Rodriguez-Merchan E C
Department of Orthopaedics and Haemophilia Unit, La Paz University Hospital, Madrid, Spain.
Haemophilia. 2008 Mar;14(2):242-7. doi: 10.1111/j.1365-2516.2007.01632.x. Epub 2008 Jan 14.
In this paper, the most recent developments on arthropathy and inhibitors in haemophilia are reviewed. Firstly, early long-term prophylaxis is strongly recommended in children with severe haemophilia. Secondly, in joint bleeds not responding to substitution of factor VIII to normal factor VIII levels angiographic embolization might be considered as a promising therapeutic option. Thirdly, recent data indicate disruption of normal development for adolescents with inhibitors. Fourthly, some case reports indicate the benefit of secondary prophylaxis with recombinant factor VIIa (rFVIIa) or factor eight inhibitor bypassing activity (FEIBA) in patients with severe haemophilia A and inhibitors. Finally, surgical procedures in haemophilia patients with factor inhibitors can be accomplished safely and effectively with FEIBA and/or rFVIIa, although the risk of complications is higher than in haemophilia patients without inhibitors.
本文综述了血友病关节病和抑制剂的最新进展。首先，强烈建议对重度血友病患儿进行早期长期预防。其次，对于关节出血经替代治疗后因子VIII水平未恢复正常的情况，血管造影栓塞术可能是一种有前景的治疗选择。第三，近期数据表明，有抑制剂的青少年正常发育受到破坏。第四，一些病例报告显示，对于重度甲型血友病且有抑制剂的患者，使用重组因子VIIa（rFVIIa）或因子VIII抑制剂旁路活性药物（FEIBA）进行二级预防有益。最后，尽管有因子抑制剂的血友病患者手术并发症风险高于无抑制剂的血友病患者，但使用FEIBA和/或rFVIIa可安全有效地完成手术。
