Department of Haematology, Guy's & St. Thomas' NHS Foundation Trust, London SE1 7EH, United Kingdom.
Eur J Obstet Gynecol Reprod Biol. 2010 Mar;149(1):10-7. doi: 10.1016/j.ejogrb.2009.11.022. Epub 2009 Dec 23.
Evans' syndrome, the coexistence of immune thrombocytopenia (ITP) with autoimmune haemolytic anaemia (AIHA), is rare in pregnancy, with a few published cases. Concerns about the teratogenic effect of pharmacological agents used in the management of Evans' syndrome limit the treatment options in pregnancy. In this paper we performed a systematic review of the literature of all published cases with Evans' syndrome in pregnancy and we report two new cases. The review was performed by searching the electronic databases PubMed, EMBASE, Cochrane Library and Google scholar up to the end of December 2008. The selection criteria were Evans' syndrome in pregnancy; autoimmune haemolytic anaemia; immune thrombocytopenia. Thirteen papers reporting 14 pregnancies in women with Evans' syndrome have been published: 7 papers are written in English. Evans' syndrome can be diagnosed with a full blood count, film and Coombs testing. It runs a more benign course in pregnancy than in non-pregnant state (notably neutropenia does not occur) and very often resolves post-delivery. The fetal outcome may be less favourable: a minority of fetuses are affected by transplacental passage of antibody and have a significant morbidity and mortality. With appropriate treatment, women with Evans' syndrome can have successful pregnancies, with a good response to conventional treatment. More detailed studies of Evans' syndrome in pregnancy, especially of fetal outcome, are required.
Evans 综合征是指同时伴有免疫性血小板减少性紫癜（ITP）和自身免疫性溶血性贫血（AIHA）的一种疾病，在妊娠中罕见，仅有少数已发表的病例。由于担心用于治疗 Evans 综合征的药物具有致畸作用，限制了妊娠期间的治疗选择。本文对妊娠期间 Evans 综合征的所有已发表病例进行了系统的文献复习，并报告了 2 个新病例。通过检索电子数据库 PubMed、EMBASE、Cochrane 图书馆和 Google scholar，对截至 2008 年 12 月底的所有文献进行了筛选。选择标准为妊娠期间的 Evans 综合征；自身免疫性溶血性贫血；免疫性血小板减少症。共发表了 13 篇关于 14 例妊娠妇女 Evans 综合征的论文：其中 7 篇为英文。 Evans 综合征可通过全血细胞计数、涂片和抗人球蛋白试验进行诊断。与非妊娠状态相比，其在妊娠中具有更良性的病程（特别是不会发生中性粒细胞减少），并且在产后通常会缓解。胎儿结局可能不太理想：少数胎儿因抗体经胎盘传递而受到影响，具有较高的发病率和死亡率。通过适当的治疗，患有 Evans 综合征的妇女可以成功妊娠，并且对常规治疗有良好的反应。需要对妊娠期间 Evans 综合征进行更详细的研究，尤其是对胎儿结局的研究。
