학술논문

A Case of Acquired Hemophilia A Diagnosed in Conjunction with Oral Bleeding / 口腔咽頭喉頭血腫を初発症状とした後天性血友病 A 症
Document Type
Journal Article
Source
日本耳鼻咽喉科学会会報 / Nippon Jibiinkoka Gakkai Kaiho. 2016, 119(8):1133
Subject
acquired hemophilia
factor VIII inhibitor
laryngeal hematoma
oral bleeding
口腔出血
喉頭出血
後天性血友病 A
第Ⅷ凝固抑制因子
Language
Japanese
ISSN
0030-6622
1883-0854
Abstract
Acquired hemophilia A is a rare disease in which autoantibodies to factor VIII are present. It is often manifested as a sudden onset of a critical bleeding episode, and its incidence is reported to be 1.48 cases per million persons per year. We report herein on a case of acquired hemophilia A associated with a submucosal hematoma of the oral floor, pharynx, and larynx. A 78-year-old male presented with fresh bleeding from his mouth, associated with hematoma of the oral floor, pharynx, and larynx. Laboratory test showed that the activated partial thromboplastin time was prolonged, and the platelet count and prothrombin time were normal. Coagulation tests revealed decreased factor VIII levels, and the presence of factor VIII inhibitor. A diagnosis of acquired hemophilia A was made, and immunotherapy with corticosteroids was initiated. After treatment, the bleeding tendency was controlled. On hospital day 29, the symptoms disappeared. Although acquired hemophilia A is a rare coagulopathic condition, it should be considered as one of the differential diagnoses in a case of sudden onset of severe hemorrhagic tendency of unknown origin.