血栓性血小板减少性紫癜治疗及预后分析

Treatment and prognosis analysis of thrombotic thrombocytopenic purpura

血栓性血小板减少性紫癜(thrombotic thrombocytopenic purpura,TTP)是一种以微小血管形成血栓栓塞进而导致血小板减少为特点的血栓性微血管疾病。目前认为TTP的发病机制与vWF多聚体的特异性蛋白酶缺乏密切相关。TTP的临床表现多样,诊断困难,且病死率极高(可达90%以上)。尽管血浆置换疗法的出现极大程度地降低了TTP的致死率,但该病的复发率仍旧较高。

Thrombotic thrombocytopenic purpura (TTP) is a thrombotic microvascular disease characterized by thromboembolism caused by microvascular formation and thrombocytopenia.It is currently believed that the pathogenesis of TTP is closely related to the lack of specific proteases of vWF multimers.The clinical manifestations of TTP are diverse,the diagnosis is difficult,and the mortality rate is extremely high (up to 90% or more).Although the emergence of plasmapheresis therapy has greatly reduced the mortality rate of TTP,the recurrence rate of the disease is still high.