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T-cell abnormalities in common variable immunodeficiency: the hidden defect

This review discusses how the T-cell compartment in common variable immunodeficiency is marked by the premature arrest in thymic output, leading to T-cell exhaustion and immune dysregulation. Although B cells have been the main focus of the disorder, ample experimental data suggest that T-cell abnor...

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Detalles Bibliográficos
Autores principales: Wong, Gabriel K, Huissoon, Aarnoud P
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BMJ Publishing Group 2016
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4975840/
https://www.ncbi.nlm.nih.gov/pubmed/27153873
http://dx.doi.org/10.1136/jclinpath-2015-203351
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author Wong, Gabriel K
Huissoon, Aarnoud P
author_facet Wong, Gabriel K
Huissoon, Aarnoud P
author_sort Wong, Gabriel K
collection PubMed
description This review discusses how the T-cell compartment in common variable immunodeficiency is marked by the premature arrest in thymic output, leading to T-cell exhaustion and immune dysregulation. Although B cells have been the main focus of the disorder, ample experimental data suggest that T-cell abnormalities can be seen in a large proportion of Freiburg Group 1a patients and those suffering from inflammatory complications. The reductions in T-cell receptor excision circles, naïve T cells, invariant NKT cells and regulatory T cells suggest a diminished thymic output, while CD8 T cells are driven towards exhaustion either via an antigen-dependent or an antigen-independent manner. The theoretical risk of anti-T-cell therapies is discussed, highlighting the need for an international effort in generating longitudinal data in addition to better-defined underlying molecular characterisation.
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spelling pubmed-49758402016-08-18 T-cell abnormalities in common variable immunodeficiency: the hidden defect Wong, Gabriel K Huissoon, Aarnoud P J Clin Pathol Review This review discusses how the T-cell compartment in common variable immunodeficiency is marked by the premature arrest in thymic output, leading to T-cell exhaustion and immune dysregulation. Although B cells have been the main focus of the disorder, ample experimental data suggest that T-cell abnormalities can be seen in a large proportion of Freiburg Group 1a patients and those suffering from inflammatory complications. The reductions in T-cell receptor excision circles, naïve T cells, invariant NKT cells and regulatory T cells suggest a diminished thymic output, while CD8 T cells are driven towards exhaustion either via an antigen-dependent or an antigen-independent manner. The theoretical risk of anti-T-cell therapies is discussed, highlighting the need for an international effort in generating longitudinal data in addition to better-defined underlying molecular characterisation. BMJ Publishing Group 2016-08 2016-05-06 /pmc/articles/PMC4975840/ /pubmed/27153873 http://dx.doi.org/10.1136/jclinpath-2015-203351 Text en Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://www.bmj.com/company/products-services/rights-and-licensing/ This is an Open Access article distributed in accordance with the terms of the Creative Commons Attribution (CC BY 4.0) license, which permits others to distribute, remix, adapt and build upon this work, for commercial use, provided the original work is properly cited. See: http://creativecommons.org/licenses/by/4.0/
spellingShingle Review
Wong, Gabriel K
Huissoon, Aarnoud P
T-cell abnormalities in common variable immunodeficiency: the hidden defect
title T-cell abnormalities in common variable immunodeficiency: the hidden defect
title_full T-cell abnormalities in common variable immunodeficiency: the hidden defect
title_fullStr T-cell abnormalities in common variable immunodeficiency: the hidden defect
title_full_unstemmed T-cell abnormalities in common variable immunodeficiency: the hidden defect
title_short T-cell abnormalities in common variable immunodeficiency: the hidden defect
title_sort t-cell abnormalities in common variable immunodeficiency: the hidden defect
topic Review
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4975840/
https://www.ncbi.nlm.nih.gov/pubmed/27153873
http://dx.doi.org/10.1136/jclinpath-2015-203351
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