ADAMTS13在TTP发病机制、诊断及治疗中作用的研究进展  

Research progress of ADAMTS13 in the pathogenesis,diagnosis and treatment of TTP

作　　者：杨赛男[1] 万佳[1] 陈洁平[1] 张扬[1] 裴莉[1] 

机构地区：[1]第三军医大学附属西南医院血液科,重庆400038

出　　处：《临床血液学杂志》2014年第3期434-436,共3页Journal of Clinical Hematology

摘　　要：血栓性血小板减少性紫癜（thrombotic thrombocytopenic purpura,TTP）是一种以微血管性溶血性贫血,血小板减少性紫癜,神经系统异常,伴有不同程度的肾脏损害及发热为主要临床表现的严重的血栓性微血管病。TTP的病理生理学机制主要是血小板减少及微小动脉和毛细血管形成血栓;且起病急聚,病情险恶,既往的总死亡率超过90%。1976年Bukowski、Regulatory disorder of vWF multimer size is a central pathophysiological mechanism of thrombotic thrombocytopenic purpura（TTP）.As the key regulator of vWF size,ADAMTS13is seriously reduced in most of TTP patients.Recent researches show that severe ADAMTS13deficiency could be divided into acquired ADAMTS13deficiency and congenital ADAMTS13deficiency.The acquired ADAMTS13deficiency is always caused by auto-antibodies inhibiting the activity of ADAMTS13or increasing the degradation of ADAMTS13in the blood.Congenital ADAMTS13deficiency is due to the severe genic mutation of ADAMTS13.In conclusion,studying the role of ADAMTS13in the pathogenesis of TTP will help to diagnose and treat this disease.This paper will review the role of ADAMTS13in the pathogenesis,diagnosis and treatment of TTP in recent years.

关 键 词：血栓性血小板减少性紫癜 获得性ADAMTS13缺失 遗传性ADAMTS13缺失 超大分子量血管性血友病因子 

分 类 号：R558.2[医药卫生—血液循环系统疾病]