폐경색 및 다발성 동맥 혈전증을 보인 소아의 항인지질항체증후군 1례
A Case of Antiphospholipid Syndrome Presenting as Pulmonary Infarction and Multiple Arterial Thromboembolism
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저자들은 발열, 객혈, 빈호흡 및 양하지 통증 및 감각저하를 보인 7세 여아에서 폐와 양하지의 다발성 동맥 혈전증 및 항인지질항체 양성을 보여 1차성 항인지질항체증후군으로 진단하고, 저분자량 헤파린 투여 후 호전되어 혈전증의 재발없이 와파린 경구복용하며 3년간 외래 관찰 중인 1례를 경험하였기에 문헌고찰과 함께 보고하는 바이다.
Abstract
Antiphospholipid syndrome(APS) is a noninflammatory autoimmune disorder characterized by the association of arterial and/or venous thrombosis, recurrent fetal loss and elevated titres of antiphospholipid antibodies, namely lupus anticoagulant(LAC) and/or anticardiolipin antibodies(aCL). It can either occur as a free-standing condition(primary APS) or be associated with another autoimmune disease(secondary APS), mainly systemic lupus erythematosus. The precise pathogenesis of thrombosis in APS in unknown. For children with unexplainable venous or arterial thrombosis, APS should be considered. The diagnosis of APS in children requires a clinical event including venous or arterial thrombosis or immune thrombocytopenia and a laboratory abnormality including positive LAC test or positive aCL antibody test, moderate or high titer IgG. The laboratory abnormality should persist for at least 2 months. We report a 7-year-old girl who had pulmonary embolism and multiple arterial thrombosis caused by primary APS. The laboratory abnormality was positive LAC and β2-glycoprotein I. She was treated successfully with low molecualr weight heparin (LMWH). After recovery, she was continuously treated with subcutaneous LMWH for another 3 months and switched to oral warfarin. She was symptom-free through 3 years of follow-up
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