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전신성 홍반성 루프스와 동시 발병한 혈전성 혈소판 감소성 자반증 2예

Two Cases of Thrombotic Thrombocytopenic Purpura in Systemic Lupus Erythematosus

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참고문헌

주제분류소아과학

초록

TTP는 발열, 미세혈관용혈성빈혈, 혈소판 감소, 신경계 장애, 다양한 정도의 신기능 이상을 특징으로 하는 임상 증후군으로 SLE 등의 자가면역질환과 드물게 동반되어 발생한다[1]. TTP의 증상은 SLE의 임상증상과 유사하며, 두 질환이 동반되어 발생할 수 있어 이들의 감별은 쉽지 않다. 그러나 치료에 있어 두 질환의 감별은 중요함으로 병리기전에 대한 충분한 연구가 필요하다[1-4]. 저자들은 청소년기 두 명의 남아에서 TTP와 SLE가 동시에 발생한 예를 경험하였기에 문헌고찰과 함께 보고하는 바이다.

Abstract

Thrombotic thrombocytopenic purpura(TTP) is a rare but life-threatening multi-system disorder characterized by the classic pentad of clinical features that includes fever, microangiopathic hemolytic anemia, thrombocytopenia, neurologic abnormalities and renal dysfunction. TTP has been rarely reported to simultaneously present with systemic lupus erythematosus (SLE). While it is important to distinguish between the two diseases of therapeutic implication, cases of concurrent TTP and SLE help to elucidate the pathophysiology that underlies each condition. We describe two adolescents with synchronous TTP and SLE, and review the literature.

참고문헌 (15)

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  11. "Thrombotic thrombocytopenic purpura. Transfusion Medicine Check Sample, TM-3", pp.29~42 (2003), Chicago, IL: ASCP Press
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  14. "Thrombotic thrombocytopenic purpura: new insights in disease pathogenesis and therapy", 14, pp.244~257 (2000)
  15. "The initial neurologic manifestations", 358-62

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