| An Experience of High Dose Intravenous Immunoglobulin Therapy in Refractory Evans Syndrome. |
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Hee Sup Kim, Won Sup Shin, Sang Il Lee, Sang Woo Kim |
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Department of Pediatrics, Seoul Paik Hospital, Inje University, Seoul, Korea |
| Evans 증후군에서 대량의 정맥 감마글로불린
치료의 경험 1례 |
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김희섭, 신원섭, 이상일, 김상우 |
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인제대학교 서울백병원 소아과학교실 |
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Received: 7 November 1988 • Accepted: 26 September 1989 |
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| Abstract |
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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. |
| Key Words:
Evans syndrome, Intravenous immunoglobulin therapy |
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