Evans syndrome

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.

Also indexed as

Nearby in Acquired haemolytic anaemia, immune

• 3A20.0Autoimmune haemolytic anaemia, warm type
• 3A20.1Autoimmune haemolytic anaemia, cold type
• 3A20.2Autoimmune haemolytic anaemia, mixed type, cold and warm
• 3A20.3Paroxysmal cold haemoglobinuria
• 3A20.4Alloimmune haemolytic anaemia
• 4B4YOther specified diseases of the immune system
• 4B4ZDiseases of the immune system, unspecified