T cell phenotypes in patients with common variable immunodeficiency disorders: associations with clinical phenotypes in comparison with other groups with recurrent infections

Clin Exp Immunol. 2012 Nov;170(2):202-11. doi: 10.1111/j.1365-2249.2012.04643.x.


Common variable immunodeficiency disorders (CVID) are a group of heterogeneous conditions that have in common primary failure of B cell function, although numerous T cell abnormalities have been described, including reduced proliferative response and reduced regulatory T cells. This study compared the T cell phenotype of CVID patients subdivided into clinical phenotypes as well as patients with partial antibody deficiencies [immunoglobulin (Ig)G subclass deficiency and selective IgA deficiency], X-linked agammaglobulinaemia (XLA) and healthy and disease controls. Absolute numbers of T cell subpopulations were measured by four-colour flow cytometry: naive T cells, central and effector memory and terminally differentiated (TEM) T cells, using CD45RA and CCR7 expression. Early, intermediate and late differentiation status of T cells was measured by CD27/CD28 expression. Putative follicular T cells, recent thymic emigrants and regulatory T cells were also assessed. Significant reduction in naive CD4 T cells, with reduced total CD4 and recent thymic emigrant numbers, was observed in CVID patients, most pronounced in those with autoimmune cytopenias or polyclonal lymphoproliferation. These findings suggest a lack of replenishment by new thymically derived cells. CD8 naive T cells were reduced in CVID patients, most significantly in the autoimmune cytopenia subgroup. There was a reduction in early differentiated CD4 and CD8 T cells and increased CD8 TEM in the CVID patients, particularly autoimmune cytopenia and polyclonal lymphoproliferation subgroups, suggesting a more activated T cell phenotype, due perhaps to an antigen-driven process. XLA patients had significantly reduced putative follicular T cells, which may depend on B cells for survival, while no significant alterations were observed in the T cells of those with IgG subclass deficiency or selective IgA deficiency.

Publication types

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

MeSH terms

  • Adolescent
  • Adult
  • Agammaglobulinemia / immunology
  • Aged
  • Aged, 80 and over
  • Antigens, CD / immunology
  • B-Lymphocyte Subsets / immunology
  • Cell Differentiation / immunology
  • Child
  • Child, Preschool
  • Common Variable Immunodeficiency / immunology*
  • Female
  • Genetic Diseases, X-Linked / immunology
  • Humans
  • Immunoglobulin A / immunology
  • Immunoglobulin G / immunology
  • Infant
  • Lymphocyte Activation / immunology
  • Male
  • Middle Aged
  • Phenotype
  • Receptors, CCR7 / immunology
  • T-Lymphocyte Subsets / immunology*
  • Young Adult


  • Antigens, CD
  • CCR7 protein, human
  • Immunoglobulin A
  • Immunoglobulin G
  • Receptors, CCR7

Supplementary concepts

  • Bruton type agammaglobulinemia