B-cell subsets imbalance and reduced expression of CD40 in ataxia-telangiectasia patients
Allergol. immunopatol
; 46(5): 438-446, sept.-oct. 2018. tab, graf
Article
en En
| IBECS
| ID: ibc-177878
Biblioteca responsable:
ES1.1
Ubicación: BNCS
ABSTRACT
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-gamma 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
RESUMEN
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Colección:
06-national
/
ES
Base de datos:
IBECS
Asunto principal:
Ataxia Telangiectasia
/
Subgrupos de Linfocitos B
Límite:
Adolescent
/
Child
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Child, preschool
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Female
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Humans
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Male
Idioma:
En
Revista:
Allergol. immunopatol
Año:
2018
Tipo del documento:
Article