关于免疫性血小板减少性紫癜的管理和新出现的治疗方案的最新进展:综述和病例报告

R. Krishnan, P. MohammedFaisalK, S. MerlinT, Cijo Oommen
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引用次数: 2

摘要

免疫性血小板减少性紫癜影响儿童和成人。它是一种自身免疫性疾病,其特征是持续的血小板减少(外周血小板计数小于150 × 10 9 /L),原因是自身抗体与血小板抗原结合,导致血小板抗原被网状内皮系统(特别是脾脏)过早破坏。没有金标准诊断测试来确认ITP。ITP的诊断仍然是临床诊断,主要基于病史、体格检查、全血细胞计数、外周血膜和自身免疫筛查排除血小板减少症的其他原因。一线药物治疗的医疗选择是皮质类固醇、静脉注射免疫球蛋白和静脉注射Rh - d。二线和三线治疗包括单克隆抗体和血小板生成素受体激动剂。如果发生危及生命的出血,输血血小板是必要的。本文就免疫性血小板减少性紫癜的病理生理、临床表现、诊断和治疗作一综述,并附1例报告。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
An update on the management of immune thrombocytopenic purpura and emerging treatment options: A review and case report
Immune thrombocytopenic purpura affects both children and adults. It is an autoimmune disorder characterised by persistent thrombocytopenia (peripheral platelet count of less than 150 x10 9 /L) due to autoantibody binding to platelet antigen(s) causing their premature destruction by the reticulo-endothelial system, in particular the spleen. There is no gold standard diagnostic test to confirm ITP. The diagnosis of ITP remains clinical and is based principally on the exclusion of other causes of thrombocytopenia by the history, physical examination, full blood count, peripheral blood film and autoimmune screen. Medical options for front-line drug therapy are corticosteroids, intravenous immunoglobulin, and intravenous Rh anti-D. Second and third line therapy includes monoclonal antibodies and thrombopoirtin receptor agonist. Transfusion of platelet is warranted if life threatening hemorrhage occurs. This review gives a brief discussion on the pathophysiology, clinical presentation, diagnosis and treatment of Immune Thrombocutopenic Purpura and a case report.
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