血栓性血小板减少性紫癜(TTP)是一种临床急重症,以广泛微血管血栓形成和血小板减少为病理特征。典型的临床表现为微血管病性溶血性贫血、血小板减少、神经精神异常、发热及肾脏损害。本病发病急骤病情凶险,如不及时治疗则病死率可达90%。近年来随着血浆置换疗法的临床应用,TTP急性发作期治疗效果虽有明显改观,但仍然是临床上的急重症,并存在易反复发作等难以解决等问题。 Thrombotic thrombocytopenic purpura (TTP) is a clinical critically ill patient with extensive microvascular thrombosis and thrombocytopenia as pathological features. Typical clinical manifestations of microangiopathic hemolytic anemia, thrombocytopenia, neuropsychiatric disorders, fever and kidney damage. The sudden onset of the disease is dangerous, if not treated, the case fatality rate of up to 90%. In recent years, with the clinical application of plasma exchange therapy, although the treatment effect of acute exacerbation of TTP significantly improved, but it is still clinically critical illness, and there is easy to solve such issues as recurrent episodes.