Dimichele Donna M
Weill Medical College of Cornell University, New York, NY 10021, USA.
Int J Hematol. 2006 Feb;83(2):119-25. doi: 10.1532/IJH97.05129.
The development of inhibitory alloantibodies to factor VIII (FVIII) is a major complication of clotting factor replacement therapy for hemophilia A. Inhibitor development compromises effective hemostasis management in affected individuals and results in higher morbidity and costs of care compared with hemophilic individuals without anti-FVIII antibodies. The therapeutic approach to the management of bleeding in the presence of low- and high-titer inhibitors is founded on the principles of either saturating antibody with excess FVIII or bypassing the FVIII requirement altogether. Although spontaneous antibody disappearance does occur, immune tolerance is often required for antibody eradication. Studies aimed at optimizing this treatment approach and developing newer strategies for inhibitor prevention are ongoing.
产生针对凝血因子 VIII(FVIII)的抑制性同种抗体是甲型血友病凝血因子替代疗法的主要并发症。与没有抗 FVIII 抗体的血友病患者相比,抑制性抗体的产生会影响受影响个体的有效止血管理,并导致更高的发病率和护理成本。在存在低滴度和高滴度抑制剂的情况下,出血管理的治疗方法基于用过量 FVIII 饱和抗体或完全绕过对 FVIII 的需求的原则。虽然确实会发生自发抗体消失,但通常需要免疫耐受来消除抗体。旨在优化这种治疗方法和开发预防抑制剂新策略的研究正在进行中。