扁摘・アデノイド切除後出血を契機に診断された血友病Aキャリアーの1症例 [in Japanese] Hemophilia A carrier diagnosed after post-adenotonsillectomy hemorrhage : A case report [in Japanese]
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症例は4歳女児で, 睡眠時無呼吸に対して, 口蓋扁桃摘出術およびアデノイド切除を施行した. 術前血液検査ではAPTTの延長以外は正常範囲内であった. 手術は特に問題なく行われたが, 術後出血を繰り返し, 止血術を2回, 輸血を1回行った. 術後に家族歴を詳しく聴取したところ, 患児の叔父が軽症血友病であることが分かり, 患児は凝固因子検査の結果血友病Aキャリアーと診断された. 2回目の止血術後も出血が続いていたが, 第VIII因子製剤投与により止血した. 術前検査でAPTTの延長があった場合は, 血友病/血友病キャリアーを鑑別診断の1つとして念頭に置き精査を行い, 術前に診断をつけておくことが安全に手術を行うために重要である.
Hemophilia A is an X-linked hereditary bleeding disorder caused by coagulation factor VIII deficiency, and although males are usually affected, there have been a few reports of excessive bleeding in female carriers. Here we report a case of a hemophilia A carrier diagnosed after postadenotonsillectomy hemorrhage.<BR>A 4-year-old girl with sleep apnea was referred to one of the authors. Adenotonsillar hypertrophy was detected, and her parents requested surgical treatment. Preoperative blood tests showed an elevated activated partial thromboplastin time (APTT) of 41.5s (normal range: 25.8-37.7s, normal control: 27.0s), but other routine laboratory data yielded no abnormal fundings. Adenotonsillectomy was carried out uneventfully under general anesthesia with little intraoperative blood loss. However, there was significant wound hemorrhage within several hours after the operation, and reoperation was performed, but oozing from the wound persisted. A second reoperation with blood transfusion was performed on postoperative day 5, and detailed questioning about the family history revealed that her mother's brother had mild hemophilia. The patient was tested for clotting function, and was diagnosed as a hemophilia A carrier. Coagulation factor VIII activity was 68%, the lower limit of the normal range, but the vWF (von Wiilebrand factor) level was 152% and within the normal range. Infusion of factor VIII concentrate was started and there was immediate and complete resolution of the oozing. The patient was discharged 17 days after the adenotonsillectomy.<BR>We wish to emphasize that whenever the APTT of female patients as well as male patients is prolonged 10 seconds or more above normal control in a preoperative blood test, clotting factor tests should be performed in addition to careful questioning about the family history. Preoperative diagnosis and adequate correction of factor VIII activity, if necessary, allow surgery, such as tonsillectomy to be performed safely in hemophilia patients and hemophilia carriers.
Stomato-pharyngology 20(3), 363-368, 2008-06-10
Japan Society of Stomato-pharyngology