Intravenous gamma globulin for thrombocytopenia in children with Evans syndrome

Abstract

We describe three patients with Evans syndrome (immune hemolytic anemia and immune thrombocytopenia) who were refractory to conventional therapy, including steroids and splenectomy in all of the patients, vincristine in two, and cyclophosphamide in one. The patients were then treated with modified intravenous gamma globulin 0.4 g/kg/day for 5 consecutive days. Two patients failed to respond, but the third had a clinical remission after gamma globulin therapy. Given the usual chronic and relapsing course of Evans syndrome and the poor response to conventional therapy, we recommend that high dose i.v. gamma globulin be considered an alternative therapy in the management of these patients.