혈전성 혈소판감소성 자반증의 임상상을 보인 전신성 홍반성 낭창 2예
Two Cases of Systemic Lupus Erythromatosis with Manifestation of Thombotic Thrombocytopenic Purpura
소속 상세정보
이영기
권영주/이구/윤종우/조상경/차대룡/조원용/표희정/김형규
KMID : 0359919970160030584
Abstract
서론
전신성 홍반성 낭창은 면역복합체의 침착과 보체 활성화를 통해 여러 장기에서 각종 염증
반응을 일으키는 자가면역 질환으로, 전신성 홍반성 낭창의 9-12%에서 혈전성 합병증이 발
생하며, 드물게 혈전성 혈소판감소성 자반증이 병발한다. 혈전성 혈소판감소성 자반증은 혈
소판감소증, 미세혈관병성 용혈성빈혈, 발열, 신기능부전, 신경학적 이상을 특징으로 하는 질
환으로, 조직학적 소견상 소혈관의 혈전에 의한 폐색을 동반하며 치명적일 수 있다. 1925년
Moschowitz에 의해 알려진 이후 전세계적으로 증례들이 보고되고 있으나, 아직 원인이나
병리기전은 확실하게 알려져 있지 않다. 저자들은 전신성 홍반성 낭창으로 진단 받은 환자
에서 병발된 혈전성 혈소판감소성 자반증 2예를 경험하였기에 보고하는 바이다.
#초록#
Thrombotic thrombocytopenic purpura(TTP) is a clinical syndrome of unknown
etiology and characterized by microangiopathic hemolytic anemia, thrombocytopenia,
fluctuating neurological status, renal dysfunction and fever. Systemic lupus
erythromatosus(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 previous 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.
키워드
Thrombotic thrombocytopenic purpura; Systemic lupus erythromatosus;
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