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KMID : 0387820220290010021
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Clinical Pediatric Hematology-Oncology
2022 Volume.29 No. 1 p.21 ~ p.24
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A Rare Case of Acquired Hemophilia A in Adolescents and Young Adults
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Lee Min-Jeong
Park Young-Shil
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Abstract
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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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KEYWORD
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Hemophilia A, Acquired hemophilia A, Coagulation factor VIII, Autoanti-bodies, Immunosuppressive treatment
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