口腔内出血を繰り返した5p症候群および血友病A女児の1例

• Published in: 日本口腔外科学会雑誌 Vol. 56; no. 8; pp. 485 - 489
• Main Authors: 今井, 裕一郎, 石田, 純一, 上田, 順宏, 吉岡, 元, 稲掛, 耕太郎, 桐田, 忠昭
• Format: Journal Article
• Language: Japanese
• Published: 社団法人 日本口腔外科学会 20.08.2010
• Subjects: 5p症候群; 局所止血; 血友病A; 補充療法
• ISSN: 0021-5163; 2186-1579
• DOI: 10.5794/jjoms.56.485

We report the rare case of oral bleeding in a patient with 5p-syndrome and hemophilia A. A 6-year-old girl with 5p-syndrome and hemophilia A had three episodes of oral bleeding. Hemostasis was performed, with an uneventful course. The first and second episodes were caused by self-inflicted injuries to the gingiva of the maxillary anterior teeth. To control the bleeding episodes, 500 mg/day of tranexamic acid was administered intravenously, and the bleeding site of the cervical gingiva was packed with Surgicel. A hemostatic splint was placed for wound protection and hemostasis, resulting in an uneventful recovery and discharge. The third episode bleeding involved the tongue and was treated by administering tranexamic acid at a dose of 500 mg/day for 7 days from the first hospital day. However, since hemostasis was difficult to achieve, 1,000 units of factor VIII concentrate was administered. The level of factor VIII activity rose from 5.0 % immediately after admission to 120 % 1 hour after administration, and the lingual bleeding stopped. Subsequently, the hemostatic and wound healing status remained favorable and stable, and the patient was discharged on the 15th hospital day.