle cerebral artery

    Directory of Open Access Journals (Sweden)

    Luciano Marcondes Machado Nardozza

    2006-02-01

    Full Text Available OBJETIVO: Avaliar e confrontar a presença de alterações ultra-sonográficas nas gestações Rh negativo sensibilizadas, quando a anemia fetal foi determinada ou pela espectrofotometria do líquido amniótico, ou pela dopplervelocimetria da artéria cerebral média. MATERIAIS E MÉTODOS: Observacional descritivo com grupo de comparação. Nosso grupo de estudo foi constituído por 99 pacientes, avaliadas no período de janeiro de 1995 a janeiro de 2004. Foram analisados e comparados dois grupos: 74 gestantes sensibilizadas pelo fator Rh cuja anemia fetal foi acompanhada pela espectrofotometria (grupo SE e 25 gestantes sensibilizadas pelo fator Rh cuja anemia fetal foi acompanhada pela dopplervelocimetria (grupo SD. Avaliamos a presença ou não de alterações ultra-sonográficas no acompanhamento pré-natal e confrontamos os dois grupos de estudo. RESULTADOS: No grupo cuja anemia fetal foi acompanhada através da espectrofotometria (grupo SE, apuramos modificações placentárias, principalmente o aumento da espessura e sua alteração textural, mais assiduamente que as encontradiças no grupo de gestantes sensibilizadas, em que a anemia foi determinada através da dopplervelocimetria (grupo SD (64% X 32%, p = 6,294. CONCLUSÃO: As alterações ultra-sonográficas foram detectadas em dobro quando a anemia foi avaliada pela espectrofotometria em comparação com o grupo seguido pela dopplervelocimetria.OBJECTIVE: To evaluate and compare existing ultrasound findings in women with Rh-alloimmunized pregnancies with diagnosis of fetal anemia by spectrophotometric analysis of amniotic fluid or Doppler ultrasound of the fetal middle cerebral artery. MATERIALS AND METHODS: This was an observational descriptive study involving 99 patients evaluated between January 1995 and January 2004. Patients were divided into two groups: 74 Rh-isoimmunized women submitted to spectrophotometry of the amniotic fluid (group S and 25 Rh-isoimmunized women submitted to

  19. Correlation between food intolerance and Henoch-Sch(o)nlein purpura in children%食物不耐受与儿童过敏性紫癜的相关性

    Institute of Scientific and Technical Information of China (English)

    张建江; 史佩佩; 张利果; 陆凤霞; 赵丽丽

    2011-01-01

    Objective To investigate the correlation between food intolerance and Henoch-Sch(o)nlein purpura (HSP) in children and the efficacy of food forbidden or alternative therapy. Methods The levels of IgG against several common food in serum obtained from 40 children with HSP were measured by ELISA. The efficacy of food forbidden or alternative food therapy was assessed after 3 months. Results Total positive rate of serum food-intolerant IgG antibodies in HSP children was 92.5%. Among these 14 intolerant foods, the positive percentage of egg was the highest (33.8%), followed by tomato (14.9%), milk (13.5%) and morrhua (12.2%). Significant differences of the sort and degree of food intolerance existed among different age groups (t=2.257, P=0.045), but not between boys and girls (t=1.053, P=0.315), city and countryside (t= 1.388, P=0.193). There was no linear correlation between total food intolerance and serum IgG level (t=0.793, P=0.445). Food intolerance had no direct relation to immune complex deposition in kidneys of HSP nephritis (r =-0.262, P =0.387). The efficacy of adjusting diet was 95% . Conclusions HSP is closely related to food intolerance. Egg is the most common intolerant food. Food forbidden or alternative therapy shows acceptable efficacy in the treatment of most Henoch-Sch(o)nlein purpura children.%目的 探讨食物不耐受与儿童过敏性紫癜的相关性及不耐受食物禁食或轮替疗法对过敏性紫癜患儿的疗效.方法 ELISA法检测40例过敏性紫癜患儿血清14种食物特异性IgG浓度及相关生化指标,同时进行3个月不耐受食物禁食或轮替疗法,观察疗效.结果 过敏性紫癜息儿食物不耐受阳性率为92.5%,鸡蛋的阳性率最高,其次为西红柿、牛奶、鳕鱼等.食物不耐受种类在男女、城乡分布间差异无统计学意义(t=1.053,P=0.315;t=1.388,P=0.193),但不同年龄间差异有统计学意义(t=2.257,P=0.045).食物不耐受累计敏感度与血

  20. 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

  1. Acute scrotum as the first symptom of Henoch-Schonlein purpura: report of 14 cases%以阴囊急症为首发症状的过敏性紫癜的治疗

    Institute of Scientific and Technical Information of China (English)

    白明; 孙熠; 张敬悌; 葛文安; 王盛兴; 郭涛; 杨增雷; 张岗

    2012-01-01

    目的 总结14例以阴囊急症为首发症状的过敏性紫癜的发病及转归特点,以期提高对该类疾病的综合认识.方法 回顾2001年7月至2010年3月本科收治的14例以阴囊急症为首发症状的过敏性紫癜的临床资料.14例入院时均没有皮疹,其中8例诊断为睾丸附睾感染;3例诊断为睾丸附件扭转;2例诊断为睾丸扭转;1例诊断为双侧睾丸挫伤、会阴腹股沟软组织挫伤.1例合并腹痛,1例合并左膝关节肿痛.结果 5例患儿在入院后2~8 h出现皮肤紫癜,8例在入院后12~36 h出现皮肤紫癜,外伤的1例患儿入院后逐渐出现右膝关节肿痛转内科治疗,转科后2d出现皮疹.内科综合治疗后阴囊症状逐渐消失.所有患儿出院后均在血液科门诊随访3个月至5年,所受累阴囊睾丸未见异常.结论 以阴囊急症为首发症状的过敏性紫癜临床上不多见,超声在小儿阴囊急症的诊断中具有较高的准确性.对临床表现为阴囊急症尤其合并腹痛、关节肿痛的儿童需考虑到存在过敏性紫癜的可能性,综合的判断分析能够尽可能减少不必要的探查手术.%Objective To summarize the characteristics and clinical outcome of 14 cases of Henoch-Schonlein purpura (HSP) in children with acute scrotum as its first symptom.Methods We retrospectively reviewed the clinical data of 14 male children with HSP between July 2001 and March 2010.None of the children had skin eruption at the time of admission.Eight children were diagnosed as epididymo-orchitis.Three were diagnosed as torsion of the testicular appendage.Two children were diagnosed as testicular torsion.And one child was diagnosed as having bilateral testicular contusions.One case had associated abdominal pain,one case had left knee joint swelling and pain.Results Five children developed skin purpura 2-8 hours after admission.In eight cases,eruptions were found 12 36 hours after admission in 12 to 36 hours.In the patient who had

  2. Petechial rash in children: a clinical dilemma.

    Science.gov (United States)

    Barnetson, Laura; Heaton, Paul Anthony; Palmer, Sarah; Paul, Siba Prosad

    2016-05-01

    Children with a petechial rash commonly present to emergency departments. The rash can be associated with serious illnesses, such as invasive meningococcal disease (IMD), but is usually associated with less severe conditions. This article discusses the common and important causes of petechial rash, including IMD, viral illnesses, trauma, Henoch-Schönlein purpura and idiopathic thrombocytopenic purpura. It also analyses the National Institute for Health and Care Excellence (NICE) ( 2014 ) pathway for management of petechial rash in children and examines treatment of the various causes. The article includes two relevant case studies and discusses the role of emergency nurses.

  3. Neutropenia Inmune - Aloinmune neonatal: IgG sérica reactiva y fenotipo específico de los neutrófilos evaluados por citometría de flujo Autoimmune-alloimmune neonatal neutropenia: Serum reactive IgG and neutrophil-specific phenotype detected by flow cytometry

    Directory of Open Access Journals (Sweden)

    Norma E. Riera

    2006-10-01

    Full Text Available La neutropenia inmune se diagnostica por la presencia de auto o aloanticuerpos reactivos con los neutrófilos. La neutropenia aloinmune neonatal es consecuencia de la sensibilización materna a los antígenos específicos de los neutrófilos paternos que afectan al neonato al atravesar la barrera placentaria. Se presentan 4 casos de niños, 2 de ellos hermanos consanguíneos con doble vínculo. Se estudiaron los sueros de los pacientes y sus padres. Por citometría de flujo se establecen los valores de referencia de la IgG sérica reactiva con los neutrófilos en voluntarios sanos, para 3 diluciones (1/2, 1/5 y 1/20 en reacción autóloga (suero y células de un mismo individuo y heteróloga (suero y células de diferentes individuos. Los resultados se expresan por un índice definido como el cociente entre la mediana de la intensidad de fluorescencia media del suero incógnita y la de un suero utilizado como referencia. Por leucoaglutinación se evaluó la dilución del suero 1/20. Se determinó el nivel de complejos inmunes circulantes. Se determinó el fenotipo, para los epitopes HNA-1a, HNA-1b y HNA-2a. En los 4 niños se encontró IgG reactiva y/o factores aglutinantes; 2/3 sueros maternos fueron reactivos con los neutrófilos del cónyuge y de los hijos. Los complejos inmunes circulantes fueron positivos en 2/4 sueros negativos en 3/3 sueros maternos. Se encontró incompatibilidad materno-infantil en los 4 casos. Las 3 madres tenían igual fenotipo: homocigotos NA1/NA1, NB1+. En síntesis, se presenta el hallazgo de 4 casos con neutropenia inmune: 3/4 auto-inmune, 1/3 se asocia a complejos inmunes circulantes y 1/4 con neutropenia neonatal aloinmune.Auto or alloantibodies reactive with neutrophils define immune neutropenia. Alloimmune neonatal neutropenia is caused by maternal sensitization to paternal neutrophil antigens, resulting in IgG antibodies that are transferred to the fetus through the placenta. We present the studies in 4

  4. Correlação entre medidas ultra-sonográficas do coração e o deficit de hemoglobina em fetos de gestantes aloimunizadas Correlation between echographic cardiac measurements and hemoglobin deficit in fetus of red cell alloimmunized pregnancies

    Directory of Open Access Journals (Sweden)

    Marília Zicker Hanan

    2008-07-01

    Full Text Available OBJETIVO: verificar a correlação entre as medidas ultra-sonográficas do coração fetal e o deficit de hemoglobina em fetos de gestantes aloimunizadas. MÉTODOS: estudo transversal, no qual foram incluídos 60 fetos de 56 mulheres aloimunizadas, com idade gestacional entre 21 e 35 semanas. Foram realizados 139 procedimentos. Antes da cordocentese para a coleta de sangue fetal, as medidas cardíacas e o comprimento do fêmur (CF foram avaliados pela ultra-sonografia. O diâmetro biventricular externo (DBVE foi obtido no final da diástole, com o cursor modo-M perpendicular ao septo interventricular, nas válvulas atrioventriculares, medindo-se a distância entre as partes externas dos epicárdios. A medida do diâmetro atrioventricular (DAV foi obtida posicionando-se o mesmo cursor ao longo do septo interventricular, avaliando-se a distância entre a base e o ápice do coração. O CF foi determinado do trocanter maior à metáfise distal. Foi calculada a circunferência cardíaca (CC. Para ajustar as medidas cardíacas à idade gestacional, dividiu-se cada uma dessas medidas pela medida do CF. A concentração de hemoglobina foi determinada por espectrofotometria no sistema Hemocue®. O deficit de hemoglobina foi calculado baseado na curva de normalidade de Nicolaides. RESULTADOS: observaram-se correlações diretas e significativas entre as medidas cardíacas avaliadas e o deficit de hemoglobina. Para a predição das anemias moderada e grave, a sensibilidade e a especificidade encontradas foram, respectivamente, de 71,7 e 66,3% para a razão DBVE e CF; 65,8 e 62,4% para a DAV e CF e 73,7 e 60,4% para a CC e CF. CONCLUSÕES: nos fetos de gestantes aloimunizadas, as medidas cardíacas ultra-sonográficas avaliadas correlacionam-se diretamente com o deficit de hemoglobina.PURPOSE: to verify the correlation between ultrasonography heart measures and hemoglobin deficit in fetuses of alloimmunized pregnant women. METHODS: a transversal study

  5. Immunoregulatory Effects of Everolimus on In Vitro Alloimmune Responses

    Science.gov (United States)

    Levitsky, Josh; Miller, Joshua; Huang, Xuemei; Gallon, Lorenzo; Leventhal, Joseph R.; Mathew, James M.

    2016-01-01

    Everolimus (EVL) is a novel mTOR-inhibitor similar to sirolimus (SRL) that is used in organ transplant recipients, often in combination with tacrolimus (TAC) or mycophenolate (MPA). The current study aims to determine its effects on regulatory T cells. Increasing concentrations of EVL, MPA and TAC alone or in combination were added to MLRs of healthy volunteers. Lymphoproliferation by 3H-TdR incorporation and the percentage of newly generated CD4+CD127-CD25+FOXP3+ (total Treg) and CD4+CD127-CD25HighFOXP3+ (natural Treg) in CFSE labeled responder cells were assessed by flow cytometry. In comparison to medium controls, EVL and other agents dose-dependently inhibited 3H-TdR incorporation in HLA-2DR-matched and HLA-mismatched MLRs (n = 3–10). However, EVL significantly amplified newly generated total and natural Tregs in CFSE labeled responder cells (p SRL> MPA > TAC. If the results from these in vitro studies are extrapolated to clinical transplantation, it would suggest EVL plus low concentrations of MPA may be the most tolerogenic combination. PMID:27275747

  6. Immunoregulatory Effects of Everolimus on In Vitro Alloimmune Responses.

    Directory of Open Access Journals (Sweden)

    Josh Levitsky

    Full Text Available Everolimus (EVL is a novel mTOR-inhibitor similar to sirolimus (SRL that is used in organ transplant recipients, often in combination with tacrolimus (TAC or mycophenolate (MPA. The current study aims to determine its effects on regulatory T cells. Increasing concentrations of EVL, MPA and TAC alone or in combination were added to MLRs of healthy volunteers. Lymphoproliferation by 3H-TdR incorporation and the percentage of newly generated CD4+CD127-CD25+FOXP3+ (total Treg and CD4+CD127-CD25HighFOXP3+ (natural Treg in CFSE labeled responder cells were assessed by flow cytometry. In comparison to medium controls, EVL and other agents dose-dependently inhibited 3H-TdR incorporation in HLA-2DR-matched and HLA-mismatched MLRs (n = 3-10. However, EVL significantly amplified newly generated total and natural Tregs in CFSE labeled responder cells (p SRL> MPA > TAC. If the results from these in vitro studies are extrapolated to clinical transplantation, it would suggest EVL plus low concentrations of MPA may be the most tolerogenic combination.

  7. Alloimmunization in autoimmune hemolytic anemia patient: The differential adsorption approach

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    Ravi C Dara

    2017-01-01

    Full Text Available Patients of β-thalassemia major are dependent on regular blood transfusions for their entire lifetime. Development of antibodies against red blood cell (RBC antigen which may be alloantibody or autoantibody, several times as a result of frequent red cell component transfusions, further complicates the subsequent transfusion therapy. Among the autoantibodies, warm-reactive autoantibodies are commoner and interfere in the pretransfusion testing. These RBC autoantibodies present in patient's serum potentially react with all the cells of antibody identification panel giving “pan-reactive” picture and making alloantibody identification complex. In this report, we present our approach in a thalassemia patient who presented with warm-type autoimmune hemolytic anemia, low hemoglobin of 5.8 g/dl, and three significant alloantibodies (anti-D, anti-S, and anti-Jk b which were masked by pan-reactive warm autoantibody(s. Differential adsorption was used to unmask underlying alloantibodies. We suggest that differential adsorption procedure is an effective and efficient method for autoantibody adsorption, detection, and identification of masked alloantibody(s, especially in patients with low hemoglobin and history of recent blood transfusion.

  8. Alloimmunization in autoimmune hemolytic anemia patient: The differential adsorption approach

    Science.gov (United States)

    Dara, Ravi C.; Tiwari, Aseem Kumar; Arora, Dinesh; Mitra, Subhasis; Acharya, Devi Prasad; Aggarwal, Geet; Sharma, Jyoti

    2017-01-01

    Patients of β-thalassemia major are dependent on regular blood transfusions for their entire lifetime. Development of antibodies against red blood cell (RBC) antigen which may be alloantibody or autoantibody, several times as a result of frequent red cell component transfusions, further complicates the subsequent transfusion therapy. Among the autoantibodies, warm-reactive autoantibodies are commoner and interfere in the pretransfusion testing. These RBC autoantibodies present in patient's serum potentially react with all the cells of antibody identification panel giving “pan-reactive” picture and making alloantibody identification complex. In this report, we present our approach in a thalassemia patient who presented with warm-type autoimmune hemolytic anemia, low hemoglobin of 5.8 g/dl, and three significant alloantibodies (anti-D, anti-S, and anti-Jkb) which were masked by pan-reactive warm autoantibody(s). Differential adsorption was used to unmask underlying alloantibodies. We suggest that differential adsorption procedure is an effective and efficient method for autoantibody adsorption, detection, and identification of masked alloantibody(s), especially in patients with low hemoglobin and history of recent blood transfusion. PMID:28316442

  9. Frequency of anti-glycoprotein Ia/IIa (anti-HPA-5b,-5a and anti-glycoprotein IIb/IIIa (anti-HPA-1a,-3a,-4a alloantibodies in multiparous women of African descent

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    Zaccheaus A Jeremiah

    2010-05-01

    Full Text Available Zaccheaus A Jeremiah1, Justina E Oburu2, Osaro Erhabor1, Fiekumo I Buseri1, Teddy C Adias31Haematology and Blood Transfusion Science Unit, Department of Medical Laboratory Sciences, College of Health Sciences, Niger Delta University, Wilberforce Island, Nigeria; 2Department of Haematology and Blood Transfusion, University of Port Harcourt Teaching Hospital, Port Harcourt, Nigeria; 3Rivers State University of Science and Technology, Port Harcourt, NigeriaBackground: Human platelet antibodies are often implicated in some disease conditions, such as neonatal alloimmune thrombocytopenia (NAIT, idiopathic thrombocytopenic purpura (ITP and platelet refractoriness. The frequencies of these alloantibodies have not been reported in Nigeria and West Africa.Methods: Screening for allontibodies to human platelet antigens (HPA was undertaken using the GTI PakPlus® qualitative solid phase ELISA reagent. Platelet count was done using the ICSH approved procedure using 1% ammonium oxalate reagent.Study design: A cross-section of apparently healthy adult Nigerian multiparous non-pregnant women, who were staff of a tertiary health facility in the Niger Delta, Nigeria, were screened for alloantibodies to human platelet antigens.Results: Of the one hundred (100 women screened, the prevalence of anti-glycoprotein IIb/IIIa (anti-HPA-Ia,-3a,-4a was zero percent (0%, anti-glycoprotein Ia/IIa (anti-HPA-5b accounted for 30% of results, while anti-glycoprotein Ia/IIa (anti-HPA-5a was 18%. Parity was found to exert significant influence on the development to HPA antibodies (Fisher’s Exact Test = 11.683, P < 0.05; 13.577, P < 0.01. The platelet count of the women did not appear to exert any influence on the development of the antibodies (P > 0.05.Conclusion: This study has observed a high prevalence of anti-HPA-5b in our sample population. The prevalence of alloantibodies to HPA antigens was found to associate strongly with parity. These results indicate that there is a

  10. Logistic regression analysis of the risk factors for kidney damage in children with Henoch-Schonlein purpura%儿童过敏性紫癜肾脏损害危险因素Logistic回归分析

    Institute of Scientific and Technical Information of China (English)

    刘静; 陆彪

    2011-01-01

    目的:分析过敏性紫癜(HSP)患儿肾脏损害的相关危险因素.方法:对2008年8月至2010年8月住院确诊为HSP207例患儿的临床资料进行回顾性分析.结果:单因素分析年龄、临床类型、M、B、SD、PLT、PDW、CER、PTA、PTR、INR、APTT-R、AG、TP、ALB、GGT、LDH、TG、CHOL、CREA、IgG 差异有统计学意义(P<0.05),年龄、皮疹反复、消化道症状、ALB、LDH在肾脏损害易感因素Logistic回归分析中有统计学意义(P=0.002、P=0.008、P=0.007、P=0.005、P=0.04).结论:年龄、皮疹反复、消化道症状、ALB、LDH是HSP肾脏损害的危险因素,对于有上述因素的HSP患儿更应加强观察.%Objective To investigate the risk factors for kidney damage in children with Henoch-Schonlein purpura (HSP). Methods Clinical data of 204 children with HSP from August 2008 to August 2010 were retrospectively analyzed. Results Univariate analysis indicated that age, clinical types, M, B, SD, PLT, PDW, CER, PTA, PTR, INR, APTT-R, AG, TP, ALB, GGT, LDH, TG, CHOL, CREA, and IgG werre significantly positive (P < 0.05). The stepwise logistic regression analysis showed that age, repeated shin rash, gastrointestinal symptoms, ALB, and LDH were the risk factors for kidney damage in children with HSP (P= 0.002, P= 0.008, P= 0.007, P= 0.005, P= 0.04). Conclusion Age, repeated shin rash, gastrointestinal symptoms, ALB, and LDH were the risk factors for kidney damage in children with HSP, patients who had these factors would need reinforced observation.

  11. Uso de Terapia VAC® en paciente pediátrico con exposición ósea postamputación tras púrpura fulminans VAC® Therapy in pediatric patient with bone exposition after purpura fulminans

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    J. Navarro Cecilia

    2011-12-01

    Full Text Available La sepsis meningocóccica se asocia con un alto índice de mortalidad. Los pacientes afectados pueden mostrar signos de coagulación intravascular diseminada (CID y necrosis de partes blandas debidas a baja perfusión tisular. Los procedimientos quirúrgicos precoces suelen demorarse en estos casos, pues inicialmente es difícil determinar el grado de la lesión. Una vez que el paciente está estable, es esencial el desbridamiento de todo tejido necrótico, teniendo como objetivo el preservar la máxima cantidad de tejido posible, especialmente las articulaciones. Muchos niños sobreviven a esta patología, pero sufren gran morbilidad: amputación de una o varias extremidades y otras complicaciones derivadas como exposiciones óseas. Existen pocas publicaciones que hagan referencia al tratamiento no quirúrgico de este tipo de lesiones. Presentamos un caso de aplicación del sistema de terapia de presión negativa VAC® en paciente en edad pediátrica tras pérdida de sustancia como resultado de una púrpura fulminans secundaria a meningococcemia.Meningococcal sepsis is associated with a high mortality rate. These patients may show severe disseminated intravascular coagulation (DIC and skin, subcutaneous tissue and muscle necrosis due to poor tissue perfusion. It is rare that early surgical intervention be required, as it is relatively difficult to determine the degree of tissue loss early on. Once the patient is stable, debridement of all necrotic tissue is essential, and the goals are still to preserve as much tissue as possible, specially the joints. Many children survive the disease, but suffer major morbidities such as extremities amputation, which may result of complications like bone exposure. There is very little published experience regarding the non-surgical treatment of this complication. We report a new possible application of the vacuum-assisted closure system (VAC® in very young patients with loss of tissue as a result of purpura

  12. 肝素钠治疗小儿过敏性紫癜的护理体会%Nursing Experience on Treatment of the Children’s Anaphylactoid Purpura with Heparin Sodium

    Institute of Scientific and Technical Information of China (English)

    2013-01-01

    Objective:To explore how nurses improve their skills efficiently by summarizing the nursing experience on treatment of the children’s anaphylactoid purpura with heparin sodium with the use of heparin sodium in author’s department.Method:11 cases of children who suffered AP in our pediatrics dept from 1st Jan.2011 to 30 Nov.2012 were collected.Combined with characteristics of young patients,the nursing strategies were raised with analyzing of nursing interventions.Result:The HS treatment to children’s AP was highly safe and convenient.Apart from the general nursing cares,the study of HS’nursing essentials was necessary.Conclusion:In order to have more and more patients healed,it’s imperative to familiarize its medicinal properties and operational skills.%  目的:总结本科在使用肝素钠(Heparin sodium,HS)治疗儿童过敏性紫癜(Anaphylactoid purpura,AP)的护理体会,探讨护士如何有效地提高自己的技术水平。方法:收集本科自2011年1月1日-2012年11月30日以来发作 AP 的患儿11例,结合本科患者年纪小的特点,并对这些病例的护理措施加以分析,并提出相应的护理对策。结果:HS 治疗 AP 安全性高且使用方便,除了一般的护理要素,掌握该药物的护理要点有十分必要性。结论:为了使该药物能更好地为患者服务,熟练掌握其药物特性及操作技能势在必行。

  13. Lymphoplasmacytoid lymphoma elaborating lambda chain paraprotein with amyloid deposition in Sjögren's syndrome.

    OpenAIRE

    Savage, A. W.; Shareef, D. S.; Maher, E. J.; Bennett, M. H.; Thomas, D J

    1989-01-01

    A woman presented with painful enlargement of her parotid and submandibular glands. She was shown to have the previously unreported combination of idiopathic thrombocytopenic purpura, Sjögren's syndrome, Hashimoto's disease, and myasthenia gravis. Parotid gland biopsy and bone marrow examination showed the presence of a rare lymphoplasmacytoid lymphoma. There was amyloid deposition in the parotid glands, gums and on muscle biopsy. Immunohistochemical staining of the parotid lymphoma and amylo...

  14. Haematological manifestations of lupus

    OpenAIRE

    2015-01-01

    Our purpose was to compile information on the haematological manifestations of systemic lupus erythematosus (SLE), namely leucopenia, lymphopenia, thrombocytopenia, autoimmune haemolytic anaemia (AIHA), thrombotic thrombocytopenic purpura (TTP) and myelofibrosis. During our search of the English-language MEDLINE sources, we did not place a date-of-publication constraint. Hence, we have reviewed previous as well as most recent studies with the subject heading SLE in combination with each manif...

  15. Púrpura trombocitopénico inmune asociado a infección por virus hepatitis C en paciente hemofílico: Caso clínico

    OpenAIRE

    Pilleux C,Lilian; MARTÍNEZ G,ALEJANDRA; DONOSO S,MARIO; CARRASCO L,CRISTIAN

    2010-01-01

    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 stero...

  16. 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 ...

  17. Oral vesiculobullous lesions: Consider the platelets

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    Clare Steel

    2014-01-01

    Full Text Available Oral vesiculobullous lesions or "blood blisters" can be found on a routine dental examination and may have many causes. Trauma is often the first diagnosis followed by a variety of bullous conditions, such as pemphigus and pemphigoid. Using a case report, we highlight the other, more serious, possibility of idiopathic thrombocytopenic purpura (ITP to raise awareness to the General Dental Practitioners and the need for prompt treatment.

  18. Multiple tuberculous aneurysms of the aorta.

    Science.gov (United States)

    Pierret, Charles; Tourtier, Jean-Pierre; Grand, Bertrand; Boddaert, Guillaume; Laurian, Claude; de Kerangal, Xavier

    2011-06-01

    Tuberculous aneurysms of the aorta are quite rare, but are exceptional when found in multiple locations. We report the case of multiple tuberculous aortic aneurysms of the thoracic and abdominal aorta in a 19-year-old female discovered when she consulted for thrombocytopenic purpura. The treatment for both locations included prolonged antituberculous therapy and surgical resection with cryopreserved aortic allograft patch for the reconstruction.

  19. Lack of Association between Anti-Phospholipid Antibodies (APLA) and Attention Deficit/Hyperactivity Disorder (ADHD) in Children

    OpenAIRE

    Yael Leitner; Isaac Vinograd; Yehuda Shoenfeld; Miriam Katz; Yair Levy; Shay Bujanover

    2003-01-01

    Numerous studies have shown the pathological influence anti-phospholipid antibodies (APLA) have on the physiology of the single neuron as well as the function of the entire human nervous system. The influence is well demonstrated in the antiphospholipid syndrome (APS). This syndrome is characterized by a triad of arterial or venous thrombotic events, recurrent fetal loss and thrombocytopenic purpura. The syndrome exhibits different neurological pathologies such as: chorea, seizures, transvers...

  20. Dapsone Induced Methaemoglobinemia: Early Intraoperative Detection by Pulse Oximeter Desaturation

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    Mahmood Rafiq

    2008-01-01

    Case signifies the importance of knowledge of any preoperative drug intake and its anaesthetic implications. Also patients on dapsone therapy especially children should be monitored for methaemoglobin levels. Since children with immune thrombocy-topenic purpura are being treated with dapsone these days and many of these patients would be planned for splenectomy, monitoring of preoperative methaemoglobin levels and methaemoglobinemia as a cause of intraoperative pulse oximeter desaturation should be kept in mind.

  1. New Diagnostic Strategies for Detection of Helicobacter pylori Infection in Pediatric Patients

    OpenAIRE

    Gold, Benjamin D; Gilger, Mark A.; Steven J Czinn

    2014-01-01

    Helicobacter pylori (H pylori) is a common chronic bacterial infection that is an important cause of peptic ulcer disease and gastroduodenal disease in children. H pylori is also associated with extragastric manifestations, including growth reduction, iron-deficiency anemia, and idiopathic thrombocytopenic purpura. Current guidelines recommend endoscopy with biopsy for the definitive demonstration of H pylori infection. In contrast to serology, the fecal antigen test and the urea breath test ...

  2. Thrombotic Microangiopathy with Skin Localization Secondary to Cytarabine-Daunorubicin Association: Report of a Case

    Directory of Open Access Journals (Sweden)

    S. Regragui

    2012-01-01

    Full Text Available The thrombotic microangiopathy is a syndrome characterized by the combination of mechanical hemolytic anemia, peripheral thrombocytopenia, and organ failure of variable severity. In addition to the idiopathic form, several cases are identified as secondary to pregnancy, infections, disease systems, organ transplants, and cancer. Other forms are secondary to drugs including antimitotics. We report the case of a patient followed for acute myelogenous leukemia. She received induction chemotherapy combining daunorubicin and cytarabine, complicated by thrombotic thrombocytopenic purpura.

  3. Intraveous gammaglobulin in systemic lupus erythematosus

    Directory of Open Access Journals (Sweden)

    Badillo Tenorio Rocio Elizabeth

    2014-07-01

    Full Text Available Systemic lupus erythematosus (SLE is a chronic autoinmune multisystemic disease. Intravenous immunoglobulins (IVIg have been previously used for the treatment of primary immunodefi- ciency diseases. In addition IVIg is used for a few autoimmune diseases, such as immune thrombocytopenic purpura. IVIg is an immunomodulatory agent capable of modulating autoinmu- nity and also provide defense against infection, IVIg has been used successfully in SLE. We review the role of IVIg in SLE as a therapeutic option.

  4. Effect of Leflunomide on the Urine Protein and Urine Red Blood Cell of Patients with Allergic Purpura%来氟米特对过敏性紫癜肾型患者尿蛋白及尿红细胞的影响

    Institute of Scientific and Technical Information of China (English)

    李跃; 刘喜

    2015-01-01

    目的 观察来氟米特对过敏性紫癜肾型(亦称肾型紫癜)患者尿蛋白及尿红细胞的影响.方法 将62例确诊肾型紫癜患者随机分为治疗组33例,对照组29例,治疗组在抗过敏、止血、保肾治疗基础上加服来氟米特治疗,对照组仅给予抗过敏、止血、保肾治疗,观察2组患者治疗前后尿蛋白定量、尿红细胞计数差异.结果 治疗组较对照组在治疗后3月尿蛋白水平明显降低,尿红细胞计数明显减少,差异均具有统计学意义(P0.05).结论 来氟米特用于治疗肾型紫癜具有很好的降低尿蛋白和尿红细胞的作用,疗效明显且不良反应小,对于机体的免疫调节作用明显,将其用于临床是安全和有效的.%Objective To observe the effect of leflunomide on the urine protein and urine red blood cell of patients with allergic purpura. Method The 62 patients with allergic purpura were divided randomly into two groups, one with 33 cases, the other one 29 cas-es. Treatment group were given leflunomide besides anti allergy and hemostasis and renal therapy while control group were not given leflunomide. The amount of urine protein and urine red blood cell before and after treatment were observed. Result the level of urine protein and number of urine red blood cell of control group decreased after three months'treatment. The difference has statistical meaning (P0.05). Conclusion Leflunomide has good effect in reducing the urine protein and urine red blood cell of pa-tients with allergic purpura, with obvious curative effect and little untoward effect, good impact on body 's immune regulation, which is safe and effective in treatment.

  5. Envolvimento renal na púrpura de Henoch-Schönlein: uma análise multivariada de fatores prognósticos iniciais Renal involvement in Henoch-Schönlein purpura: a multivariate analysis of initial prognostic factors

    Directory of Open Access Journals (Sweden)

    José Luiz J. de Almeida

    2007-06-01

    Full Text Available OBJETIVOS: Identificar fatores preditivos iniciais de envolvimento renal nas crianças e adolescentes com púrpura de Henoch-Schönlein. MÉTODOS: Por um período de 21 anos, os prontuários de 142 pacientes com diagnóstico de púrpura de Henoch-Schönlein admitidos em nosso Hospital Universitário foram revistos. Os fatores preditivos iniciais avaliados nos primeiros 3 meses incluíram: dados demográficos, manifestações clínicas (púrpura palpável persistente, artrite, dor abdominal, dor abdominal intensa, sangramento gastrointestinal, orquite, envolvimento do sistema nervoso central e hemorragia pulmonar, exames laboratoriais (níveis séricos de IgA e tratamento utilizado (corticosteróides, imunoglobulina endovenosa e medicação imunossupressora. Os pacientes foram divididos em dois grupos (com presença ou ausência de nefrite e avaliados de acordo com a análise univariada e multivariada. RESULTADOS: Nefrite foi evidenciada em 70 pacientes (49,3%. A análise univariada revelou que dor abdominal intensa (p = 0,0049; OR = 1,6; IC95% 1,18-2,21, sangramento gastrointestinal (p = 0,004; OR = 1,6; IC95% 1,10-2,26 e uso dos corticosteróides (p = 0,0012; OR = 1,7; IC95% 1,28-2,40 foram associados com uma maior incidência de envolvimento renal. Na análise multivariada, a regressão logística mostrou que a única variável independente na predição da ocorrência de nefrite foi dor abdominal intensa (p OBJECTIVES: To identify initial predictive factors of renal involvement in children and adolescents with Henoch-Schönlein purpura. METHODS: We reviewed the medical records of 142 patients admitted to our University Hospital over a 21-year period with a diagnosis of Henoch-Schönlein purpura. The initial predictive factors assessed, observed during the first 3 months, included: demographic data, clinical manifestations (persistent palpable purpura, arthritis, abdominal pain, severe abdominal pain, gastrointestinal bleeding, orchitis

  6. 紫癜性肾炎患儿医院感染相关因素分析及护理%Related factors of nosocomial infections in children with Henoch-Schonlein purpura nephritis and the nursing countermeasures

    Institute of Scientific and Technical Information of China (English)

    王妍炜

    2013-01-01

    目的 分析紫癜性肾炎患儿医院感染的特点及相关因素,以制定相应对策,从而有效降低医院感染发生率.方法 回顾性分析2011年1月-2012年2月儿科住院的178例紫癜性肾炎患儿的临床资料.结果 178例患儿发生医院感染37例,感染率为20.8%;感染部位前3他为呼吸道、泌尿道和皮肤软组织,呼吸道感染最高,占40.5%;检出病原菌以革兰阴性杆菌为主占68.4%;侵入性操作、激素和免疫抑制剂的使用、住院时间与医院感染的关系密切.结论 重点关注病情严重、病程长、免疫力低下、肾脏穿刺的患儿,加强病房管理和环境监测,建立侵入性操作的护理规范,严格执行无菌操作,加强洗手消毒,做好健康宣教,防止交叉感染等均是预防医院感染的关键.%OBJECTIVE To analyze the characteristics and related factors of the nosocomial infections in the children with Henoch-Schonlein purpura nephritis (HSPN) so as to put forward the corresponding countermeasures to reduce the incidence of the nosocomial infections.METHODS The clinical data of 178 cases of HSPN chilren who hospitalized in the pediatrics department from Jan 2011 to Feb2012 were retrospectively analyzed.RESULTS of totally 178 cases of children investigated,the nosocomial infections occurred in 37 case-time of patients with the case-time infection rate of 20.8%.The respiratory tract,urinary tract,and the skin soft tissue were the top three infection sites,the cases with the respiratory tract infections accounted for 68.4%.The invasive operation,use of hormones and immunosuppressive agents,and hospitalization duration were closely related to the nosocomial infections.CONCLUSION Paying attention to the children with severe condition,long treatment course,low immunity,and renal biopsy,strengthening the ward management and monitoring of environment to establish the nursing standards of the invasive operation,strictly implementing the aseptic

  7. Testing results and analysis of 218 patients with Henoch Schonlein purpura in children with food intolerance%218例过敏性紫癜患儿食物不耐受检测结果及分析

    Institute of Scientific and Technical Information of China (English)

    高健

    2016-01-01

    Objective:To understand about the food allergies of children with HSP by the detection of Henoch Schonlein purpura (HSP) in children with food allergen IgG level in serum,then guiding the HSP children's diet. Method:218 cases of children with HSP were selected for food allergen specific IgG (sIgG) detection.The food allergen specific IgG antibody levels are divided into 4 levels:negative:(IgG0.10~0.20U/L,+++),severe sensitive(IgG>0.20U/L).Results:①There were 181 patients with food intolerance in the 218 cases of HSP;②Animal protein allergenicity was significantly higher than that of plant source;③The children of HSP who had food allergy were absolute fasting corresponding sensitive food in three months,then we found the level of IgG about food allergens and secondary were obviously decreased.Conclusion:Food intolerance IgG levels are higher in children with HSP.Through the detection of food allergens in children with HSP IgG level,it can guide the diet to reduce food borne allergies.%目的:通过检测过敏性紫癜(HSP)患儿血清中食物过敏原IgG(sIgG)水平,了解HSP患儿食物过敏的情况,进而指导HSP患儿的饮食及治疗。方法:选取某医院HSP患儿共218例,进行食物变应原特异性IgG(sIgG)检测,将sIgG水平分为4级:阴性:IgG<0.05U/L,+(轻度敏感):IgG0.05~0.10U/L,++(中度敏感):IgG>0.10~0.20U/L,+++(重度敏感):IgG>0.20U/L。结果:①218例中181例阳性;②动物源蛋白致敏性明显高于植物源;③要求患儿绝对禁食对应敏感食物3月后复测食物IgG水平,可发现sIgG阳性率明显降低。结论:食物不耐受IgG水平在HSP患儿中较高,同时通过检测食物sIgG水平可指导HSP患儿的饮食以达到降低食物源性过敏的目的。

  8. 血栓弹力图在儿童过敏性紫癜中的临床应用研究%Study on the clinical application of thrombelastography in children with Henoch-Schonlein purpura

    Institute of Scientific and Technical Information of China (English)

    郭飞波

    2015-01-01

    目的:应用血栓弹力图(TEG)分析儿童过敏性紫癜(HSP)的凝血状态。方法选取2013年2~7月于同济医院小儿内科住院治疗的 HSP患儿81例,并根据临床表现分为 HSP发作组(43例)和HSP缓解组(38例),另选取41例健康查体儿童为对照组,采用TEG仪分别测定凝血反应时间(R)、凝血形成时间(K)、α角、最大振幅(MA)、血凝块溶解百分比(EPL)、30 min血凝块幅度减少速率(Ly30)和凝血指数(CI),并进行比较分析。结果与对照组比较,HSP发作组R值和K值降低,α角、MA值、CI、EPL和Ly30均增加,差异均有统计学意义(P<0.05);与 HSP发作组比较,HSP缓解组患儿R值和K值增高,α角、MA值、CI、EPL及Ly30均减小,差异均有统计学意义(P<0.05)。结论 HSP患儿血液凝固性存在异常,并随着病情缓解而改变, TEG对HSP患儿血凝状态的检测具有重要的参考价值。%Objective To analyse coagulation status in children with Henoch‐Schonlein purpura(HSP) by using thrombelastog‐raphy(TEG) .Methods A total of 81 cases of children hospitalized in Tongji Hospital were collected and divided into attacking stage of HSP group(43 cases) and remitting stage of HSP group(38 cases) .Another 41 cases of children receiving physical exami‐nation were collected as control group .The clotting reaction time(R) ,clot formation time(K) ,α‐angle ,maximum amplitude(MA) , percentage of blood clot dissolution(EPL) ,the rate of 30 minutes clot amplitude reduce(Ly30) and coagulation index(CI) were de‐tected by using TEG instrument .Results Compared with the control group ,the R and K values were decreased ,while theα‐angle , MA value ,CI ,EPL and Ly30 were increased in the attacking stage of HSP group ,there were statistically significant differences(P<0 .05) .Compared with the attacking stage of HSP group ,the R and K values were increased ,while theα‐angle ,MA

  9. 孟鲁司特钠治疗小儿过敏性紫癜疗效观察%Clinical Effect of Montelukast Soudium in Treating Children with Henoch-Sch(o)nlein Purpura

    Institute of Scientific and Technical Information of China (English)

    赵素香

    2013-01-01

    目的:探讨孟鲁司特钠治疗小儿过敏性紫癜(HSP)的临床疗效.方法:96例HsP患儿随机分为治疗组和对照组各48例.两组患儿均予抗组胺药物、钙剂、维生素C治疗,并停止服用可能引起过敏的食物、药物,避免接触可疑过敏物,有感染征象的给予抗生素治疗,消化道出血者卧床休息,暂禁食,给予西咪替丁及胃粘膜保护剂,腹痛、关节肿痛者加用激素,治疗组在此基础上给予孟鲁司特钠咀嚼片口服,<10岁患儿每次5 mg,≥10岁患儿每次10 mg,每晓睡前服1次,疗程1个月.观察比较两组疗效.结果:治疗组总有效率95.8%(46/48),对照组总有效率85.4% (41/48),治疗组高于对照组,但两组总有效率比较差异无统计学意义(x2=3.065,P>0.05);治疗组皮肤紫癜、消化道病变、关节肿痛、肾脏病变消失时间等方面均比对照组明显缩短,两组比较差异均有统计学意义(P<0.05).结论:孟鲁司特钠治疗小儿HSP,疗效确切,能较快缓解症状、缩短病程,未发现明显不良反应,值得临床推广使用.%Objective: To explore the clinical effect of montelukast soudium in treating children with Henoch-Schonlein Puipura ( HSP). Methods: Ninety six cases of children with HSP were randomly divided into a treatment group and a control group equally. Patients in the two groups were given anlihistamine drugs, calcium, vitamin C, and antibiotic treatment to those with signs of infection. All patients stopped taking food and medications that may cause allergy, and avoided contacting with suspicious allergens. Patients with gastrointestinal bleeding received cimetidine and mucosa protective agent, and had bed rest and fasting. Patients with abdominal pain, joint swelling and pain were given glucocorticoids. Based on the comprehensive therapy, the treatment group was given montelukast soudium chewing tablets, 5 mg/day for 0. 05). The durations of cutaneous purpura, gastrointestinal lesions, joint

  10. Fireogfyrre svangerskaber med idiopatisk trombocytopenisk purpura

    DEFF Research Database (Denmark)

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

    2002-01-01

    three days after delivery (p newborn infants had thrombocytopenia in cord blood. The following risk factors for perinatal thrombocytopenia were found: a sibling...... with thrombocytopenia, severe maternal thrombocytopenia, male gender. The nadir platelet count in the newborn infants was seen up to seven days after delivery. The presence of an older sibling with neonatal ITP is a risk factor for neonatal ITP in subsequent pregnancies. A significant association was found between...

  11. HENOCH - SCHONLEIN PURPURA : A TYPICAL PRESENTATION

    Directory of Open Access Journals (Sweden)

    Mohammed Abrar

    2015-07-01

    Full Text Available Henoch - Schonlein P urpura (HSP is the most common vasculitis of childhood and is characterized by leukocytoclastic vasculitis and immunoglobulin (Ig A deposition in the small vessels in the skin, joints, gastrointestinal tract, and kidney. It is a systemic disease where antigen - antibody (IgA complexes activate the alternate complement pathway, resulting in inflammation and small ve ssel vasculitis. Here we are reporting an interesting case of an adolescent female with atypical presentation (hypovolemic shock, erosive gastritis, skin involvement of the extremities requiring very high degree of suspicion for the diagnosis.

  12. 甲基泼尼松龙联合肝素治疗儿童重型腹型紫癜的临床观察%Clinical observation of about heavy abdomen type of anaphylactoid purpura treated hy methylprednisolone associate with heparin

    Institute of Scientific and Technical Information of China (English)

    李琪; 戚勤; 徐静

    2008-01-01

    Objective To observe the curative effect about heavy abdomen type of anaphylactoid pur-pura treated by Methylprednisolone associate with Heparin. Methods Randomly divided eighty three children with heavy abdomen type of anaphylactoid purpura who treated in department of pediatrics of the first hospital affiliated to Kunming medical college from January, 2006 to december, 2007 into group A (treated by Hydro-cortisone) and group B (treated by Methylprednisolone) and group C(treated by Methylprednisolone associate with Heparin). Group A treated by mainline Hydrocortisone dose of 5~8 mg/kg body weight once a day,mixed into 100ml 5% glucose injection, after 7~14 days later, instead of take orally Predisone tablet and de-grees dose by gradually. Group B treated by mainline Methylprednisolone dose of 2~4 mg/kg body weight once a day, mixed into 100ml 5% glucose injection, after 7~14 days later, instead of take orally Predisone tablet, and group C treated by Methylprednisolone by oneself (dose like group B) associate with mainline Hep-arin (dose of 0.25~0.75 mg/kg body weight once a day, mixed into 100ml 5% glucose injection) 7~10 days. Observe clinic symptomatic like as tetter, bellya