遺伝子組換え活性型第VII因子製剤の持続輸注が奏功した血友病Aインヒビターの下顎咽頭軟部組織出血 Successful Treatment of Life-threatening Maxillopharyngeal Hemorrhage by Continuous Infusion of Recombinant Activated Factor VII in a Hemophilia A Patient with Inhibitor

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抜歯後の下顎咽頭軟部組織出血により生命危機に瀕した血友病Aインヒビター患者の止血管理として, 遺伝子組換え活性型第VII因子 (rFVIIa) 製剤の持続輸注を行った. 患者は高力価 (640 BU) のインヒビターを保有しており, プロトロンビン複合体製剤 (PCC) 50 U/kgの投与下に左上下智歯の抜歯を行ったが, 顎下軟部組織に徐々に血腫を形成した. さらにPCC 50 U/kgを2回追加投与したが, 止血効果は一時的であり, 咽頭へ増大した血腫は気道を圧迫し, 抜歯19時間後, 窒息に至った. 活性型PCC 50 U/kg投与下に気管切開を施行したが, 術後, 気管切開部から再出血をきたすとともに血腫はさらに増大した. 緊急措置としてrFVIIaの持続輸注を行った. rFVIIa 120 μg/kgをボーラス投与後, 30 μg/kg/hで持続静脈内投与し, トラネキサム酸1gを6時間ごとに静脈内投与した. 持続輸注開始後, 気管切開部は止血し, 血腫は速やかに縮小した. 本症例の重症出血は, 従来のバイパス療法の止血効果は一過性で不十分であったが, rFVIIaの持続投与が著効を示した.

We report a successful treatment with recombinant activated factor VII (rFVIIa) of a life-threatening maxillopharyngeal hemorrhage that occurred after extraction of the left wisdom tooth in a hemophilia A patient associated with an inhibitor. Although the extraction of the tooth had been conducted under administration of 50 U/kg of the prothrombin complex concentrate (PCC), a hematoma developed in the submandibular soft tissue. Despite additional administration of PCC twice within 19 hours after the tooth extraction, the hamatoma enlarged and grew toward the pharynx and compressed the respiratory tract. To maintain the airway, a tracheotomy was performed under administration of the activated PCC, but bleeding started at the incision site 3 hours later and formed a hematoma that grew continuously. After a bolus infusion of 120 μg/kg of rFVIIa, we administered rFVIIa continuously for 12 hours at a rate of 30 μg/kg/h together with 1.0 g of tranexamic acid given at every 6 hours intravenously. Within 2 hours after the initiation of rFVIIa treatment, bleeding ceased and the hematoma began to diminish obviously. In this case, administration of rFVIIa brought about a remarkable sustained hemostatic effect for a life-threatening hematoma, for which PCC and activated PCC manifested only a transitory effect.

収録刊行物

  • 日本血栓止血学会誌 = The Journal of Japanese Society on Thrombosis and Hemostasis

    日本血栓止血学会誌 = The Journal of Japanese Society on Thrombosis and Hemostasis 12(1), 39-46, 2001-02-01

    The Japanese Society on Thrombosis and Hemostasis

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各種コード

  • NII論文ID(NAID)
    10008635246
  • NII書誌ID(NCID)
    AN10353762
  • 本文言語コード
    JPN
  • 資料種別
    NOT
  • ISSN
    09157441
  • データ提供元
    CJP書誌  CJP引用  J-STAGE 
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