Major CD4 T-Cell Depletion and Immune Senescence in a Patient with Chronic Granulomatous Disease

Albuquerque, Adriana S.; Fernandes, S.; Tendeiro, Rita; Cheynier, Rémi; Lucas, Margarida; Silva, Susana L.; Victorino, Rui M. M.; Sousa, Ana E.

doi:10.3389/fimmu.2017.00543

Cited by 12 publications

(4 citation statements)

References 42 publications

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“…We noticed unexpectedly that one of the five patients with chronic granulomatous disease (CGD), a phagocyte defect, had a low TREC count, an observation that was previously observed in some patients with CGD, which is thought to be due to T cell depletion (through unknown mechanisms) [43,44]. This finding supports our observation, and calls for additional comprehensive T cell evaluation and monitoring in these patients.…”

Section: Discussionsupporting

confidence: 87%

TREC and KREC profiling as a representative of thymus and bone marrow output in patients with various inborn errors of immunity

Dasouki

Jabr

Aldakheel

et al. 2020

Clinical and Experimental Immunology

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RT‐qPCR quantification analysis of TRECs and KRECs in gDNA of patients with many diverse molecularly confirmed Primary Immunodeficiency Diseases was performed. Our data suggest that in addition to classical SCID and agammaglobulinemia, the TREC/KREC assay may identify secondary targets including some patients with ZAP70 deficiency, DOCK8 deficiency, ataxia telangiectasia, Wiskott‐Aldrich and bare lymphocyte syndromes.

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Section: Discussionsupporting

confidence: 87%

TREC and KREC profiling as a representative of thymus and bone marrow output in patients with various inborn errors of immunity

Dasouki

Jabr

Aldakheel

et al. 2020

Clinical and Experimental Immunology

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“…1). Previous studies reported contradictory observations, where increased, reduced or not affected T cell numbers were shown (83)(84)(85)(86). The discrepancy between findings might be due to the differential treatment of patients at the time of analysis, different distribution of patients' age or causative mutations in p91 phox or p47 phox coding genes within cohorts.…”

Section: Discussionmentioning

confidence: 95%

Peripheral T cell populations are differentially affected in monogenic autoinflammatory syndromes and gout

Al¹,

Bruno²,

Röring³

et al. 2023

Preprint

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Purpose Both monogenic autoinflammatory syndromes, such as Chronic Granulomatous Disease (CGD) and Familial Mediterranean Fever (FMF), and the common inflammatory disease gout are characterized by episodes of sterile inflammatory attacks in the absence of an infection. While these disorders encompass distinct pathologies due to differentially affected metabolic pathways and inflammasome activation mechanisms, their common features are the excessive production of interleukin (IL)-1ß and innate immune cell hyperreactivity. On the other hand, the role of T cells and innate-like lymphocytes such as gamma delta (γδ) T cells in these pathologies is ill-defined. Methods In order to widen our understanding of T cell involvement in FMF, CGD and gout pathology we developed multicolour immunophenotyping panels for flow cytometry and functional assays to characterise gd T cells, as well as CD4 and CD8 T cell populations in terms of their cytokine production, activation status, memory or naive phenotypes, exhaustion status, homing receptor expression, and cytotoxic activity. Results Our study is the first deep immunophenotyping analysis of T cell populations in FMF, CGD and gout patients. We found that CGD affects the frequencies and activation status of T cells, while gout impairs cytokine production capacity of Vd2 T cells and reshapes homing receptor expression by T cells. FMF was characterized by only minor effects on T cell populations. Conclusion Autoinflammatory syndromes differentially affect T cell compartments. Future studies are warranted to assess whether these phenotypical changes are relevant for disease pathology.

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“…Reduced naive T cell pools are a characteristic feature of aging and may contribute to the impaired immune responses observed in elderly individuals 38 . Depletions in naive T cell populations, and the corresponding de cits in adaptive immune responses, have also been reported in in ammatory states like chronic hepatitis C infection 39 and chronic granulomatous disease 40 ; however, this phenomenon has been less well documented in solid organ transplantation. Additionally, it has been consistently observed that solid organ transplant recipients develop poor adaptive response to new antigens either during immunization or new infections -including SARS-CoV-2 mRNA vaccination [41][42][43] .…”

Section: Discussionmentioning

confidence: 99%

Longitudinal immune profiling of a SARS-CoV-2 reinfection in a solid organ transplant recipient

Klein

Trubin²,

Lu³

et al. 2021

Preprint

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The underlying immunologic deficiencies enabling SARS-CoV-2 reinfections are currently unknown. Here we describe a renal-transplant recipient who developed recurrent, symptomatic SARS-CoV-2 infection 7 months after primary infection. To elucidate the immunological mechanisms responsible for reinfection, we performed longitudinal profiling of cellular and humoral responses during both primary and recurrent SARS-CoV-2 infection. We found that the patient responded to the primary infection with transient, poor-quality adaptive immune responses that was further compromised by intervening treatment for acute rejection of the renal allograft prior to reinfection. Importantly, we identified the development of neutralizing antibodies and humoral memory responses prior to SARS-CoV-2 reinfection. However, these neutralizing antibodies failed to confer protection against reinfection, suggesting that additional factors are required for efficient prevention of SARS-CoV-2 reinfection. Further, we found no evidence supporting viral evasion of primary adaptive immune responses, suggesting that susceptibility to reinfection may be determined by host factors rather than pathogen adaptation.

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Major CD4 T-Cell Depletion and Immune Senescence in a Patient with Chronic Granulomatous Disease

Cited by 12 publications

References 42 publications

TREC and KREC profiling as a representative of thymus and bone marrow output in patients with various inborn errors of immunity

TREC and KREC profiling as a representative of thymus and bone marrow output in patients with various inborn errors of immunity

Peripheral T cell populations are differentially affected in monogenic autoinflammatory syndromes and gout

Longitudinal immune profiling of a SARS-CoV-2 reinfection in a solid organ transplant recipient

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