B-cell subsets imbalance and reduced expression of CD40 in ataxia-telangiectasia patients
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C.T.M. Pereira [1] ; D.C. Bichuetti-Silva [1] ; N.V.F. da Mota [1] ; R. Salomão [1] ; M.K.C. Brunialti [1] ; B.T. Costa-Carvalho [1]
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[1] Universidade Federal de São Paulo
Universidade Federal de São Paulo
Brasil
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- Localización: Allergologia et immunopathologia: International journal for clinical and investigate allergology and clinical immunology, ISSN-e 1578-1267, ISSN 0301-0546, Vol. 46, Nº. 5, 2018, págs. 438-446
- Idioma: inglés
- Texto completo no disponible (Saber más ...)
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Resumen
Background Ataxia-telangiectasia (AT) is a well-known primary immunodeficiency with recurrent sinopulmonary infections and variable abnormalities in both the humoral and cellular immune system. Dysfunctions in immunoglobulin production, reduced number of B cells, and B-cell receptor excision circles copies have been reported. We aimed to understand the immunological mechanisms involving the humoral compartment in AT patients by analysing peripheral blood B cells subsets, B-T lymphocyte cooperation through the expression of CD40 and CD40 ligand (CD40L), and cytokines involved in class-switch recombination production.
Methods We compared the proportion of B-cell subsets, the expression of CD40/CD40L, and the plasma levels of IL-6 and IFN-γ of 18 AT patients and 15 healthy age-sex-matched controls using flow cytometry.
Results We found that some steps in peripheral B cell development were altered in AT with a pronounced reduction of cell-surface CD40 expression. The proportions of transitional and naïve-mature B cells were reduced, whereas CD21-low, natural effector memory, IgM-only memory, and IgG atypical memory B cells were present in a higher proportion.
Conclusions These findings revealed a disturbed B-cell homeostasis with unconventional maturation of B lymphocyte memory cells, which can explain the consequent impairment of humoral immunity.
