Albuquerque, AS;
Fernandes, SM;
Tendeiro, R;
Cheynier, R;
Lucas, M;
Silva, SL;
Victorino, RMM;
(2017)
Major CD4 T-Cell Depletion and Immune Senescence in a Patient with Chronic Granulomatous Disease.
Frontiers in Immunology
, 8
, Article 543. 10.3389/fimmu.2017.00543.
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Abstract
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.
| Type: | Article |
|---|---|
| Title: | Major CD4 T-Cell Depletion and Immune Senescence in a Patient with Chronic Granulomatous Disease |
| Open access status: | An open access version is available from UCL Discovery |
| DOI: | 10.3389/fimmu.2017.00543 |
| Publisher version: | https://doi.org/10.3389/fimmu.2017.00543 |
| Language: | English |
| Additional information: | © 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. |
| Keywords: | Primary immunodeficiency, chronic granulomatous disease, genetic phagocytic defect, reactive oxygen species, CD4 T-cell lymphopenia, immune senescence, gut mucosa, interleukin-17 |
| UCL classification: | UCL UCL > Provost and Vice Provost Offices UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Medical Sciences UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Medical Sciences > Div of Infection and Immunity |
| URI: | https://discovery.ucl.ac.uk/id/eprint/1562545 |
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