{"title":"Efanesoctocog alfa:因子 VIII 替代疗法的复兴。","authors":"Yesim Dargaud, Alexandre Leuci, Alejandra Reyes Ruiz, Sebastien Lacroix-Desmazes","doi":"10.3324/haematol.2023.284498","DOIUrl":null,"url":null,"abstract":"<p><p>Efanesoctocog alfa (Altuviiio,TM Sanofi-SOBI) is a B domain-deleted single-chain Factor VIII (FVIII) connected to D'D3 domain of von Willebrand Factor (vWF). Its ingenious design allows efanesoctocog alfa to operate independently of endogenous vWF and results in an outstanding 3-4 times longer half-life compared to standard and extended half-life (EHL) FVIII products. The prolonged half-life ensures sustained high levels of factor activity, maintaining normal to near-normal ranges for the majority of the week, facilitating the convenience of once-weekly administration. Efanesoctocog alfa received regulatory approval in 2023 for application in both adults and children with inherited hemophilia A in the United States and Japan. Its sanctioned use encompasses both prophylaxis and 'on demand' treatment for bleeding episodes. The European Medicines Agency (EMA) is currently undertaking a comprehensive review of Altuviiio. TM This comprehensive review focuses on the immunological profile of efanesoctocog alfa, a highly sophisticated new class of EHL FVIII molecule. The integration of the vWF D'D3 domain, XTEN polypeptides, and potential regulatory T-cell epitopes within various segments of efanesoctocog alfa collectively serves as a mitigating factor against the development of a neutralizing T-cell-mediated immune response. We hypothesize that such distinctive attribute may significantly reduce the risk of neutralizing antibodies, particularly in previously untreated patients. The discussion extends beyond regulatory approval to encompass the preclinical and clinical development of efanesoctocog alfa, including considerations for laboratory monitoring. 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引用次数: 0
摘要
Efanesoctocog alfa(ALTUVIIIOTM,赛诺菲-SOBI)是一种 B 结构域缺失的单链因子 VIII(FVIII),与 von Willebrand Factor(VWF)的 D'D3 结构域相连。其巧妙的设计使 efanesoctocog alfa 能够独立于内源性 VWF 运行,与标准和延长半衰期(EHL)的 FVIII 产品相比,半衰期延长了 3-4 倍。延长的半衰期可确保持续的高水平因子活性,在一周的大部分时间内维持正常或接近正常的范围,为一周一次的给药提供了便利。Efanesoctocog alfa 于 2023 年获得监管部门批准,可用于美国和日本的成人和儿童遗传性 A 型血友病患者。其批准用途包括预防和按需治疗出血发作。欧洲药品管理局(EMA)目前正在对 ALTUVIIIOTM 进行全面审查。此次全面审查的重点是 efanesoctocog alfa 的免疫学特征,这是一种高度复杂的新型 EHL FVIII 分子。VWF D'D3 结构域、XTEN 多肽和 efanesoctocog alfa 不同片段中潜在的调节性 T 细胞表位的整合共同起到了减轻 T 细胞介导的中和免疫反应的作用。我们假设,这种独特的属性可能会大大降低产生中和抗体的风险,尤其是在以前未接受过治疗的患者中。讨论范围超出了监管审批,涵盖了依法尼辛可克α的临床前和临床开发,包括实验室监测的注意事项。综述还强调了需要进一步研究的领域,以加深我们对这种突破性治疗药物的了解。
Efanesoctocog alfa: the renaissance of Factor VIII replacement therapy.
Efanesoctocog alfa (Altuviiio,TM Sanofi-SOBI) is a B domain-deleted single-chain Factor VIII (FVIII) connected to D'D3 domain of von Willebrand Factor (vWF). Its ingenious design allows efanesoctocog alfa to operate independently of endogenous vWF and results in an outstanding 3-4 times longer half-life compared to standard and extended half-life (EHL) FVIII products. The prolonged half-life ensures sustained high levels of factor activity, maintaining normal to near-normal ranges for the majority of the week, facilitating the convenience of once-weekly administration. Efanesoctocog alfa received regulatory approval in 2023 for application in both adults and children with inherited hemophilia A in the United States and Japan. Its sanctioned use encompasses both prophylaxis and 'on demand' treatment for bleeding episodes. The European Medicines Agency (EMA) is currently undertaking a comprehensive review of Altuviiio. TM This comprehensive review focuses on the immunological profile of efanesoctocog alfa, a highly sophisticated new class of EHL FVIII molecule. The integration of the vWF D'D3 domain, XTEN polypeptides, and potential regulatory T-cell epitopes within various segments of efanesoctocog alfa collectively serves as a mitigating factor against the development of a neutralizing T-cell-mediated immune response. We hypothesize that such distinctive attribute may significantly reduce the risk of neutralizing antibodies, particularly in previously untreated patients. The discussion extends beyond regulatory approval to encompass the preclinical and clinical development of efanesoctocog alfa, including considerations for laboratory monitoring. The review also highlights areas that warrant further investigation to deepen our understanding of this groundbreaking therapeutic agent.
期刊介绍:
Haematologica is a journal that publishes articles within the broad field of hematology. It reports on novel findings in basic, clinical, and translational research.
Scope:
The scope of the journal includes reporting novel research results that:
Have a significant impact on understanding normal hematology or the development of hematological diseases.
Are likely to bring important changes to the diagnosis or treatment of hematological diseases.