Major CD4 T-cell depletion and immune senescence in a patient with chronic granulomatous disease

Detalhes bibliográficos
Autor(a) principal: Albuquerque, Adriana S.
Data de Publicação: 2017
Outros Autores: Fernandes, Susana M., Tendeiro, Rita, Cheynier, Rémi, Lucas, Margarida, Silva, Susana L., Victorino, Rui M. M., Sousa, Ana E.
Tipo de documento: Artigo
Idioma: eng
Título da fonte: Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)
Texto Completo: http://hdl.handle.net/10451/28767
Resumo: Copyright © 2017 Albuquerque, Fernandes, Tendeiro, Cheynier, Lucas, Silva, Victorino and Sousa. This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) or licensor are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.
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spelling Major CD4 T-cell depletion and immune senescence in a patient with chronic granulomatous diseasePrimary immunodeficiencyChronic granulomatous diseaseGenetic phagocytic defectReactive oxygen speciesCD4 T-cell lymphopeniaImmune senescenceCopyright © 2017 Albuquerque, Fernandes, Tendeiro, Cheynier, Lucas, Silva, Victorino and Sousa. This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) or licensor are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.Chronic granulomatous disease (CGD) results from primary defects in phagocytic reactive oxygen species (ROS) production. T-cell evaluation is usually neglected during patients' follow-up, although T-cell depletion has been reported in CGD through unknown mechanisms. We describe here a 36-year-old patient with X-linked CGD with severe CD4 T-cell depletion <200 CD4 T-cells/μl, providing insights into the mechanisms that underlie T-cell loss in the context of oxidative burst defects. In addition to the typical infections, the patient featured a progressive T-cell loss associated with persistent lymphocyte activation, expansion of interleukin (IL)-17-producing CD4 T-cells, and impaired thymic activity, leading to a reduced replenishment of the T-cell pool. A relative CD4 depletion was also found at the gut mucosal level, although no bias to IL-17-production was documented. This immunological pattern of exhaustion of immune resources favors prompt, potentially curative, therapeutic interventions in CGD patients, namely, stem-cell transplantation or gene therapy. Moreover, this clinical case raises new research questions on the interplay of ROS production and T-cell homeostasis and immune senescence.AA, SF, RT, and SS received scholarships from Fundação para a Ciência e Tecnologia (FCT), Portugal.Frontiers MediaRepositório da Universidade de LisboaAlbuquerque, Adriana S.Fernandes, Susana M.Tendeiro, RitaCheynier, RémiLucas, MargaridaSilva, Susana L.Victorino, Rui M. M.Sousa, Ana E.2017-08-24T13:12:11Z20172017-01-01T00:00:00Zinfo:eu-repo/semantics/publishedVersioninfo:eu-repo/semantics/articleapplication/pdfhttp://hdl.handle.net/10451/28767engFrontiers in Immunology | May 2017 | Volume 8 | Article 5431664-322410.3389/fimmu.2017.00543info:eu-repo/semantics/openAccessreponame:Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)instname:Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informaçãoinstacron:RCAAP2023-11-08T16:20:49Zoai:repositorio.ul.pt:10451/28767Portal AgregadorONGhttps://www.rcaap.pt/oai/openaireopendoar:71602024-03-19T21:44:55.936407Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) - Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informaçãofalse
dc.title.none.fl_str_mv Major CD4 T-cell depletion and immune senescence in a patient with chronic granulomatous disease
title Major CD4 T-cell depletion and immune senescence in a patient with chronic granulomatous disease
spellingShingle Major CD4 T-cell depletion and immune senescence in a patient with chronic granulomatous disease
Albuquerque, Adriana S.
Primary immunodeficiency
Chronic granulomatous disease
Genetic phagocytic defect
Reactive oxygen species
CD4 T-cell lymphopenia
Immune senescence
title_short Major CD4 T-cell depletion and immune senescence in a patient with chronic granulomatous disease
title_full Major CD4 T-cell depletion and immune senescence in a patient with chronic granulomatous disease
title_fullStr Major CD4 T-cell depletion and immune senescence in a patient with chronic granulomatous disease
title_full_unstemmed Major CD4 T-cell depletion and immune senescence in a patient with chronic granulomatous disease
title_sort Major CD4 T-cell depletion and immune senescence in a patient with chronic granulomatous disease
author Albuquerque, Adriana S.
author_facet Albuquerque, Adriana S.
Fernandes, Susana M.
Tendeiro, Rita
Cheynier, Rémi
Lucas, Margarida
Silva, Susana L.
Victorino, Rui M. M.
Sousa, Ana E.
author_role author
author2 Fernandes, Susana M.
Tendeiro, Rita
Cheynier, Rémi
Lucas, Margarida
Silva, Susana L.
Victorino, Rui M. M.
Sousa, Ana E.
author2_role author
author
author
author
author
author
author
dc.contributor.none.fl_str_mv Repositório da Universidade de Lisboa
dc.contributor.author.fl_str_mv Albuquerque, Adriana S.
Fernandes, Susana M.
Tendeiro, Rita
Cheynier, Rémi
Lucas, Margarida
Silva, Susana L.
Victorino, Rui M. M.
Sousa, Ana E.
dc.subject.por.fl_str_mv Primary immunodeficiency
Chronic granulomatous disease
Genetic phagocytic defect
Reactive oxygen species
CD4 T-cell lymphopenia
Immune senescence
topic Primary immunodeficiency
Chronic granulomatous disease
Genetic phagocytic defect
Reactive oxygen species
CD4 T-cell lymphopenia
Immune senescence
description Copyright © 2017 Albuquerque, Fernandes, Tendeiro, Cheynier, Lucas, Silva, Victorino and Sousa. This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) or licensor are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.
publishDate 2017
dc.date.none.fl_str_mv 2017-08-24T13:12:11Z
2017
2017-01-01T00:00:00Z
dc.type.status.fl_str_mv info:eu-repo/semantics/publishedVersion
dc.type.driver.fl_str_mv info:eu-repo/semantics/article
format article
status_str publishedVersion
dc.identifier.uri.fl_str_mv http://hdl.handle.net/10451/28767
url http://hdl.handle.net/10451/28767
dc.language.iso.fl_str_mv eng
language eng
dc.relation.none.fl_str_mv Frontiers in Immunology | May 2017 | Volume 8 | Article 543
1664-3224
10.3389/fimmu.2017.00543
dc.rights.driver.fl_str_mv info:eu-repo/semantics/openAccess
eu_rights_str_mv openAccess
dc.format.none.fl_str_mv application/pdf
dc.publisher.none.fl_str_mv Frontiers Media
publisher.none.fl_str_mv Frontiers Media
dc.source.none.fl_str_mv reponame:Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)
instname:Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informação
instacron:RCAAP
instname_str Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informação
instacron_str RCAAP
institution RCAAP
reponame_str Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)
collection Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)
repository.name.fl_str_mv Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) - Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informação
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