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Abstract: TH-OR090

Safety and Efficacy of B Cell Maturation Antigen (BCMA)/CD70-Targeted Chimeric Antigen Receptor T Cell (CAR-T) Therapy in Pediatric Patients with Multidrug-Resistant, Steroid-Resistant Nephrotic Syndrome

Session Information

Category: Pediatric Nephrology

  • 1900 Pediatric Nephrology

Authors

  • Meng, Hanyan, Children's Hospital of Zhejiang University School of Medicine, Hangzhou, Zhejiang, China
  • Huang, Guoping, Children's Hospital of Zhejiang University School of Medicine, Hangzhou, Zhejiang, China
  • Liu, Fei, Children's Hospital of Zhejiang University School of Medicine, Hangzhou, Zhejiang, China
  • Zhang, Yingzi, Chongqing Precision Biotech Co., Ltd., Chongqing, China
  • He, Xue, Children's Hospital of Zhejiang University School of Medicine, Hangzhou, Zhejiang, China
  • Wang, Jingjing, Children's Hospital of Zhejiang University School of Medicine, Hangzhou, Zhejiang, China
  • Mao, Jianhua, Children's Hospital of Zhejiang University School of Medicine, Hangzhou, Zhejiang, China
Background

Patients with SRNS who could not achieve CR after 12 months of treatment with 2 mechanistically distinct steroid-sparing agents administered at standard doses are defined as MDR-SRNS. Currently, available treatments for MDR-SRNS are limited. Here we discuss the safety and efficacy of BCMA/CD70-targeted CAR T-Cell therapy in pediatric patients with MDR-SRNS.

Methods

In this Phase I/II study (NCT06553898), C-4-29 CAR T cells were manufactured by the PRIMCAR manufacturing platform. Per protocol, this study employed a dose-escalation and expansion design. Toxicity was graded by CTCAE V5.0, CRS and ICANS were graded by ASTCT criteria.

Results

By August 2025, 8 patients were infused with C-4-29 (Table 1). 4 experienced CRS, all at grade 1. No ICANS were observed. Among the 8 patients, 1 could not be evaluated for treatment efficacy due to the short duration since reinfusion. 4 achieved CR, and 2 attained PR. However, as of the latest assessment, 5 exhibited an increase in urinary protein excretion, suggesting disease relapse.

Conclusion

BCMA/CD70-targeted CAR T-Cell therapy demonstrates favorable safety profile in pediatric patients with MDR-SRNS while shedding light on disease pathogenesis.The expected completion time of this research is July 2027.

Disease characteristics and safety outcomes
IDAge,yearsGenderDisease duration, (months)Immunosuppressant agentsKidney biopsyDoseCRSICANS
17Male466MCD0.3×10^5/kg1/
212Female1276MCD0.3×10^5/kg//
314Male594FSGS0.3×10^5/kg1/
48Male514FSGS1.0×10^5/kg1/
513Female444FSGS1.0×10^5/kg//
67Female455FSGS1.0×10^5/kg1/
716Male1645FSGS1.0×10^5/kg//
98Female296FSGS1.0×10^5/kg//

Digital Object Identifier (DOI)