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依艾凝血因子VIII:凝血因子VIII替代疗法的复兴。

Efanesoctocog alfa: the renaissance of Factor VIII replacement therapy.

作者信息

Dargaud Yesim, Leuci Alexandre, Ruiz Alejandra Reyes, Lacroix-Desmazes Sebastien

机构信息

French Reference Center for Haemophilia, Clinical Haemostasis Unit, Hopital Louis Pradel, Lyon, France; UR4609 Research Unit on Haemostasis and Thrombosis, University Claude Bernard Lyon 1, Lyon.

UR4609 Research Unit on Haemostasis and Thrombosis, University Claude Bernard Lyon 1, Lyon.

出版信息

Haematologica. 2024 Aug 1;109(8):2436-2444. doi: 10.3324/haematol.2023.284498.

Abstract

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.

摘要

依艾凝血因子α(Altuviiio,赛诺菲-索比公司)是一种B结构域缺失的单链凝血因子VIII(FVIII),与血管性血友病因子(vWF)的D'D3结构域相连。其巧妙的设计使依艾凝血因子α能够独立于内源性vWF发挥作用,与标准和延长半衰期(EHL)的FVIII产品相比,半衰期延长了3至4倍,十分突出。半衰期的延长确保了因子活性的持续高水平,在一周的大部分时间内维持正常至接近正常的范围,便于每周一次给药。依艾凝血因子α于2023年在美国和日本获得监管批准,用于治疗患有遗传性血友病A的成人和儿童。其获批用途包括预防和出血发作时的“按需”治疗。欧洲药品管理局(EMA)目前正在对Altuviiio进行全面审查。此次全面审查聚焦于依艾凝血因子α的免疫特性,这是一种高度复杂的新型EHL FVIII分子。依艾凝血因子α各个片段中vWF D'D3结构域、XTEN多肽和潜在调节性T细胞表位的整合共同起到减轻中和性T细胞介导免疫反应发生的作用。我们推测,这种独特属性可能会显著降低产生中和抗体的风险,尤其是在未经治疗的患者中。讨论不仅限于监管批准,还涵盖了依艾凝血因子α的临床前和临床开发,包括实验室监测方面的考虑。审查还强调了一些值得进一步研究的领域,以加深我们对这种开创性治疗药物的理解。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9a87/11290510/61cdb255a591/1092436.fig1.jpg

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