ArticleFrontiers in immunology2018
Identification of CVID Patients With Defects in Immune Repertoire Formation or Specification.
Article in Frontiers in immunology, 2018. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 16 papers.
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Who cites it
16 citing papers in PubMed, 26 citations in OpenAlex.
- Failure of B Cell Tolerance in CVID.Frontiers in immunology · 2019Trial
- Antibody Secretion Capacity in CVID Patients: Immunoglobulin Isotypes and Antigen Specificities After T-Cell-Dependent In Vitro Stimulation.Journal of clinical medicine · 2025Article
- Article
- Sequencing the B Cell Receptor Repertoires of Antibody-Deficient Individuals With and Without Infection Susceptibility.Journal of clinical immunology · 2023Article
- B-cell receptor repertoire sequencing: Deeper digging into the mechanisms and clinical aspects of immune-mediated diseases.iScience · 2022Review
- Turning walking pneumonia into recurrent abscesses: a curious case of CVID and review of the literature.Allergy, asthma, and clinical immunology : official journal of the Canadian Society of Allergy and Clinical Immunology · 2022Article
- NGS-Based B-Cell Receptor Repertoire AnalysisRepertoire analyses in the Context of Inborn Errors of Immunity.Methods in molecular biology (Clifton, N.J.) · 2022Article
- Members of the Regulatory Lymphocyte Club in Common Variable Immunodeficiency.Frontiers in immunology · 2022Review
- Deciphering imprints of impaired memory B-cell maturation in germinal centers of three patients with common variable immunodeficiency.Frontiers in immunology · 2022Article
- Decreased ATM Function Causes Delayed DNA Repair and Apoptosis in Common Variable Immunodeficiency Disorders.Journal of clinical immunology · 2021Article
- Inborn Errors of Adaptive Immunity in Down Syndrome.Journal of clinical immunology · 2020Review
- Primary Humoral Immune Deficiencies: Overlooked Mimickers of Chronic Immune-Mediated Gastrointestinal Diseases in Adults.International journal of molecular sciences · 2020Review
- Defective formation of IgA memory B cells, Th1 and Th17 cells in symptomatic patients with selective IgA deficiency.Clinical & translational immunology · 2020Article
- Recent advances in elucidating the genetics of common variable immunodeficiency.Genes & diseases · 2020Review
- Flow Cytometry for Diagnosis of Primary Immune Deficiencies-A Tertiary Center Experience From North India.Frontiers in immunology · 2019Review
- Article
Corrections and comments
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Authors and funding
9 authors at 2 institutions in 2 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Common variable immune deficiency disorder (CVID) is the most clinically relevant cause of antibody failure. It is a highly heterogeneous disease with different underlying etiologies. CVID has been associated with a quantitative B cell defect, however, little is known about the quality of B cells present. Here, we studied the naïve and antigen selected B-cell receptor (BCR) repertoire in 33 CVID patients using next generation sequencing, to investigate B cells quality. Analysis for each individual patient revealed whether they have a defect in immune repertoire formation [V(D)J recombination] or specification (somatic hypermutation, subclass distribution, or selection). The naïve BCR repertoire was normal in most of the patients, although alterations in repertoire diversity and the junctions were found in a limited number of patients indicating possible defects in early B-cell development or V(D)J recombination in these patients. In contrast, major differences were found in the antigen selected BCR repertoire. Here, most patients (15/17) showed a reduced frequency of somatic hypermutation (SHM), changes in subclass distribution and/or minor alterations in antigen selection. Together these data show that in our CVID cohort only a small number of patients have a defect in formation of the naïve BCR repertoire, whereas the clear majority of patients have disturbances in their antigen selected repertoire, suggesting a defect in repertoire specification in the germinal centers of these patients. This highlights that CVID patients not only have a quantitative B cell defect, but that also the quality of, especially post germinal center B cells, is impaired.
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