妊娠合并血栓性血小板减少性紫癜的研究进展

The Current Research of Thrombotic Thrombocytopenic Purpura in Pregnancy

血栓性血小板减少性紫癜(TTP)是罕见的血液科致死性疾病,围生期更为罕见。临床常表现为三联征(微血管性溶血性贫血、进行性血小板减少、神经系统异常)或五联征(三联征加发热及肾脏损害)。TTP发病机制不清,可能为遗传性血管性血友病因子裂解酶缺失,或妊娠期血管性血友病因子裂解酶分泌增加所致。妊娠为其发作的独立诱因,母儿预后不良,围生期规范使用血浆置换、血浆输注等综合治疗,适时终止妊娠,可改善预后。

Thrombotic thrombocytopenic purpura （TTP） is a rare life-threatening disorder. It is characterized by microangiopathic hemolytic anemia,progressive thrombocytopenia,central nervous system disorder,fever and acute renal failure. It is caused by the absent or severe deficiency of the von Willebrand factor-cleaving protease named ADAMTS13. The pathogenesis of TTP is unknown,usually triggered after pregnancy. It causes maternal morbidity and fetal loss. Prompt aggressive management should include timely termination of pregnancy,plasma transfusion and/or plasma exchange.