All issues > Volume 33(5); 1990

Original Article

J Korean Pediatr Soc. 1990;33(5):701-707. Published online May 31, 1990.

An Experience of High Dose Intravenous Immunoglobulin Therapy in Refractory Evans Syndrome.

Hee Sup Kim¹, Won Sup Shin¹, Sang Il Lee¹, Sang Woo Kim¹

¹Department of Pediatrics, Seoul Paik Hospital, Inje University, Seoul, Korea

Received: November 7, 1988;  Accepted: September 26, 1989.

Abstract

Evans syndrome, a combination of autoimmune hemolytic anemia and idiopathic thrombocytopenic purpura, relapses freguently and is refractory to conventional treatment such as corticosteroid, immunosuppressive agents, splenectomy and plasma exchange. Recently Oda et al applied high dose immunoglobulin (400/kg/day for 4 days repeated 2 weeks later) to 5 month-old infant with refractory Evans syndrome, and was successful in maintaining remission for five years and nine months without any other treatment. Our patient, 12 year old girl, responded well to the steroid therapy initially but became refractory to it when the disease relapsed. As a next step we applied Oda’s regimen to the patient. She responded more rapidly than the previous steroid therapy but subsequently relapsed again with rapid reduction of platelet count. At this time she did not respond to high dose gamma globulin and finally expired 5 months after the onset of the disease.

Keywords :Evans syndrome, Intravenous immunoglobulin therapy