title: The first case of postpartum acquired hemophilia A in Korea.
abstract: Acquired hemophilia A (AHA) is a rare coagulopathy caused by autoantibodies to coagulation factor VIII (FVIII). Most patients with AHA have been previously healthy; however, a variety of morbidities have been associated with the condition including pregnancy. A 40-yr-old woman visited our institution with extensive hematoma on the right hip area. Her medical history revealed no personal or familial history of bleeding diathesis. Her coagulation tests showed markedly prolonged aPTT (117 sec), markedly decreased level of FVIII activity (0.4%) and high-titer FVIII inhibitor (77 BU). Collectively, she was diagnosed as having postpartum AHA and was treated with bypassing agents and corticosteroids. Her aPTT was normalized on the 174 th postpartum day and FVIII inhibitor showed negative conversion on the 224 th postpartum day. This is the first case of postpartum AHA with high-titer FVIII inhibitor in Korea. Timely diagnosis and management can reduce morbidity and mortality of this potentially life-threatening condition.
authors.affiliation: Department of Laboratory Medicine & Genetics, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea.
authors.forename: Ji Hyun
authors.initials: JH
authors.lastname: Lee
authors.forename: Dong-Hwan
authors.initials: DH
authors.lastname: Kim
authors.forename: Kiyoung
authors.initials: K
authors.lastname: Yoo
authors.forename: Yongmook
authors.initials: Y
authors.lastname: Choi
authors.forename: Sun-Hee
authors.initials: SH
authors.lastname: Kim
authors.forename: Hee-Jin
authors.initials: HJ
authors.lastname: Kim
mesh_terms: D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D000328:Adult; D001323:Autoantibodies / Q000097:blood; D001779:Blood Coagulation Factors / Q000627:therapeutic use; D005169:Factor VIII / Q000276:immunology; D015942:Factor VIIa / Q000627:therapeutic use; D005260:Female; D006406:Hematoma / Q000175:diagnosis; D006467:Hemophilia A / Q000175:diagnosis* / Q000628:therapy; D006801:Humans; D010314:Partial Thromboplastin Time; D049590:Postpartum Period; D011247:Pregnancy; D011994:Recombinant Proteins / Q000627:therapeutic use; D056910:Republic of Korea
chemical_list: D000305:Adrenal Cortex Hormones; D001323:Autoantibodies; D001779:Blood Coagulation Factors; D011994:Recombinant Proteins; D005169:Factor VIII; C103587:recombinant FVIIa; C065655:anti-inhibitor coagulant complex; D015942:Factor VIIa