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Haematology and oncology
Published in Jagdish M. Gupta, John Beveridge, MCQs in Paediatrics, 2020
Jagdish M. Gupta, John Beveridge
9.20. A boy aged 6 years has a history of severe bruising and petechiae of several days' duration. He is not anaemic. There is no enlargement of liver or spleen. Which of the following statements is/are true of this patient?The most likely diagnosis is idiopathic thrombocytopenic purpura.Bone marrow examination will show numerous megakaryocytes.Complete recovery would be expected in 3-8 weeks in more than 70% of cases.Bleeding in joints is a likely complication,Splenectomy is recommended if the disease is still active after 3-4 months.
Infection in the Hematopoeitic Stem Cell Transplant Recipient with Autoimmune Disease
Published in Richard K. Burt, Alberto M. Marmont, Stem Cell Therapy for Autoimmune Disease, 2019
Valentina Stosor, Teresa R. Zembower
Idiopathic thrombocytopenic purpura (ITP) is an immunoregulatory disorder in which antibodies damage platelets leading to their removal by the reticuloendothelial system.45,46 In children, a history of preceding viral illness or live virus immunization has frequently been described.47 Because of the significant association with HIV, eliciting risk factors and testing for HIV in any patient presenting with ITP is warranted.48
Immunopathology
Published in Constantin A. Bona, Francisco A. Bonilla, Textbook of Immunology, 2019
Constantin A. Bona, Francisco A. Bonilla
As mentioned above, other cytopenias may also be the result of autoantibody production. Idiopathic thrombocytopenic purpura (ITP) is caused by anti-platelet autoantibodies. These are directed mainly against the surface glycoprotein heterodimer gpIIb/IIIa (CD41/CD61 β3 integrin, see Chapter 10). Autoimmune neutropenia is caused by antibodies reactive with neutrophil surface antigens.
Prevalence of primary immune thrombocytopenia and related healthcare resource utilization among Texas Medicaid beneficiaries
Published in Current Medical Research and Opinion, 2021
Yi Liang, Karen Rascati, Kristin Richards
Immune thrombocytopenia (ITP), also known as idiopathic thrombocytopenic purpura, is an autoimmune disorder characterized by a low platelet count resulting from excessive platelet destruction or/and impaired platelet production1. A platelet count less than 100 × 109/L in peripheral blood has been established as the threshold for diagnosis of ITP2. Primary ITP occurs in isolation (in absence of other causes or disorders that may be associated with thrombocytopenia); and secondary ITP includes all forms of immune-mediated thrombocytopenia caused by underlying factors, including autoimmune diseases, hepatitis C (HCV) and human immunodeficiency virus (HIV), or drug exposures3. This study focused on primary ITP, as the clinical courses and treatments of the two types of ITP can be very different1.
Recurrent melena in a diagnosed case of Bernard Soulier syndrome
Published in Journal of Community Hospital Internal Medicine Perspectives, 2021
Omair Ali Khan, Sheharyar Raashid, Sohaib Asghar, Ramsha Majeed, Mahnoor Fatima Sherazi, Fakeha Nayyer, Aisha Anis, Zainab Ehsan
Bernard Soulier Syndrome is a frequently misdiagnosed condition. The clinical picture is similar to other platelet disorders and hence many cases have been misdiagnosed as idiopathic thrombocytopenic purpura (ITP), leading to unsuccessful treatment [8]. It is therefore imperative that misdiagnosis be avoided through a thorough history taking with special emphasis on age, the initial presenting complaints and any positive family history. The subsequent lab evaluation must include a general assessment of coagulation parameters followed by platelet function specific testing [9]. The additional specific tests include flowcytometry and platelet aggregation studies. Flowcytometry is the confirmatory test which demonstrates abnormalities of platelet membrane glycoprotein. Care should be taken to perform this test on a plasma sample with abundant platelets [10]. Aggregation studies characteristically show platelets’ inability to aggregate with ristocetin and a slow response with low doses of thrombin. The addition of normal plasma doesn’t help aggregation, thus differentiating BSS from VWD. However, normal aggregation is seen with epinephrine, adenosine diphosphate, collagen, and arachidonic acid [11]. Testing for genetic mutations can differentiate between functional or synthetic problems of the GPIB-IX-V receptor complex, and this approach greatly improves the diagnosis of rare inherited platelet disorders [12].
Tacrolimus is effective in relapsed/refractory autoimmune cytopenias: results of a single-center retrospective study
Published in Hematology, 2020
Yali Du, Chen Yang, Miao Chen, Jing Ruan, Yuzhou Huang, Fangfei Chen, Bing Han
Autoimmune cytopenia is considered a special ‘autoimmune disease’ in which hematologic system manifestations are dominant. In some patients, the condition may be secondary to autoimmune disease, or autoimmune disease can subsequently develop. Tacrolimus has shown some advantages over other immunosuppressive agents, with better reaction rates and fewer side effects for systematic lupus erythematosus, a typical autoimmune disease, indicating that tacrolimus may also work for autoimmune cytopenia [5,16]. Recently, Gergis U [8] reported one patient diagnosed with severe idiopathic thrombocytopenic purpura after allogeneic bone marrow transplantation who was refractory to sufficient corticosteroids and IVIG and had a durable response to tacrolimus treatment. Another report by Tabchi S [9] showed that one patient diagnosed with ES achieved CR on tacrolimus, and this patient had failed with corticosteroids, IVIG, splenectomy, rituximab, cyclophosphamide, mycophenolate mofetil, cyclosporin, vincristine and HSCT for approximately 10 ten years before the initialization of tacrolimus therapy.