B-cell and T-cell phenotypes in CVID patients correlate with the clinical phenotype of the disease

J Clin Immunol. 2010 Sep;30(5):746-55. doi: 10.1007/s10875-010-9424-3. Epub 2010 May 1.


Background: Common variable immunodeficiency (CVID) is a heterogeneous disorder characterized by recurrent infections and defective immunoglobulin production.

Methods: The DEFI French national prospective study investigated peripheral T-cell and B-cell compartments in 313 CVID patients grouped according to their clinical phenotype, using flow cytometry.

Results: In patients developing infection only (IO), the main B-cell or T-cell abnormalities were a defect in switched memory B cells and a decrease in naive CD4(+) T cells associated with an increase in CD4(+)CD95(+) cells. These abnormalities were more pronounced in patients developing lymphoproliferation (LP), autoimmune cytopenia (AC), or chronic enteropathy (CE). Moreover, LP and AC patients presented an increase in CD21(low) B cells and CD4(+)HLA-DR(+) T cells and a decrease in regulatory T cells.

Conclusion: In these large series of CVID patients, the major abnormalities of the B-cell and T-cell compartments, although a hallmark of CVID, were only observed in half of the IO patients and were more frequent and severe in patients with additional lymphoproliferative, autoimmune, and digestive complications.

Publication types

  • Multicenter Study
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Aged
  • B-Lymphocytes / immunology*
  • B-Lymphocytes / metabolism
  • B-Lymphocytes / pathology
  • CD4 Antigens / biosynthesis
  • Cell Separation
  • Common Variable Immunodeficiency / complications
  • Common Variable Immunodeficiency / immunology*
  • Common Variable Immunodeficiency / pathology
  • Common Variable Immunodeficiency / physiopathology
  • Disease Progression
  • Female
  • Flow Cytometry
  • France
  • Humans
  • Immunoglobulin Class Switching / drug effects
  • Immunologic Memory / drug effects
  • Immunophenotyping
  • Infections / etiology
  • Infections / immunology*
  • Infections / pathology
  • Infections / physiopathology
  • Lymphocyte Subsets / immunology*
  • Lymphocyte Subsets / metabolism
  • Lymphopenia
  • Male
  • Middle Aged
  • Protein-Losing Enteropathies
  • T-Lymphocytes / immunology*
  • T-Lymphocytes / metabolism
  • T-Lymphocytes / pathology
  • fas Receptor / biosynthesis


  • CD4 Antigens
  • fas Receptor