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

概要

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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.
Japanese Society of Oral and Maxillofacial Surgeonsの論文