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| 혈전성 혈소판감소성 자반증의 임상상을 보인 Henoch - Schonlein 자반증 1예 |
| 이영기 , 조상경 , 신진호 , 김용섭 , 차대룡 , 조원용 , 김형규 , 유기환 , 원남희 |
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| Abstract |
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Henoch-Schanlein purpura(HSP) is primarily a disorder of childhood and chracterized by skin rash, joint pain, abdominal pain and renal involvement. Thrombotic thrombocytopenic purpura(TTP) is a rare clinical syndrome of unknown etiology and classically presents with microangiopathic hemolytic anemia, thrombocytopenia, fluctuating neurological signs, renal dysfuncion and fever. Recently, we experienced one patient with histologically proven HSP, and manifested as a TTP like clinical feature. Patient is a 8-year-old boy who had been diagnosed one month earlier as HSP. He showed microangiopathic hemolytic anemia, renal failure and seizure during the course of his illness. Histological evaluation of renal biopsy confirmed the presence of intravascular thrombosis. Treatment with fresh frozen plasma, plasmapheresis, steroid therapy and peritoneal dialysis resulted in clinical impro vement. But he expired on 130th day after admission due to aspiration pneumonia and pneumothorax. |
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