%A Albuquerque,Adriana S. %A Fernandes,Susana M. %A Tendeiro,Rita %A Cheynier,Rémi %A Lucas,Margarida %A Silva,Susana L. %A Victorino,Rui M. M. %A Sousa,Ana E. %D 2017 %J Frontiers in Immunology %C %F %G English %K Primary immunodeficiency,chronic granulomatous disease,Genetic Phagocytic Defect,Reactive oxigen species,CD4 T-cell Lymphopenia,Immune senescence,Gut mucosa,Interleukin-17. %Q %R 10.3389/fimmu.2017.00543 %W %L %M %P %7 %8 2017-May-11 %9 Case Report %+ Prof Ana E. Sousa,Instituto de Medicina Molecular, Faculdade de Medicina, Universidade de Lisboa,Portugal,asousa@medicina.ulisboa.pt %+ Prof Ana E. Sousa,Centro de Imunodeficiência Primárias de Lisboa,Portugal,asousa@medicina.ulisboa.pt %# %! T-cell disturbances in oxidative burst defects %* %< %T Major CD4 T-Cell Depletion and Immune Senescence in a Patient with Chronic Granulomatous Disease %U https://www.frontiersin.org/articles/10.3389/fimmu.2017.00543 %V 8 %0 JOURNAL ARTICLE %@ 1664-3224 %X 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.