Berntorp Erik, Gringeri Alessandro, Leissinger Cindy, Négrier Claude, Key Nigel
Malmö University Hospital, Malmö, Sweden.
Semin Thromb Hemost. 2006 Jun;32 Suppl 2:22-7. doi: 10.1055/s-2006-946911.
Managing hemophilia becomes particularly difficult in patients with inhibitory antibodies, especially in those requiring surgery or with refractory bleeding events. Equally challenging are those patients who develop autoantibodies against factor VIII (FVIII) in the absence of a prior history of FVIII deficiency (acquired hemophilia). Physicians seeking both short- and long-term treatment strategies for bleeding events must often rely on FVIII-bypassing agents such as activated prothrombin complex concentrate (e.g., factor eight bypassing activity [FEIBA VH, Baxter BioScience, Westlake Village, CA]) or recombinant factor VIIa (rFVIIa [NovoSeven, NovoNordisk, Bagsvaerd, Denmark]). Surgical procedures in patients with inhibitors present a considerable challenge, from both a risk-benefit and a cost-benefit aspect. Hemostasis is difficult to achieve in these patients and new treatment options are being explored. Similarly challenging are refractory bleeds, the management of which is likely to benefit from a systematic treatment approach.
对于存在抑制性抗体的血友病患者,尤其是那些需要手术或发生难治性出血事件的患者,血友病的管理变得尤为困难。同样具有挑战性的是那些在既往无FVIII缺乏病史(获得性血友病)的情况下产生抗FVIII自身抗体的患者。寻求出血事件短期和长期治疗策略的医生通常必须依赖FVIII旁路制剂,如活化凝血酶原复合物浓缩物(例如,因子VIII旁路活性制剂[FEIBA VH,百特生物科学公司,加利福尼亚州西湖村])或重组因子VIIa(rFVIIa [诺和七,诺和诺德公司,丹麦 Bagsvaerd])。从风险效益和成本效益方面来看,存在抑制剂的患者进行外科手术都面临着相当大的挑战。这些患者很难实现止血,目前正在探索新的治疗选择。难治性出血同样具有挑战性,其管理可能受益于系统的治疗方法。