最新动态

最新动态

    Successful management of thrombotic thrombocytopenic purpura associated withpregnancy

    发布于：2014年4月30日    文字：【大】【中】【小】

    Transfus Apher Sci. 2014 Mar 12. pii: S1473-0502(14)00068-8. doi: 10.1016/j.transci.2014.02.023. [Epub ahead of print]

    Successful management of thrombotic thrombocytopenic purpura associated withpregnancy.

    U?ur Bilgin A, Karaselek MA, Caml? K.

    Thrombotic thrombocytopenic purpura (TTP) is an uncommon, severe, potentially

    life-threatening disease characterized by microangiopathic hemolytic anemia, thrombocytopenia, altered mental status, fever, and renal abnormalities. It can be seen at any age or sex but affects women of childbearing age more commonly. Pregnancy is known as one of the most common precipitating events for the onset of TTP and occurs mostly in the late third trimester or during the puerperium. Because of relatively low prevalence of pregnancy-related TTP, here we report the clinical characteristics and successful outcomes of 7 women with pregnancy-related TTP. Median age of patients was 25 (19–32). While 4 out of 7 women were primiparous, others were multiparous. Total plasma exchange (TPE) procedure was started within 24 h after admission to our hospital. All patients got into complete remission without any maternal mortality. Fetal mortality was found to be 28%. Pregnancy-related TTP is still associated with high maternal and fetal mortality rates. However, the prognosis of TTP has improved dramatically with early diagnosis and plasmabased therapies.

    血栓性血小板减少性紫癜（TTP）发病是由于遗传性或获得性ADAMTS-13缺陷。在正常妊娠过程的中晚期，ADAMTS-13活性会出现生理性下降；同时妊娠过程中促凝因素增强、纤溶活性下降；因此存在ADAMTS-13缺陷的患者，在妊娠前未发病，妊娠就很可能成为其发病的诱因；其他诱因包括自身免疫性疾病、感染、肿瘤。妊娠或产后出现的TTP占总TTP 10-25%。TTP治疗以血浆输注/血浆置换为主。既往报道胎儿死亡率为33-60%，母亲死亡率为11-26%。

    本文总结了7例妊娠TTP（5例产前、2例产后），均在入院24小时内开始每日血浆置换，置换量为1-1.5倍血浆容量（40-60ml/kg），PLT＞150×109/L、LDH正常、同时临床症状消失后开始逐渐减少血浆置换。4/7例存在急性肾衰竭和/或神经系统症状，经血浆置换后7例均完全缓解。

    2/7例发生在妊早期，因胎儿死亡而终止妊娠；1/7发生于妊中期而早产，中止妊娠后诊断TTP而开始血浆置换，其在1年后再次妊娠时未发病、同时产下正常胎儿；2/7发生于妊晚期，产下早产儿；2/7例产后（分别为产后2天和产后30天），均产下正常胎儿。只有2例行血清ADAMTS-13活性检测，均为8%（1例为先天性，1例为获得性）。本文胎儿死亡率为28%，母亲死亡率为0%。

    www.ncbi.nlm.nih.gov/pubmed/24667160