血管性血友病因子裂解蛋白酶与血栓性微血管病的研究进展

Research progress of ADAMTS13 and thrombotic microangiopathy

血栓性微血管病(Thrombotic microangiopathy,TMA)是一组以非免疫性血管内溶血和缺血性器官功能障碍为特征的疾病,包括原发性血栓性微血管病[如血栓性血小板减少性紫癜(Thrombotic thrombocytopenic purpura,TTP)等]和继发性血栓性微血管病[如弥散性血管内凝血(Disseminated intravascular coagulation,DIC)等]。目前血管性血友病因子裂解蛋白酶(A disintegrin and metalloprotease with a thrombospondin type 1 motif member 13,ADAMTS13)在TTP诊疗中的作用相对明确,但是否在其他血栓性微血管病的诊疗中存在同样重要的价值并不清楚,ADAMTS13作用于相关血栓性微血管病的具体机制较为复杂。ADAMTS13在DIC、系统性红斑狼疮肾炎以及严重肝损伤等相关血栓性微血管病中活性均有不同程度的降低,或许可作为该类疾病的辅助诊断指标。本文主要从ADAMTS13与血栓性微血管病的发病机制及其在相关血栓性微血管病诊疗中的应用价值进行综述。

Thrombotic microangiopathy(TMA)is a group of diseases characterized by non-immune intravascular hemolysis and ischemic organ dysfunction,including primary thrombotic microangiopathy(such as thrombotic thrombocytopenic purpura,TTP etc.)and secondary thrombotic microangiopathy(such as disseminated intravascular coagulation,DIC,etc.).Currently,a disintegrin and metalloprotease with a thrombospondin type 1 motif member 13(ADAMTS13)in the diagnosis and treatment of TTP is relatively clear,but whether it has the same importance in the diagnosis and treatment of other thrombotic microangiopathy is not clear.The mechanism by which ADAMTS13 acts on related thrombotic microangiopathy is complex and unclear.However,ADAMTS13 showed decreased activity in DIC、systemic lupus erythematosus nephritis,severe liver injury related thrombotic microangiopathy,which may be used as an auxiliary diagnostic indicator for this disease.This paper mainly reviews the pathogenesis of ADAMTS13 and thrombotic microangiopathy and its application value in the diagnosis and treatment of related thrombotic microangiopathy.