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Cardiac tamponade in a patient with moderate hemophilia A and factor VIII inhibitors

机译:患有中度血友病A和VIII因子抑制剂的患者的心脏填塞

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摘要

Cardiac tamponade is a rare, life-threatening complication of hemophilia. The management of pericardial bleeding in hemophilia A patients with inhibitors is particularly challenging because antibodies to factor (F) VIII render the use of high-dose FVIII ineffective. Fortunately, the management of uncontrollable bleeding in patients with hemophilia and inhibitors has improved since the introduction of treatments that bypass the need for FVIII and FIX. A case of hemopericardium complicated by cardiac tamponade occurring one month following an upper respiratory tract infection in a patient with hemophilia and FVIII inhibitors is presented. Management of the present case was based on current guidelines on the use of recombinant FVIIa for acute bleeding in patients with hemophilia and inhibitors. The subsequent development of hemothorax in the present case indicates that a more protracted course of recombinant FVIIa is justified following pericardiocentesis for pericardial bleeding in hemophilia with inhibitors. Alternative approaches to the management of this complication are also reviewed.
机译:心脏填塞是血友病的一种罕见的危及生命的并发症。血友病A患者心包出血的治疗尤其具有挑战性,因为针对因子(F)VIII的抗体使大剂量FVIII的使用无效。幸运的是,自从引入无需使用FVIII和FIX的治疗方法以来,血友病和抑制剂患者无法控制的出血处理得到了改善。提出了一例血友病和FVIII抑制剂患者上呼吸道感染后一个月发生的心包积血合并心包填塞的情况。本病例的治疗基于当前关于使用重组FVIIa治疗血友病和抑制剂患者急性出血的指南。在本例中血胸的后续发展表明,在使用抑制剂进行血友病心包出血后,进行心包穿刺是合理的,重组FVIIa的治疗过程更为延长。还审查了处理这种并发症的其他方法。

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