• 대한임상검사과학회지
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• 제54권2호
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• pp.163-166
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• 2022

Acquired hemophilia A (AHA) is an uncommon autoimmune bleeding disorder in which autoantibodies that affect the functions of factor VIII (FVIII) are present in the blood. The initial diagnosis of AHA is difficult as the presentations of AHA differ from those of congenital hemophilia A. Moreover, the treatment of AHA is more complex due to the presence of autoantibodies against FVIII. Here, we present a case report of postpartum AHA, to increase the perception and knowledge regarding the recognition and management of such cases. We present a young female with the chief complaint of vaginal bleeding and upper arm ecchymosis. Laboratory results exhibited isolated prolonged activated partial thromboplastin time (APTT) and FVIII inhibitors. The patient was treated with corticosteroids, FVIII concentrates, and a bypassing agent. In conclusion, unexplained postpartum bleeding, unmanageable with basic hemostatic measures, should lead to clinical suspicion of an acquired bleeding disease.

• Clinical and Experimental Pediatrics
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• 제49권8호
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• pp.821-829
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• 2006

Hemophilia is the most common coagulation disorder. It has a long history. Hemophilia A is caused by FVIII gene mutation, and hemophilia B by FIX gene mutation. Those genes are located on X chromosome long arm. Bleedings in hemophiliacs predominantly occur in joints and muscles. Because those site are insufficient in tissue factor to induce hemostasis. Among joints knee, ankle and elbow are most frequently affected because their synovial structure is vulnerable to injury compared to other joints. Hemophilia is diagnosed with factor assay. Severe hemophilia is below 1% of FVIII : C, moderate between 1% and 5%, mild over 5%. Carrier detection and prenatal diagnosis have been conducted with RFLP-based linkage analysis and DNA sequencing. Mainstay of treatment is factor replacement therapy so far. Bleedings can be controlled by infusion of factor concentrates. Hemophilc arthropathy and muscle contracture are representative sequelae. Complications of facotor replacement therapy are inhibitor development and infections. Hemophiliacs with inhibitor should be managed with large dose factor concentrate, bypassing agent, ITI and immunosuppression. Ultimately, hemophilia could be cured by gene therapy.