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논문 기본 정보

자료유형
학술저널
저자정보
Min Jeong Lee (Department of Pediatrics, Kyung Hee University Hospital at Gangdong, Seoul, Korea) Young Shil Park (Department of Pediatrics, Kyung Hee University Hospital at Gangdong, Seoul, Korea)
저널정보
대한소아혈액종양학회 Clinical Pediatric Hematology-Oncology Clinical Pediatric Hematology-Oncology Vol.29 No.1
발행연도
2022.4
수록면
21 - 24 (4page)
DOI
10.15264/cpho.2022.29.1.21

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Acquired hemophilia A (AHA) is a rare bleeding disorder, especially in adolescents and young adults (AYAs) attributable to the development of autoantibodies against coagulation factor VIII (FVIII). AHA diagnosis is difficult; patients lack any history of coagulopathy. We report here on an AYA with AHA who responded well to treatment. A 19-year-old woman visited our hospital with painful swelling of the right lower leg. She had no past or familial history of a bleeding disorder. The initial laboratory data revealed a prolonged activated partial thromboplastin time and an uncorrected mixing test result. The FVIII activity was below 1% and the FVIII antibody level 22.4 Bethesda units. She was diagnosed with AHA and treated with recombinant activated coagulation factor VII, activated prothrombin complex concentrates and an oral steroid. After 9 months, FVIII antibody level was negative and the FVIII activity was normalized. AHA is very rare especially in AYAs, but physicians must be suspi-cious about the disorder and plan specialized coagulation tests to diagnose the disease. An early diagnosis of acquired bleeding disorders should be done for initiat-ing the adequate treatment immediately by both controlling the acute bleeding epi-sode and eliminating FVIII antibodies.

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