Evans' syndrome in IgA deficiency. Episodic autoimmune haemolytic anaemia and thrombocytopenia during a 10 years observation period.

A 26-year-old male with a 10-year history of complete selective IgA deficiency and recurrent autoimmune anaemia and thrombocytopenia (Evans syndrome) is presented. Both serum IgA and saliva secretory IgA were below the detection limit (less than 0.05 mg/l). No other features of autoimmunity were seen. The patient had a normal % of peripheral blood lymphocytes with surface IgM and IgG cells and normal in vitro lymphocyte transformation after stimulation with mitogens and antigens. The pleomorphic and randomly appearing immunologic features of selective IgA deficiency are emphasized by the present case.