저용량 헤파린 투여로 성공한 발작성 야간혈색소뇨증환자의 분만
Delivery in a Patient with Paroxysmal Nocturnal HemoglobinuriaSuccessfully Managed with Low Molecular Weight Heparin Therapy
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초록
발작성 야간 혈색소뇨증은 드문 후천성 클론성 혈액질환으로서 혈액 줄기세포의 체세포 돌연변이로 발생한다. 발작성 야간혈색소뇨증 환자가 임신을 하는 경우는 드물며 임신되더라도 초기 유산의 빈도가 높고 임신 지속시 혈전증 빈도 증가로 산모 또는 태아의 건강에 심각한 위협이 될 수 있다. 저자들은 발작성 야간혈색소뇨증을 가진 임산부에게 산전 주기적인 예방적 수혈 및 저분자량 헤파린을 사용한 항응고치료에 의해 성공적 분만 1예를 경험하였기에 문헌 고찰과 함께 보고하는 바이다.
Abstract
Paroxysmal nocturnal hemoglobinura is a very rare acquired clonal hematopoietic cell disorder leading to chronic intravascular hemolysis caused by abnormal complement activation. Pregnancy in a patient with paroxysmal nocturnal hemoglobinuria is often complicated with thrombosis resulting serious materno-fetal morbidity and mortality. A case of successful maintenance of the pregnancy, delivery and postpartum in a woman at gestational age of 37 weeks and 2 days with paroxysmal nocturnal hemoglobinuria managed with prophylactic transfusions and anticoagulation therapy by low molecular weight heparin is presented with a review of literature.
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