血栓性血小板减少性紫癜治疗的研究进展  

Research Advance in Treatment of Thrombotic Thrombocytopenic Purpura ——Review

作　　者：王文 何杨[1] WANG Wen;HE Yang(Jiangsu Institute of Hematology,The First Affiliated Hospital of Soochow University,Suzhou 215006,Jiangsu Province,China)

机构地区：[1]苏州大学附属第一医院,江苏省血液研究所,江苏苏州215006

出　　处：《中国实验血液学杂志》2022年第1期314-318,共5页Journal of Experimental Hematology

摘　　要：血栓性血小板减少性紫癜(TTP)是一种血栓性微血管病,患者因严重缺乏血管性血友病因子裂解酶导致大量富血小板血栓堵塞血管,最终引发终末器官衰竭。血浆置换作为TTP治疗的基石,极大程度提高了患者的生存率。随着对TTP病理生理机制的进一步探索,逐渐涌现了其它类型的替代疗法、新型免疫抑制剂、靶向拮抗剂、基因治疗等新兴手段,有望进一步降低该病患者的死亡率和复发率。本文就TTP治疗的最新研究进展作一综述。Thrombotic thrombocytopenic purpura(TTP) is a thrombotic microangiopathy,in which a severe deficiency of von Willebrand factor lyase results in thrombocytopenic clots that block blood vessels and eventually lead to terminal organ failure.Therapeutic plasma exchange is the cornerstone of TTP treatment which can greatly improves the survival rate of the patients.With the further exploration to the pathophysiological mechanism of TTP,other alternative therapies,new immunosuppressive agents,targeted antagonists,gene therapy and other emerging means gradually emerge,which are expected to further reduce the mortality and recurrence rate of the patients.In this review,the new developments in TTP treatment were summarized briefly.

关 键 词：血栓性血小板减少性紫癜 重组ADAMTS13 免疫抑制剂 靶向疗法 基因治疗 

分 类 号：R554.6[医药卫生—血液循环系统疾病]