임신 중 혈전성 혈소판감소성 자반증 3예
Thrombotic thrombocytopenic purpura in three pregnancies
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혈전성 혈소판감소성 자반증-용혈성 요독 증후군은 심각한 다기관 질환으로 미세혈관병성 용혈성 빈혈, 발열, 변동성 신경학적 이상, 신기능 이상을 특징으로 한다. 임신 중 매우 드물게 발생하나, 대부분 가임기 여성에서 흔하고 임신과 합병시 높은 모성 사망률과 이환율, 주산기 사망률을 보인다. 혈장 교환술로 인해 이러한 유병률과 사망률이 현저히 감소되었으나 불응성 혈전성 혈소판감소성 자반증은 매우 위중하다. 임신 중 미세혈관병성 용혈성 빈혈 소견을 동반한 환자에서 즉각적인 진단과 혈장교환술 등의 적극적인 치료는 대단히 중요하다. 저자들은 HELLP 증후군과 면역성 혈소판감소성 자반증으로 진단하였던 혈전성 혈소판감소성 자반증 환자에서 혈장교환술을 시행하였으나 사망한 사례를 포함한 3예를 경험하여 최신 진단 검사인 ADAMTS-13 활성도 측정법과 문헌고찰을 함께 보고하는 바이다.
Abstract
Thromobotic thrombocytopenic purpura-hemolytic uremic syndrome (TTP-HUS), characterized by microangiopathic hemolytic anemia, thrombocytopenia, fever, central nervous system abnormalities, and renal dysfunction, is severe multisystem disorder. TTP-HUS occurs predominantly in the reproductive aged-women, associated with poor prognosis. Although the morbidity and mortality have been significantly decreased by using plasma exchange therapy, refractory TTP-HUS remains a tremendous problem. It is crucial to differentiate other microangiopathic hemolytic anemia disease with a confusing presentation and to perform the immediate plasmapheresis. We have experienced three cases, which were initially diagnosed as HELLP syndrome or immune thrombocytopenic purpura. Despite of aggressive plasmapheresis, two women died. We present these cases with a review of the literature on pregnancy–associated thrombotic microangiopathy, including ADAMTS-13 activity assay as a new diagnostic test.
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