Fondazione Istituto di Ricovero e Cura a Carattere Scientifico (IRCCS) Ca' Granda Ospedale Maggiore Policlinico, Angelo Bianchi Bonomi Hemophilia and Thrombosis Center, Milan, Italy; and.
Department of Pathophysiology and Transplantation, Università degli Studi di Milano, Milan, Italy.
Blood. 2020 Nov 5;136(19):2125-2132. doi: 10.1182/blood.2019000962.
Thrombotic thrombocytopenic purpura (TTP) is an acute, life-threatening thrombotic microangiopathy (TMA) caused by acquired or congenital severe deficiency of ADAMTS13. Pregnancy is a recognized risk factor for precipitating acute (first or recurrent) episodes of TTP. Differential diagnosis with other TMAs is particularly difficult when the first TTP event occurs during pregnancy; a high index of suspicion and prompt recognition of TTP are essential for achieving a good maternal and fetal outcome. An accurate distinction between congenital and acquired cases of pregnancy-related TTP is mandatory for safe subsequent pregnancy planning. In this article, we summarize the current knowledge on pregnancy-associated TTP and describe how we manage TTP during pregnancy in our clinical practice.
血栓性血小板减少性紫癜（TTP）是一种由获得性或先天性严重 ADAMTS13 缺乏引起的急性、危及生命的血栓性微血管病（TMA）。妊娠是促发急性（首发或复发）TTP 发作的已知危险因素。当首次 TTP 事件发生在妊娠期间时，与其他 TMA 的鉴别诊断特别困难；高度怀疑和及时识别 TTP 对于实现良好的母婴结局至关重要。准确区分妊娠相关性 TTP 的先天性和获得性病例对于安全的后续妊娠规划是必需的。本文总结了与妊娠相关 TTP 的现有知识，并描述了我们在临床实践中如何管理妊娠期间的 TTP。
