Molecular therapy : the journal of the American Society of Gene Therapy · 2026
Safety and efficacy of hemobeglogene autotemcel(hemo-cel) gene therapy in five patients with transfusion-dependent β-thalassemia.
СтатьяКлиническое исследование I фазыКлиническое исследование II фазыНазвание и аннотация: Перевод в очереди
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Liu Sixi, Chen Yue, Li Yue, Qian Linxia, Dong Guoyi, Zhang Qian, Zhou Xiaohui, Guo Honglian, Sun Haigang, Lin Qihong, Yu Jiaming, Zeng Xinru, Zou Huilin, He Jiajun, Yao Wenwen, Ouyang Wenjie, Liu Chao
Аннотация
Lentiviral vector-mediated hematopoietic stem cell gene therapy (HSC-GT) has emerged as a curative alternative to allogeneic transplantation for patients with transfusion-dependent β-thalassemia (TDT). However, challenges such as delayed platelet engraftment, variable transgene expression, and limited long-term safety data have hindered its broader implementation. Here, we report the safety and efficacy outcomes from a phase 1/2 clinical study evaluating hemobeglogene autotemcel (hemo-cel), an HSC-GT product developed with an optimized lentiviral vector encoding HBBT87Q and an improved manufacturing process. Five pediatric TDT patients, including three with β0/β0 genotypes, received hemo-cel and were followed for a median of 34.7 months. Hemo-cel infusion led to rapid neutrophil (median, day 18) and platelet (median, day 13) engraftment. No unexpected adverse events, clonal dominance, or replication-competent lentivirus were observed. All patients achieved transfusion independence for over 26 months (median, 34.3 months), with sustained HbAT87Q levels and vector copy numbers. Hemo-cel also contributed to iron overload control and supported normal growth in height and weight. These findings suggest that hemo-cel is a safe and effective option for TDT patients across diverse genotypes, including the most severe β0/β0.