B-cell subsets imbalance and reduced expression of CD40 in ataxia-telangiectasia patients

Autor(es)

Pereira, C. T. M. ^[1] ; Bichuetti-Silva, D. C. ^[1] ; da Mota, N. V. F. ^[2] ; Salomao, R. ^[2] ; Brunialti, M. K. C. ^[2] ; Costa-Carvalho, B. T. ^[1]

Afiliação do(s) autor(es)

^[1] Univ Fed Sao Paulo, Dept Pediat, Med Sch, 598 Botucatu St, BR-04023062 Sao Paulo, SP - Brazil

^[2] Univ Fed Sao Paulo, Div Infect Dis, Med Sch, 669 Pedro de Toledo St, BR-04039032 Sao Paulo, SP - Brazil

• Tipo de documento: Artigo Científico
• Fonte: ALLERGOLOGIA ET IMMUNOPATHOLOGIA; v. 46, n. 5, p. 438-446, SEP-OCT 2018.
• Citações Web of Science: 2

Resumo

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 na ve-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. (C) 2018 SEICAP. Published by Elsevier Espana, S.L.U. All rights reserved. (AU)

• Processo FAPESP: 13/00786-4 - Estudo funcional dos linfócitos B: maturação, ativação e produção de citocinas em pacientes com ataxia-telangiectasia
• Beneficiário: Beatriz Tavares Costa Carvalho
• Modalidade de apoio: Auxílio à Pesquisa - Regular