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

Summary 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 co...

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Bibliographic Details
Published in:Clinical and experimental immunology 2012-11, Vol.170 (2), p.202-211
Main Authors: Bateman, E. A. L., Ayers, L., Sadler, R., Lucas, M., Roberts, C., Woods, A., Packwood, K., Burden, J., Harrison, D., Kaenzig, N., Lee, M., Chapel, H. M., Ferry, B. L.
Format: Article
Language:English
Subjects:
Adolescent
Adult
Agammaglobulinemia - immunology
Aged
Aged, 80 and over
Analytical, structural and metabolic biochemistry
Antigens, CD - immunology
B-Lymphocyte Subsets - immunology
Biological and medical sciences
Cell Differentiation - immunology
Child
Child, Preschool
Common Variable Immunodeficiency - immunology
CVID
Female
Fundamental and applied biological sciences. Psychology
Genetic Diseases, X-Linked - immunology
Humans
Immunodeficiencies
Immunodeficiencies. Immunoglobulinopathies
Immunoglobulin A - immunology
Immunoglobulin G - immunology
Immunopathology
Infant
Lymphocyte Activation - immunology
Lymphocytes
Male
Medical research
Medical sciences
memory cells
Middle Aged
Original
Phenotype
primary antibody deficiency
Receptors, CCR7 - immunology
T cells
T-Lymphocyte Subsets - immunology
XLA
Young Adult
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Summary:Summary 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.
ISSN:0009-9104
1365-2249
DOI:10.1111/j.1365-2249.2012.04643.x