Ghosh Nina, Teefy Patrick, Laudenbach Lori, Rivard Georges-Etienne
University of Western Ontario, London, Ontario.
Can J Cardiol. 2006 Jan;22(1):73-5. doi: 10.1016/s0828-282x(06)70243-4.
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.
心脏压塞是血友病一种罕见的、危及生命的并发症。对于有抑制物的甲型血友病患者，心包出血的处理尤其具有挑战性，因为针对凝血因子（F）VIII的抗体使大剂量FVIII的使用无效。幸运的是，自从引入了绕过FVIII和FIX需求的治疗方法后，血友病和有抑制物患者不可控出血的处理已有改善。本文报告了1例血友病和FVIII抑制物患者在上呼吸道感染1个月后发生血心包并并发心脏压塞的病例。本病例的处理基于目前关于重组FVIIa用于血友病和有抑制物患者急性出血的指南。本病例随后发生血胸表明，对于有抑制物的血友病患者心包出血行心包穿刺术后，延长重组FVIIa的疗程是合理的。本文还综述了该并发症处理的其他方法。
