摘要
血栓性血小板减少性紫癜（thrombotic thrombocytopenic purpura,TTP）为一组罕见的微血管血栓出血综合征,临床主要表现为＂五联征＂：微血管病性溶血性贫血、血小板减少、神经精神症状、肾脏受累和发热,其诊疗困难,死亡率高。本文就TTP的发病机制和治疗进展综述如下。1 TTP发病机制TTP分为遗传性和获得性,
Thrombotic thrombocytopenic purpura（TTP）is a rare and life-threatening micro-vascular thrombotic disorder.The activity of the von Wille-brand factor cleaving metalloproteinase ADAMTS13 is associated with TTP.Although plasma exchange has significantly reduced mortality,TTP is easy to relapse.With the study of pathogenesis of TTP,more safe and effective treatment methods are explored.This review will introduce new advances in terms of pathogenesis and therapeutic approaches for the amelioration of TTP in recent years.
出处
《临床血液学杂志》
CAS
2016年第1期73-76,共4页
Journal of Clinical Hematology
关键词
紫癜
血栓性血小板减少
发病机制
治疗
进展
purpura
thrombotic thrombocytopenic
pathogenesis
treatment
progress
