Introduction: Evan’s Syndrome is a rare autoimmune haematological disorder, defined by the coexistence of immune thrombocytopenia (ITP) and autoimmune hemolytic anaemia (AIHA), in the absence of a known underlying etiology. As literature pertaining to Evan’s Syndrome in pregnancy is scanty, predicting both maternal and fetal outcome is challenging. Available reports have described a rather benign maternal disease course, often resolving postpartum, but resulting in a seemingly poorer fetal outcome.

Methods: We describe a case of a 36 year old Iban female, Mdm L, G4P1+2A, who was diagnosed with Evan’s Syndrome in 2010 and referred to our antenatal specialist clinic at 19 weeks gestation. She was managed by both obstetrics and medical teams throughout her pregnancy, and had her steroid therapy continued. She developed pre-eclampsia at 36 weeks and required C-section at 37 weeks, which was complicated by primary postpartum haemorrhage. Her baby was born well with a good weight, and normal hemoglobin and platelet count.

Discussion: Evan’s Syndrome in pregnancy has been reported to result in complications not only to the pregnant woman, but also to the developing fetus, due to transplacental passage of autoantibodies.

ITP may provoke maternal hemorrhagic complications such as placental abruption and postpartum hemorrhage. In the fetus/newborn, severe thrombocytopenia has been reported, resulting in fetal hemorrhagic complications such as intracranial hemorrhage.

The component of AIHA may pose a risk of life-threatening anaemia in patients, while causing fetal complications such as growth restriction, massive hemolysis leading to stillbirth, and severe postpartum hemolytic anaemia. Regular fetal monitoring should be performed with emphasis on predicting features of fetal anaemia (MCA peak velocity, IUGR).

A predisposition to pre-eclampsia and systemic lupus erythematosus (SLE) has also been described.

Treatment options during pregnancy are limited due to concerns regarding the teratogenic effects of drugs commonly used in the management of Evan’s syndrome. Steroid therapy and azathioprine have proven to be effective and safe in pregnancy. Intravenous gamma immunoglobulin and splenectomy can be considered in refractory cases.

Conclusion: We hope to raise the awareness of this very rare medical condition in pregnancy, by highlighting the possible complications that may arise and illustrate the importance of a multidisciplinary team approach in its management during pregnancy. Early and close surveillance, under a consultant led unit, is essential in maximizing the chances of a favorable outcome in both the pregnant woman and her developing fetus.