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| 혈전성 혈소판감소성 자반증의 임상상을 보인 전신성 홍반성 낭창 2예 |
| 이영기 , 권영주 , 이구 , 윤종우 , 조상경 , 차대룡 , 조원용 , 표희정 , 김형규 , 원남희 |
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| Abstract |
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Thrombotic thrombocytopenic purpura(TTP) is a clinical syndrome of unknown etiology and chara- cterized by microangiopathic hemolytic anemia, thro- mbocytopenia, fluctuating neurological status, renal dysfunction and fever. Systernic lupus erythro- matosus(SLE) is also multisystemic disease that some of clinical features may mimic TTP. Therefore both diseases have led to diagnostic confusion. We experienced two cases with SLE who subsequently or initially developed TTP, In case 1, a 44-year old woman had 1-year pre- vious history of SLE and presented with dyspnea. After diagnosis of thrombotic microangiopathy by renal biopsy, she was managed with steroid, cyclophosphamide pulse therapy, fresh frozen plasma infusion and plasmapheresis. She was treated by aggressive treatment; nevertheless, she died on 15th admission day. In case 2, a 22-year old man was admitted because of nausea and vomiting. SLE with TTP was diagnosed by ARA criteria and the finding of microangiopathic hemolytic anemia. He was treated with plasmapheresis, fresh frozen plasma infusion and steroid therapy. He showed clinical response to the therapy, and has shown no recurrence of disease until now on. In conclusion, we suggest that early diagnosis and prompt therapy such as plasmapheresis and plasma infusion are very important in SLE with TTP. |
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