造血干细胞移植相关的血栓性微血管病的诊断和治疗  

Diagnosis and treatment of transplant-associated thrombotic microangiopathy

作　　者：陈峰[1] 吴德沛[1] CHEN Feng;WU De-pei(National Clinical Research Center for Hematologic Diseases,Jiangsu Institute of Hematology,the First Affliated Hospital of Soochow University,Key Laboratory of Thrombosis and Hemostasis of Ministry of Health,State Key Laboratory of Radiation Medicine and Protection,Suzhou 215123,China)

机构地区：[1]国家血液系统疾病临床医学研究中心、江苏省血液研究所、苏州大学附属第一医院、国家卫生健康委员会血栓与止血重点实验室、放射医学与辐射防护国家重点实验室,江苏苏州215123

出　　处：《中国实用内科杂志》2023年第6期453-456,共4页Chinese Journal of Practical Internal Medicine

基　　金：国家重点研发计划(2022YFC2502700);国家自然科学基金(82020108003);江苏省科教能力提升工程(CXZX202201);苏州市科技计划项目(SLT201911)。

摘　　要：造血干细胞移植相关的血栓性微血管病(TA-TMA)是一种移植后严重并发症,发病机制的核心是内皮损伤和补体异常激活。依据临床表现、实验室指标及组织学可作出综合判断。治疗上强调去除病因作为一线治疗,效果不佳时,个体化选择抗C5单克隆抗体(简称单抗)、去纤苷、抗CD20单抗、血浆置换等为主的二线治疗。Transplant-associated thrombotic microangiopathy(TA-TMA)is a serious complication after hematopoietic stem cell transplantation.Endothelial injury and abnormal complement activation are the core of its pathogenesis.A comprehensive diagnosis can be made based on clinical findings,laboratory indicators and histology.In the treatment,the removal of possible causes is emphasized as the first-line treatment,and when the therapeutic efficacy is not ideal,individualized second-line treatment should be selected based on anti-C5 monoclonal antibody,defibrin,anti-CD20 monoclonal antibody and plasma exchange.

关 键 词：造血干细胞移植相关的血栓性微血管病 血栓性微血管病 诊断 治疗 

分 类 号：R543[医药卫生—心血管疾病]