Diminished T Cell Numbers in Patients with Chronic Granulomatous Disease

Heltzer, Meredith; Jawad, Abbas F.; Rae, Julie; Curnutte, John T.; Sullivan, Kathleen E.

doi:10.1006/clim.2002.5291

Cited by 25 publications

(15 citation statements)

References 28 publications

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“…Interestingly, female carriers of X-linked chronic granulomatous disease also have increased risk of autoimmunity, including SLE, oral ulcers, Raynaud's phenomenon and arthritis, possibly related to tissue-specific X-inactivation. Although this is a neutriphil defect, B-cells and T-cells are impacted [76,77]. Neutrophil apoptosis is abnormal and appears to be associated with autoantibody production [78].…”

Section: Clearance Of Apoptotic Cellsmentioning

confidence: 99%

The intersection of immune deficiency and autoimmunity

Maggadóttir

Sullivan

2014

Current Opinion in Rheumatology

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Section: Clearance Of Apoptotic Cellsmentioning

confidence: 99%

The intersection of immune deficiency and autoimmunity

Maggadóttir

Sullivan

2014

Current Opinion in Rheumatology

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“…It has a frequency of 1:100,000 to 1:200,000 people. Patients generally are normal in all other respects, although there may be mildly diminished T-cell numbers (120). The NADPH oxidase complex is expressed at its highest levels in neutrophils, although it is also seen in monocytes, B cells, and fibroblasts.…”

Section: Functional Neutrophil Disordersmentioning

confidence: 99%

“…Nevertheless, significant infections with respiratory syncytial virus and adenovirus have been reported (295). This could reflect the diminished T-cell numbers seen in patients with CGD (120).…”

Section: Functional Neutrophil Disordersmentioning

confidence: 99%

Infections in Patients with Inherited Defects in Phagocytic Function

2003

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Patients with defects in phagocytic function are predisposed to intracellular microorganisms and typically have early dissemination of the infection. Recognition of the underlying disorder and aggressive antimicrobial therapy has been beneficial for the patients. Improved understanding of the pathophysiology has also affected patient management by allowing specific, targeted immunomodulatory intervention. The disorders described in this review are not common but have had a significant impact on our understanding of the role of phagocytic cells in host defense. Conversely, understanding the role of the neutrophil and macrophage in infection has benefited not just the patients described in this review but also other patients with similar disease processes

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“…It is due to genetic defects in nicotinamide adenine dinucleotide phosphate (NADPH) oxidase (1, 2), leading to impaired reactive oxygen species (ROS) production by monocytes and neutrophils, defective microorganism clearance, and chronic inflammation (1–4). Reduced T-cell numbers were reported in an American CGD cohort, although the underlying mechanisms remain unclear (5). The defective ROS production by myeloid cells indirectly contributes to the T-cell loss through the promotion of inflammation (1, 2).…”

Section: Introductionmentioning

confidence: 99%

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

et al. 2017

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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.

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Diminished T Cell Numbers in Patients with Chronic Granulomatous Disease

Cited by 25 publications

References 28 publications

The intersection of immune deficiency and autoimmunity

The intersection of immune deficiency and autoimmunity

Infections in Patients with Inherited Defects in Phagocytic Function

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

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