血栓性血小板减少性紫癜——附4例报告及文献复习

Thrombotic Thrombocytopenic Purpura Report of 4 Case and Review of the Literature

目的:探讨血栓性血小板减少性紫癜(TTP)的诊断、治疗及发病机理。方法:用血浆置换治疗4例TTP,同时辅以皮质激素等治疗。结果:4例病人均获得缓解,其中1例再发后仍获缓解。结论:TTP是一种起病急且凶险的疾病,临床分为原发性及继发性两类。继发性者多与妊娠、感染、某些药物、转移性肿瘤、骨髓移植等因素有关。其发病机制与内皮细胞损伤、血管性血友病因子(von willebrand factor,vWF)在血中释放增加及其在血清中裂解障碍、vWF异常大分子多聚体(ULvWF)形成有关。血浆置换是目前治疗本病的首选有效方法。

Objective: To explore the diagnosis, the ideal treatment and the mechanisms of thrombotic thrombocytopenic purpura(TTP). Methods: To treat four patients with plasma exchange(PE). Results: The clinical manifestation become lysis in all the four cases. Conclusion: TTP is a kind of acute and fatal diseases. And clinically divided into primary and secondary TTP. Secondary TTP is associated with pregnancy, infection, drug, metastatic cancer, and bone marrow transplantation et al. The mechanisms of TTP may be relating to the injury of mierorascular endothelia cell, the increase of von willebrand factor and the forming of unusual large von willebrand factor multimer(ULvWF) in the blood. Plasma exchange(PE) is the first selected and most effective therapy for patients with TTP at present.