Evans' syndrome in pregnancy: a systematic literature review and two new cases

Abstract

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.