Knott P Daniel, Khariwala Samir S, Minarchek Joseph
Head and Neck Institute, The Cleveland Clinic Foundation, Cleveland, OH 44195, USA.
Ann Plast Surg. 2005 Mar;54(3):336-8.
Hemophilia B (Christmas disease) is a rare, X-linked bleeding diathesis, which may present with life-threatening hemorrhage. Management of the coagulopathy in the setting of free tissue transfer may be particularly challenging. The authors present the first case in the English literature of a male with hemophilia B undergoing microvascular free flap reconstruction, as well as a review of the current surgical and medical management of hemophilia B. Based upon this experience, perioperative specific factor replacement is recommended. Given physiologic trough levels of the replaced factor, routine antiplatelet therapy appears appropriate. Management of free tissue transfer in the setting of severe hemophilia is significantly more challenging and should benefit from multidisciplinary coordination.
血友病B（克里斯马斯病）是一种罕见的X连锁出血素质，可能出现危及生命的出血。在游离组织移植情况下对凝血病的处理可能特别具有挑战性。作者报告了英文文献中首例患血友病B的男性接受微血管游离皮瓣重建的病例，并对目前血友病B的外科和内科治疗进行了综述。基于这一经验，建议围手术期进行特定因子替代治疗。考虑到所替代因子的生理谷值水平，常规抗血小板治疗似乎是合适的。在严重血友病情况下进行游离组织移植的处理要困难得多，多学科协作应会使其受益。
