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  

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

作　　者：王雅丹[1] 魏文宁[1] 

机构地区：[1]华中科技大学同济医学院附属协和医院血液病研究所,武汉430022

出　　处：《血栓与止血学》2005年第5期200-202,共3页Chinese Journal of Thrombosis and Hemostasis

摘　　要：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。Objective In order to improve the knowledge of mechanism and therapy in thrombotic thrombocytopenic purpura （TIP） ,we reported 2 cases of TIP and analysis the activity of von Willebrand factor-cleaving protease （vWF-CP）. Methods 2 cases of TTP were reported and a review of literature was given. Moreover,vWF-CP activity in patients with TIP beware 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 decrease of vWF-CP activity was the primary mechanism of TIP. Plasmapheresis prior to plasma infusion in TTP treatment.

关 键 词：血栓性血小板减少性紫癜 血管性血友病因子裂解酶 

分 类 号：R554.6[医药卫生—血液循环系统疾病]