血栓性血小板减少性紫癜患者血管性血友病因子裂解酶活性的分析

目的通过对2例血栓性血小板减少性紫癜（TTP）患者诊疗经过的回顾及对血管性血友病因子裂解酶（vWF-CP）活性研究,以提高对TTP发病机制及治疗的认识.方法报道2例TTP患者的诊疗经过并作文献复习,采用分析蛋白裂解片段法检测2名患者和正常人血浆vWF-CP活性.结果2例TTP患者均痊愈出院.血浆置换治疗前血浆vWF-CP活性明显低于正常人,而治疗后其活性明显上升.结论vWF-CP活性下降或丧失是TTP的主要病因;血浆置换治疗明显优于血浆输注.

Activity of von Willebrand Factor-cleaving Protease in Patient with Thrombotic Thrombocytopenic Purpura

Objective In order to improve the knowledge of mechanism and therapy in thrombotic thrombocytopenic purpura(TTP),we reported 2 cases of TTP and analysis the activity of von Willebrand fac- tor-cleaving protease(vWF-CP).Methods 2 cases of TTP were reported and a review of literature was giv- en.Moreover,vWF-CP activity in patients with TTP before and after plasmapheresis were also investigated. Results Before plasmapheresis,the level of vWF-CP activity in patients with TTP was lower than the health individual.However,it was significantly increased after plasmapheresis.Conclusion The deficiency or de- crease of vWF-CP activity was the primary mechanism of TTP.Plasmapheresis prior to plasma infusion in TTP treatment.