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免疫性血小板减少症的病理生理学与治疗的最新进展

[Recent advances in pathophysiology and treatment of immune thrombocytopenia].

作者信息

Kashiwagi Hirokazu, Tomiyama Yoshiaki

机构信息

Department of Hematology and Oncology, Graduate School of Medicine, Osaka University.

出版信息

Rinsho Ketsueki. 2015 Feb;56(2):177-84. doi: 10.11406/rinketsu.56.177.

Abstract

Immune thrombocytopenia (ITP) is an autoimmune disorder characterized by isolated thrombocytopenia caused by immune-mediated platelet destruction and impairment of platelet production. Recent studies have uncovered details involving the target regions of platelet-associated anti-GPIIb/IIIa antibodies, pathological differences depending on the specificity of target antigens, and cellular abnormalities, especially impairment of regulatory T cells contributing to the pathogenesis of ITP. Treatment of ITP has been changed dramatically by the application of thrombopoietin receptor agonists, TPO-RAs, in patients unresponsive to traditional steroids and splenectomy. Rituximab has also been used in Western countries for ITP patients and its long-term efficacy has become increasingly clear. Clinical problems awaiting solution in ITP management include improving the efficacy of treatments for newly-diagnosed ITP, confirmation of the long-term efficacy and safety of TPO-RAs, and determination of the positioning of rituximab in the treatment sequence of ITP.

摘要

免疫性血小板减少症(ITP)是一种自身免疫性疾病,其特征为免疫介导的血小板破坏和血小板生成受损导致的孤立性血小板减少。最近的研究揭示了涉及血小板相关抗GPIIb/IIIa抗体的靶区域、取决于靶抗原特异性的病理差异以及细胞异常等细节,尤其是调节性T细胞功能受损在ITP发病机制中的作用。血小板生成素受体激动剂(TPO-RAs)应用于对传统类固醇和脾切除术无反应的患者后,ITP的治疗发生了巨大变化。利妥昔单抗在西方国家也已用于ITP患者,其长期疗效日益明确。ITP管理中有待解决的临床问题包括提高新诊断ITP的治疗效果、确认TPO-RAs的长期疗效和安全性以及确定利妥昔单抗在ITP治疗序列中的定位。

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