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#21483 : Exploration of persistant hypogammaglobulinemia after B-Cell depleting therapy in patients with idiopathic nephrotic syndrome in remission
Topics: Single Cells
Origin: Academic
Project type: Development

Name of Applicant: Noémie Blumenthal
Date of application: 05-06-2026
Unit: Department of Immunology
Location:
Phone:
@ Mail: noemie.blumenthal@aphp.fr
@ PI-Mail: guislaine.carcelain@aphp.fr

Project context and summary:

Over the past decade, B-cell depletion has become an increasingly used treatment to maintain remission without oral immunosupressive drugs in complicated frequently relapsing, or steroid-dependent nephrotic syndrome (FR/SDNS) with a good safety profile. However, hypogammaglobulinemia occurs in 14-50 % of patients during B-cell depletion and some patients experience prolonged hypogammaglobulinemia long after their B-cell levels have returned to the normal range. We hypothesize that patients with persistent hypogammaglobulinemia may exhibit alterations in B-cell homeostasis. Additionally, since T-follicular Helpers (TfH) play a critical role in B-cell maturation and differentiation into memory B-cells and plasma cells, we also investigated T-cell homeostasis. We compared levels of IgG, IgA, IgM, IgE and IgG subclasses, analyzed B-cell subpopulations and T-cell subpopulations using flow cytometry on fresh blood, and evaluated B cell differentiation capacity into antibody-secreting cells (ASC) following non-specific polyclonal stimulation by B-cell ELISpot assay. Further analyses are ongoing to better understand this mechanism: B cell cultures to investigate a potential impact on T-independent B-cell maturation pathways in controls, and single-cell RNA sequencing analysis on B and T cells to determine transcriptomic alterations linked to the suspected class-switching process impairment


Related team publications:
Parmentier, C. et al. Immunoglobulin serum levels in rituximab-treated patients with steroid-dependent nephrotic syndrome. Pediatr Nephrol 35, 455–462 (2020)
Colucci, M. et al. Prolonged Impairment of Immunological Memory After Anti-CD20 Treatment in Pediatric Idiopathic Nephrotic Syndrome. Front. Immunol. 10, (2019)
Marzuillo, P. et al. Rituximab-induced IgG hypogammaglobulinemia in children with nephrotic syndrome and normal pre-treatment IgG values. World J Clin Cases 7, 1021–1027 (2019)
Service Delivery
Manager: marc.monot@pasteur.fr
Status: Kick-off meeting


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