Yanagi Eriko, Kishi Toshiyuki, Matsumura Takao, Tani Yoshinori, Miyahara Nobuaki
Nihon Jibiinkoka Gakkai Kaiho. 2016 Aug;119(8):1133-6.
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.
获得性血友病A是一种罕见疾病,体内存在针对凝血因子VIII的自身抗体。它常表现为严重出血事件的突然发作,据报道其发病率为每年每百万人中有1.48例。我们在此报告一例与口腔底部、咽部和喉部黏膜下血肿相关的获得性血友病A病例。一名78岁男性出现口腔新鲜出血,伴有口腔底部、咽部和喉部血肿。实验室检查显示活化部分凝血活酶时间延长,血小板计数和凝血酶原时间正常。凝血试验显示凝血因子VIII水平降低,且存在凝血因子VIII抑制剂。诊断为获得性血友病A,并开始使用皮质类固醇进行免疫治疗。治疗后,出血倾向得到控制。在住院第29天,症状消失。尽管获得性血友病A是一种罕见的凝血病,但在不明原因突然出现严重出血倾向的病例中,应将其视为鉴别诊断之一。