Long-Term Safety and Efficacy of Systemic DT-DEC01 Cell Therapy in Non-Ambulatory Duchenne Muscular Dystrophy Patients: a 24-Month Clinical Evaluation

Duchenne muscular dystrophy (DMD) is a severe X-linked disorder characterized by progressive muscle degeneration and premature mortality. This study evaluated the long-term safety and efficacy of DT-DEC01, a Dystrophin Expressing Chimeric (DEC) cell therapy, in non-ambulatory DMD patients following systemic intraosseous administration. Three non-ambulatory DMD patients aged 11 to 16 years, each carrying a different DMD mutation (deletion of exons 48-50, deletion of exon 52, or nonsense mutation), received DT-DEC01 at doses of 2 × 10⁶, 4 × 10⁶, and 6 × 10⁶ cells/kg, respectively, without immunosuppression. Safety assessment included monitoring of Adverse Events (AE), Serious Adverse Events (SAE), and Donor-Specific anti-HLA Antibodies (DSA). Efficacy evaluations included Performance of Upper Limb (PUL 2.0) test, grip strength, electromyography (EMG)-assessed Motor Unit Potential (MUP) duration, echocardiography, spirometry, and wristband-based arm movement quantification. No treatment-related AE, SAE, or DSA were detected through 24 months of follow-up. All three patients demonstrated measurable and sustained improvements across multiple functional domains following systemic DT-DEC01 administration. Patient-specific gains included improved cardiac parameters, prolonged MUP duration, enhanced respiratory capacity, and increased upper-limb strength. Notably, these improvements occurred in non-ambulatory patients - a disease stage typically associated with progressive cardiac and pulmonary decline, rather than functional recovery. These sustained functional benefits up to 24 months suggest that DT-DEC01 therapy may promote multisystem functional improvements in advanced DMD, independent of dystrophin mutation type or disease stage.

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Journal
Stem Cell Reviews and Reports
Published
2025-12-18
DOI
https://doi.org/10.1007/s12015-025-11039-8
Primary Topic
Muscle Physiology and Disorders
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article
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article

Long-Term Safety and Efficacy of Systemic DT-DEC01 Cell Therapy in Non-Ambulatory Duchenne Muscular Dystrophy Patients: a 24-Month Clinical Evaluation

Grzegorz Biegański, Jarosław Czarnota, Agnieszka Sobkowiak-Sobierajska, Katarzyna Bożyk et al.
Stem Cell Reviews and Reports
Muscle Physiology and Disorders
article

Long-Term Safety and Efficacy of Systemic DT-DEC01 Cell Therapy in Non-Ambulatory Duchenne Muscular Dystrophy Patients: a 24-Month Clinical Evaluation

Grzegorz Biegański, Jarosław Czarnota, Agnieszka Sobkowiak-Sobierajska, Katarzyna Bożyk, Anna Ziemiecka, Maria Siemionow, Jacek Wachowiak, Adam Niezgoda, Krzysztof Siemionow
article en

Abstract

Duchenne muscular dystrophy (DMD) is a severe X-linked disorder characterized by progressive muscle degeneration and premature mortality. This study evaluated the long-term safety and efficacy of DT-DEC01, a Dystrophin Expressing Chimeric (DEC) cell therapy, in non-ambulatory DMD patients following systemic intraosseous administration. Three non-ambulatory DMD patients aged 11 to 16 years, each carrying a different DMD mutation (deletion of exons 48-50, deletion of exon 52, or nonsense mutation), received DT-DEC01 at doses of 2 × 10⁶, 4 × 10⁶, and 6 × 10⁶ cells/kg, respectively, without immunosuppression. Safety assessment included monitoring of Adverse Events (AE), Serious Adverse Events (SAE), and Donor-Specific anti-HLA Antibodies (DSA). Efficacy evaluations included Performance of Upper Limb (PUL 2.0) test, grip strength, electromyography (EMG)-assessed Motor Unit Potential (MUP) duration, echocardiography, spirometry, and wristband-based arm movement quantification. No treatment-related AE, SAE, or DSA were detected through 24 months of follow-up. All three patients demonstrated measurable and sustained improvements across multiple functional domains following systemic DT-DEC01 administration. Patient-specific gains included improved cardiac parameters, prolonged MUP duration, enhanced respiratory capacity, and increased upper-limb strength. Notably, these improvements occurred in non-ambulatory patients - a disease stage typically associated with progressive cardiac and pulmonary decline, rather than functional recovery. These sustained functional benefits up to 24 months suggest that DT-DEC01 therapy may promote multisystem functional improvements in advanced DMD, independent of dystrophin mutation type or disease stage.

Stem Cell Reviews and ReportsVol. 22(2)
Poznan University of Medical Sciences (PL), University of Illinois Chicago (US), Allogene Therapeutics (United States) (US), United Therapeutics (United States) (US)
Openalex Percentile: Top 63%
Muscle Physiology and Disorders
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