면역 글로블린 투여로 호전된 용형성 요독 증후군 1 예

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)