심한 증식성 루푸스 신염과 혈전성 혈소판 감소성 자반증이 병발한 남성 환자에서 cyclophosphamide 치료 경험 1예
Cyclophosphamide treatment in a patient with thrombotic thrombocytopenic purpura and lupus nephritis : report of one case
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초록
TTP가 병발한 SLE에서 조기에 신선 냉동 혈장 투여와 혈장 교환술을 적극적으로 시행해야 한다. 이러한 적극적인 초기 치료에도 불구하고 병이 진행하거나, 루푸스 신염이 동반된 경우에 cyclophosphamide의 병합 요법이 치료에 도움이 됨을 저자들은 경험하여 보고하는 바이다.
Abstract
Thrombotic thrombocytopenic purpura is a rare but fatal complication of systemic lupus erythematosus. The diagnosis of thrombotic thrombocytopenic purpura as a syndrome distinct from systemic lupus erythematosus may be challenging particularly when thrombotic thrombocytopenic purpura is presented concomitantly with systemic lupus erythematosus. Early diagnosis and aggressive treatment including plasmapheresis would be required. However, recent reports have suggested that the use of cyclophosphamide may have a role. We describe a patient with systemic lupus erythematosus who was first presented with severe thrombotic thrombocytopenic purpura. Diagnosis was based on typical clinical features of thrombotic thrombocytopenic purpura and laboratory findings of active lupus nephritis. Renal biopsy also confirmed the coexistence of thrombotic thrombocytopenic purpura and diffuse proliferative lupus nephritis. Although prompt extensive plasmapheresis and high dose steroid therapy were performed, oliguric renal failure and thrombocytopenia persisted. After addition of cyclophosphamide to the treatment with plasmapheresis and steroid, clinical manifestations of thrombotic thrombocytopenic purpura and lupus nephritis were markedly improved. (Korean J Med 71:214-218, 2006)
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