Persistent hypogammaglobulinemia after rituximab therapy in pediatric patients, prevalence and clinical outcomes

Hypogammaglobulinemia is a known side effect of rituximab (RTX) in adults, but its prevalence and persistence in children remain underexplored. This retrospective cohort study at a tertiary care center examines the prevalence and clinical outcomes of hypogammaglobulinemia in pediatric patients after...

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Main Authors: Susanna P.C. Höppener, Saskia R. Veldkamp, Mark C.H. de Groot, Saskia Haitjema, Julia Drylewicz, Jaap Jan Boelens, Caroline A. Lindemans, Joris van Montfrans, Annet van Royen-Kerkhof, Marc H.A. Jansen
Format: Article
Language:English
Published: Elsevier 2025-06-01
Series:Clinical Immunology Communications
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Online Access:http://www.sciencedirect.com/science/article/pii/S2772613425000046
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author Susanna P.C. Höppener
Saskia R. Veldkamp
Mark C.H. de Groot
Saskia Haitjema
Julia Drylewicz
Jaap Jan Boelens
Caroline A. Lindemans
Joris van Montfrans
Annet van Royen-Kerkhof
Marc H.A. Jansen
author_facet Susanna P.C. Höppener
Saskia R. Veldkamp
Mark C.H. de Groot
Saskia Haitjema
Julia Drylewicz
Jaap Jan Boelens
Caroline A. Lindemans
Joris van Montfrans
Annet van Royen-Kerkhof
Marc H.A. Jansen
author_sort Susanna P.C. Höppener
collection DOAJ
description Hypogammaglobulinemia is a known side effect of rituximab (RTX) in adults, but its prevalence and persistence in children remain underexplored. This retrospective cohort study at a tertiary care center examines the prevalence and clinical outcomes of hypogammaglobulinemia in pediatric patients after RTX therapy. Patients aged ≤ 18 years treated with RTX for various indications between 2000 and 2020 were included. Patients were classified as having hypogammaglobulinemia when (1) IgG levels were <-2SD below reference for age, or (2) when they received immunoglobulin replacement therapy (IGRT) for the indication hypogammaglobulinemia. Hypogammaglobulinemia after RTX treatment was observed in 74/134 patients (55.2 %). Persistent hypogammaglobulinemia (>6 months) was observed in 46/91 patients (50.5 %), of whom 9 patients remained hypogammaglobulinemic >5 years. Low baseline IgG and IgM levels were significantly associated with persistent hypogammaglobulinemia, while patients receiving RTX therapy for autoimmune diseases were less frequently affected. CD19+ B cells reconstituted in a median of 11 months (IQR=[7.3–18.0]), while CD19+CD27+IgG+ switched memory B cells took significantly longer, with a median of 1.8 years (IQR=[1.0–2.9]). Three patients developed class-switch recombination-deficiencies and never recovered. Recurrent infections, of which two fatal, were recorded in 18 patients and were significantly more prevalent in those with persistent hypogammaglobulinemia. In conclusion, over half of children had low IgG levels and/or required IGRT for hypogammaglobulinemia following RTX therapy. Persistent hypogammaglobulinemia was associated with low pre-RTX IgG and/or IgM levels. Children with hypogammaglobulinemia after RTX are often IGRT-dependent, experience recurrent (and sometimes fatal) infections, and may develop secondary immunoglobulin class-switch defects.
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spelling doaj-art-2789cceee24d43b8b255d8f48636daeb2025-08-20T02:31:00ZengElsevierClinical Immunology Communications2772-61342025-06-017556310.1016/j.clicom.2025.04.001Persistent hypogammaglobulinemia after rituximab therapy in pediatric patients, prevalence and clinical outcomesSusanna P.C. Höppener0Saskia R. Veldkamp1Mark C.H. de Groot2Saskia Haitjema3Julia Drylewicz4Jaap Jan Boelens5Caroline A. Lindemans6Joris van Montfrans7Annet van Royen-Kerkhof8Marc H.A. Jansen9Pediatric Rheumatology and Immunology, Wilhelmina Children’s Hospital, University Medical Center Utrecht, Utrecht, the NetherlandsCenter for Translational Immunology, University Medical Center Utrecht, Utrecht University, the NetherlandsCentral Diagnostic Laboratory, University Medical Center Utrecht, Utrecht, the NetherlandsCentral Diagnostic Laboratory, University Medical Center Utrecht, Utrecht, the NetherlandsCenter for Translational Immunology, University Medical Center Utrecht, Utrecht University, the NetherlandsTransplantation and Cellular Therapies, Memorial Sloan Kettering Cancer Center, New York, NY, USAPediatric Rheumatology and Immunology, Wilhelmina Children’s Hospital, University Medical Center Utrecht, Utrecht, the Netherlands; Blood and Bone Marrow Transplantation, Princess Máxima Center for Pediatric Oncology, Utrecht, the NetherlandsPediatric Rheumatology and Immunology, Wilhelmina Children’s Hospital, University Medical Center Utrecht, Utrecht, the NetherlandsPediatric Rheumatology and Immunology, Wilhelmina Children’s Hospital, University Medical Center Utrecht, Utrecht, the NetherlandsPediatric Rheumatology and Immunology, Wilhelmina Children’s Hospital, University Medical Center Utrecht, Utrecht, the Netherlands; Corresponding author at: Suite KC.03.064.1, Postbus 85090, 3508 AB Utrecht, The Netherlands.Hypogammaglobulinemia is a known side effect of rituximab (RTX) in adults, but its prevalence and persistence in children remain underexplored. This retrospective cohort study at a tertiary care center examines the prevalence and clinical outcomes of hypogammaglobulinemia in pediatric patients after RTX therapy. Patients aged ≤ 18 years treated with RTX for various indications between 2000 and 2020 were included. Patients were classified as having hypogammaglobulinemia when (1) IgG levels were <-2SD below reference for age, or (2) when they received immunoglobulin replacement therapy (IGRT) for the indication hypogammaglobulinemia. Hypogammaglobulinemia after RTX treatment was observed in 74/134 patients (55.2 %). Persistent hypogammaglobulinemia (>6 months) was observed in 46/91 patients (50.5 %), of whom 9 patients remained hypogammaglobulinemic >5 years. Low baseline IgG and IgM levels were significantly associated with persistent hypogammaglobulinemia, while patients receiving RTX therapy for autoimmune diseases were less frequently affected. CD19+ B cells reconstituted in a median of 11 months (IQR=[7.3–18.0]), while CD19+CD27+IgG+ switched memory B cells took significantly longer, with a median of 1.8 years (IQR=[1.0–2.9]). Three patients developed class-switch recombination-deficiencies and never recovered. Recurrent infections, of which two fatal, were recorded in 18 patients and were significantly more prevalent in those with persistent hypogammaglobulinemia. In conclusion, over half of children had low IgG levels and/or required IGRT for hypogammaglobulinemia following RTX therapy. Persistent hypogammaglobulinemia was associated with low pre-RTX IgG and/or IgM levels. Children with hypogammaglobulinemia after RTX are often IGRT-dependent, experience recurrent (and sometimes fatal) infections, and may develop secondary immunoglobulin class-switch defects.http://www.sciencedirect.com/science/article/pii/S2772613425000046HypogammaglobulinemiaRituximabPediatricB cell reconstitution
spellingShingle Susanna P.C. Höppener
Saskia R. Veldkamp
Mark C.H. de Groot
Saskia Haitjema
Julia Drylewicz
Jaap Jan Boelens
Caroline A. Lindemans
Joris van Montfrans
Annet van Royen-Kerkhof
Marc H.A. Jansen
Persistent hypogammaglobulinemia after rituximab therapy in pediatric patients, prevalence and clinical outcomes
Clinical Immunology Communications
Hypogammaglobulinemia
Rituximab
Pediatric
B cell reconstitution
title Persistent hypogammaglobulinemia after rituximab therapy in pediatric patients, prevalence and clinical outcomes
title_full Persistent hypogammaglobulinemia after rituximab therapy in pediatric patients, prevalence and clinical outcomes
title_fullStr Persistent hypogammaglobulinemia after rituximab therapy in pediatric patients, prevalence and clinical outcomes
title_full_unstemmed Persistent hypogammaglobulinemia after rituximab therapy in pediatric patients, prevalence and clinical outcomes
title_short Persistent hypogammaglobulinemia after rituximab therapy in pediatric patients, prevalence and clinical outcomes
title_sort persistent hypogammaglobulinemia after rituximab therapy in pediatric patients prevalence and clinical outcomes
topic Hypogammaglobulinemia
Rituximab
Pediatric
B cell reconstitution
url http://www.sciencedirect.com/science/article/pii/S2772613425000046
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