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[免疫性血小板减少性紫癜发病机制及其临床治疗研究进展——综述]

[Recent progress of study on mechanism of immune thrombocytopenic purpura and its clinical treatment - review].

作者信息

Lu Xue-Chun, Zhu Hong-Li, Yao Shan-Qian

机构信息

Department of Geriatric Hematology, PLA General Hospital, Beijing 100853, China.

出版信息

Zhongguo Shi Yan Xue Ye Xue Za Zhi. 2008 Oct;16(5):1232-6.

Abstract

Immune thrombocytopenia purpura (ITP) is a disorder mediated by antiplatelet antibodies and characterized by accelerated destruction of platelets and impaired platelet production. The mainstay therapies for ITP have included corticosteroids, the immune globulin intravenous immunoglobulin and IV anti-D (monoclonal antibodies against the D antigen of the Rh system), vinblastine or a monoclonal anti-CD20 antibody that transiently depletes CD20(+) B cells, danazol, cyclophosphamide and even splenectomy to refractory one. Most of ITP patients responded to those treatment, while more than 30% of whom may relapse sooner or later. The recombinant forms of human TPO were discontinued from human use in clinic because recipients of these agents developed significant thrombocytopenia secondary to production of neutralizing antibodies that cross-reacted with endogenous TPO. All above mentioned treatments have side effects and severe infection may arise post splenectomy. The more powerful treatment with less side effects are needed. There are two TPO receptor agonist, AMG531 and Eltrompobag, have approved in Europe for the treatment of ITP. Both of them can improve the differentiation of megakaryocyte and platelets production. Combination treatment including pancytoprotector shows good effect in the treatment of refractory and relapsed ITP in China. Altogether, individual treatment of ITP is the contemporary trend in both clinical and preclinical practice. In this review the pathogenesis of ITP and its clinical therapies were reviewed, the individual regiments for treating ITP patients were discussed.

摘要

免疫性血小板减少性紫癜(ITP)是一种由抗血小板抗体介导的疾病,其特征为血小板加速破坏和血小板生成受损。ITP的主要治疗方法包括皮质类固醇、静脉注射免疫球蛋白和IV抗-D(针对Rh系统D抗原的单克隆抗体)、长春碱或一种可短暂清除CD20(+) B细胞的单克隆抗CD20抗体、达那唑、环磷酰胺,甚至对于难治性患者进行脾切除术。大多数ITP患者对这些治疗有反应,但其中超过30%的患者迟早可能复发。重组形式的人血小板生成素(TPO)已停止在临床上供人使用,因为这些药物的接受者会因产生与内源性TPO发生交叉反应的中和抗体而出现严重血小板减少。上述所有治疗都有副作用,脾切除术后可能会发生严重感染。需要更有效且副作用更小的治疗方法。有两种TPO受体激动剂,AMG531和艾曲泊帕乙醇胺片,已在欧洲获批用于治疗ITP。它们都可以改善巨核细胞的分化和血小板生成。在中国,包括全血细胞保护剂在内的联合治疗在难治性和复发性ITP的治疗中显示出良好效果。总之,ITP的个体化治疗是临床和临床前实践的当代趋势。在本综述中,对ITP的发病机制及其临床治疗进行了综述,并讨论了治疗ITP患者的个体化方案。

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