Evans syndrome

**Definition:** Evans syndrome is characterised by the association of autoimmune haemolytic anaemia with another haematological anomaly. The thrombocytopaenia may precede, occur concurrently with, or secondary to the autoimmune haemolytic anaemia.

**Long definition:** Evans syndrome is characterised by the association of autoimmune haemolytic anaemia with another haematological anomaly (such as destruction of the polynuclear neutrophils, neutropaenia or, more frequently, platelet destruction or thrombocytopaenia). The prevalence is estimated at 1 in 1,000,000. The thrombocytopaenia may precede, occur concurrently with, or secondary to the autoimmune haemolytic anaemia. Evans syndrome is a rare disease for which the treatment strategy is poorly defined. Management usually involves administration of immunosuppressors or splenectomy.