면역 글로블린 투여로 호전된 용혈성 요독 증후군 1예

• Published in: The Korean journal of medicine pp. 11 - 435
• Main Authors: 조덕신, 이병재, 민태훈, 강보현, 김형훈, 이병완, 최동철
• Format: Journal Article
• Language: Korean
• Published: 대한내과학회 01.10.2002
• Subjects: 내과학
• ISSN: 1738-9364; 2289-0769

저자들은 국내에는 드문 성인 용혈성 요독 증후군 환자에서 혈장 교환술에 반응이 없어 이차적 치료로 면역글로불린을 투여하여 완전 치유가 되었기에 이를 문헌 고찰과 함께 보고하는 바이다. The hemolytic uremic syndrome (HUS) is a clinical syndrome defined by the presence of thrombocytopenia, microangiopathic hemolytic anemia and acute renal failure with or without a clinically apparent etiology. The conventional treatment of choice is plasmapheresis as a first-line therapy. Most patients respond to plasmapheresis whereas some patients are refractory to the therapy. The second-line therapy is not well established although various therapies such as steroid, vincristine, intravenous immunoglobulin have been suggested. The intravenous immunoglobulin therapy in refractory hemolytic-uremic syndrome have rarely been successful in complete remission. We report a case of refractory HUS in a 48 year-old man who developed hemolytic anemia, thrombocytopenia, acute renal failure and ischemic retinopathy. The patient was refractory to plasmapheresis as a first-line therapy. The patient received intravenous immunoglobulin therapy as a second-line therapy after 8 days of plasmapheresis, which subsequently resulted in a complete remission of refractory HUS. The complete remission using immunoglobulin in HUS has not been previously reported in Korea.(Korean J Med 63:431-435, 2002) KCI Citation Count: 0