Abstract: Albutrepenonacog-alfa (Idelvion^®, CSL Behring) is a recombinant fusion protein in which the recombinant FIX (rFIX) links a recombinant human albumin, extending the half-life of rFIX even beyond 100 hours. In 2016, this drug was approved worldwide for the treatment of pediatric and adult persons with hemophilia B (PWH-B). Its efficacy and safety were described in the PROLONG-9FP program and subsequently confirmed in the real-world practice, even if to date there are not many manuscripts that extensively and completely deal with the use of albutrepenonacog-alfa in daily practice, also evaluating its impact on the quality of life of patients treated with this drug; this review therefore aims to analyze all the publications currently available regarding the real-world use of this extended half-life concentrate, also noting which topics need further study and research.

Keywords: hemophilia B, recombinant FIX, albutrepenonacog-alfa, albumin-fusion proteins, extended half-life concentrates


中文翻译：

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白蛋白融合重组 FIX 在 B 型血友病患者管理中的应用：循证综述

摘要： Albutrepenonacog-alfa (Idelvion ^® , CSL Behring) 是一种重组融合蛋白，其中重组 FIX (rFIX) 连接重组人白蛋白，将 rFIX 的半衰期延长至 100 小时以上。2016年，该药物在全球范围内被批准用于治疗患有B型血友病（PWH-B）的儿童和成人患者。其功效和安全性在 PROLONG-9FP 计划中进行了描述，并随后在现实世界的实践中得到了证实，即使迄今为止，没有多少手稿广泛而完整地处理 albutrepenonacog-alfa 在日常实践中的使用，也评估了其对接受该药物治疗的患者的生活质量的影响；因此，本次审查旨在分析目前有关这种延长半衰期浓缩物在现实世界中的使用的所有出版物，同时指出哪些主题需要进一步研究。

关键词： B 型血友病、重组 FIX、albutrepenonacog-alfa、清蛋白融合蛋白、延长半衰期浓缩物