摘要
造血干细胞移植相关血栓性微血管病(TA-TMA)是导致患者造血干细胞移植(HSCT)后病死率较高的并发症之一,其发病由H SCT过程中多种因素所致的小血管内皮损伤所介导.近年研究发现,补体系统在该病发病过程中可能发挥一定作用.关于TA-TMA的诊断,目前的相关实验室诊断标准尚未完善,研究者拟将补体因素纳入TA-TMA的诊断,以期改进对该病的诊断方式.但是病理学诊断仍是目前诊断TA-TMA的金标准.补体系统阻断剂伊库珠单抗,对高危TA-TMA患者可能是一种有效的治疗方案.笔者拟就TA-TMA的发病机制及其诊疗的研究现状进行介绍.
Transplantation associated thrombotic microangiopathy (TA-TMA ) is a significant complication after hematopoietic stem cell transplantation (HSCT),and may lead to a high risk of death.It is a multifactorial disorder focused on small vessel endothelial injury which can be trigged by varieties of mechanisms during HSCT.The complement system is found to play a role in the development of TA-TMA. Current laboratory diagnostic criteria are unsatisfactory and the latest diagnostic criterion includes activation of the complement system.But the pathological diagnosis remains to be the golden standard.Blocking the complement system with eculizumab may be one of the effective treatment for high-risk patients with TA-TMA.This article reviews the status on pathogenesis,diagnosis and treatment of TA-TMA.
作者
刘晓
张晓辉
Liu Xiao;Zhang Xiaohui(Peking University People′s Hospital,Peking University Institute of Hematology,Beijing 100044,China)
出处
《国际输血及血液学杂志》
CAS
2019年第6期461-468,共8页
International Journal of Blood Transfusion and Hematology
基金
国家重点研发计划项目(2017YFA0105503)。
关键词
血栓性微血管病
造血干细胞移植
内皮
血管
补体系统蛋白质类
移植相关血栓性微血管病
内皮损伤
伊库珠单抗
Thrombotic microangiopathies
Hematopoietic stem cell transplantation
Endothelium,vascular
Complement system proteins
Transplantation associated thrombotic microangiopathy
Endothelial injury
Eculizumab
