口底粘膜下血腫を契機に診断された後天性血友病Aの1例

• Published in: 日本口腔外科学会雑誌 Vol. 58; no. 5; pp. 312 - 316
• Main Authors: 清水, 武, 野池, 淳一, 横林, 敏夫, 傳田, 祐也, 柴田, 哲伸, 五島, 秀樹
• Format: Journal Article
• Language: Japanese
• Published: 社団法人 日本口腔外科学会 20.05.2012
• Subjects: 口底; 口腔内出血; 後天性血友病; 第VIII因子インヒビター; 血腫
• ISSN: 0021-5163; 2186-1579
• DOI: 10.5794/jjoms.58.312

Acquired hemophilia A is a rare disorder in which autoantibodies to factor VIII arise, and there is a sudden onset of a critical bleeding episode. Its incidence is reported to be 1.48 cases per million persons per year. We report the case of a patient who had a diagnosis of acquired hemophilia A with submucosal hematoma of the oral floor. A 75-year-old man presented with a swelling in the oral floor. Marked, dark red swelling was seen in the oral floor. On laboratory tests, the platelet count and prothrombin time were normal. However the activated partial thromboplastin time （APTT） was longer than normal. The patient was admitted on the same day. On hospital day 2, acquired hemophilia was suspected because of the presence of intrinsic pathway inhibitors of hemostasis. Then, hemostatic therapy and immunotherapy with corticosteroids were begun. At night of the same day, hypoxemia due to upper airway obstruction occurred, and intratracheal intubation was performed. After that, cyclophosphamide was administered. On hospital day 52, APTT returned to the normal range. The patient was discharged and is being treated as an outpatient in our hospital.