Successful use of rituximab in refractory idiopathic thrombocytopenic purpura in a patient with common variable immunodeficiency.
نویسندگان
چکیده
Idiopathic thrombocytopenic purpura (ITP) is a common autoimmune disease in patients with common variable immunodeficiency (CVID). We describe a 36-year-old woman with CVID. The clinical course of her disease was complicated by bronchiectasis, antiphospholipid antibody syndrome, and portal vein thrombosis. She developed recurrent attacks of ITP refractory to high doses of corticosteroid, intravenous immunoglobulin (IVIG), and splenectomy. She received a total of 5 doses of rituximab (375 mg/m2) and achieved an immediate and persistent response. Therapy was well tolerated. Her platelet count remained above 370,000/microL for 8 months of follow-up, despite repeated infections. During this period the patient remained off corticosteroids and on continuous IVIG replacement therapy.
منابع مشابه
Successful Treatment of Refractory Thrombotic Thrombocytopenic Purpura with Rituximab, a Monoclonal Antibody: a Case Report
Thrombotic thrombocytopenic purpura (TTP) is mostly attributed to the presence of an autoantibody against ADAMTS-13, a metalloprotease that degrades ultralarge von Willebrand protein multimers. Accumulation of vWF multimers and systemic platelet aggregation lead to microangiopathic thrombosis, hemolytic anemia, and end-organ ischemia. Most patients respond to therapeutic plasma exchange (TPE), ...
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Bernard-Soulier syndrome or idiopathic thrombocytopenic purpura: A case series
Background: Bernard-Soulier syndrome (BSS) is a rare, autosomal recessive platelet function disorder which is commonly mistaken for idiopathic thrombocytopenic purpura (ITP).The report includes seven cases of BSS that have been diagnosed and treated as ITP for a long time. Methods: Between 2006 and 2016, data of seven BSS patients who have long been diagnosed and treated as ITP were collected ...
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Background: Rituximab can induce a durable remission in plasma exchange refractory thrombotic thrombocytopenic purpura (TTP). Timing of Rituximab infusion in combination with plasma exchange (PE) and long term follow-up for probable side effects of such treatment is still lacking. Methods: This study was conducted among 10 patients with plasma exchange refractory TTP. According to the study de...
متن کاملResponse of refractory ITP in a patient with common variable immunodeficiency (CVID) to treatment with rituximab
CVID is the commonest serious antibody deficiency in adults. There are a number of associated complications including autoimmunity, of which immune thrombocytopenic purpura (ITP) is a relatively common example. ITP usually responds to treatment with corticosteroids, other immunosuppression, splenectomy or high dose intravenous immunologlobulin (IVIG). The management of ITP refractory to these t...
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ورودعنوان ژورنال:
- Journal of investigational allergology & clinical immunology
دوره 20 3 شماره
صفحات -
تاریخ انتشار 2010