Diminished T cell numbers in patients with chronic granulomatous disease.

Chronic granulomatous disease is a neutrophil disorder in which phagocytic cells fail to produce a respiratory burst. Five genetic types of chronic granulomatous disease have been described and in each case the clinical manifestations relate to the inability to effectively kill catalase-positive organisms. It is classically described as a pure disorder of intracellular killing, with preservation of other aspects of phagocytic function such as migration and phagocytosis and normal function of nonmyeloid cells. This article describes a heretofore unrecognized feature of chronic granulomatous disease. Fifty-three patients with chronic granulomatous disease and 42 age-matched controls were studied by flow cytometry. Total T cell numbers and CD4 and CD8 T cell numbers were compared between patients and controls. Patients with chronic granulomatous disease had diminished T cell numbers compared to controls after 3 years of age. The difference increased with age. It is not known whether diminished T cell numbers influence the susceptibility to infections in these patients, but T cell effects could represent a significant cofactor for infection.

[1]  E. Zackai,et al.  Immunologic features of chromosome 22q11.2 deletion syndrome (DiGeorge syndrome/velocardiofacial syndrome). , 2001, The Journal of pediatrics.

[2]  M. Dinauer,et al.  The intracellular oxidation of 2',7'-dichlorofluorescin in murine T lymphocytes. , 2001, Free radical biology & medicine.

[3]  M. Gahr,et al.  Long-term follow-up and outcome of 39 patients with chronic granulomatous disease. , 2000, The Journal of pediatrics.

[4]  K. Schulze-Osthoff,et al.  Enhancement of T Cell Receptor Signaling by a Mild Oxidative Shift in the Intracellular Thiol Pool1 , 2000, The Journal of Immunology.

[5]  David A. Williams,et al.  Dominant negative mutation of the hematopoietic-specific Rho GTPase, Rac2, is associated with a human phagocyte immunodeficiency. , 2000, Blood.

[6]  R. C. van der Veen,et al.  Superoxide Prevents Nitric Oxide-Mediated Suppression of Helper T Lymphocytes: Decreased Autoimmune Encephalomyelitis in Nicotinamide Adenine Dinucleotide Phosphate Oxidase Knockout Mice1 , 2000, The Journal of Immunology.

[7]  Richard B. Johnston,et al.  Chronic Granulomatous Disease: Report on a National Registry of 368 Patients , 2000, Medicine.

[8]  S. Holland,et al.  Genetic, biochemical, and clinical features of chronic granulomatous disease. , 2000, Medicine.

[9]  G L Johnson,et al.  Human neutrophil immunodeficiency syndrome is associated with an inhibitory Rac2 mutation. , 2000, Proceedings of the National Academy of Sciences of the United States of America.

[10]  J. Schrenzel,et al.  A novel H+ conductance in eosinophils: Unique characteristics and absence in chronic granulomatous disease , 1999 .

[11]  P. Marrack,et al.  Reactive oxygen species regulate activation-induced T cell apoptosis. , 1999, Immunity.

[12]  Y. Suzuki,et al.  Involvement of reactive oxygen species produced via NADPH oxidase in tyrosine phosphorylation in human B- and T-lineage lymphoid cells. , 1999, Biochemical and biophysical research communications.

[13]  N. Tanaka,et al.  Involvement of apoptosis in activation-induced cell death of bacteria-reactive human CD45RO+ T cells. , 1999, Research communications in molecular pathology and pharmacology.

[14]  S. Chanock,et al.  Host defense molecule polymorphisms influence the risk for immune-mediated complications in chronic granulomatous disease. , 1998, The Journal of clinical investigation.

[15]  L. Zentilin,et al.  Nicotinamide-adenine dinucleotide phosphate oxidase assembly and activation in EBV-transformed B lymphoblastoid cell lines of normal and chronic granulomatous disease patients. , 1998, Journal of immunology.

[16]  M. Hurme,et al.  Pre-exposure to oxidative stress decreases the nuclear factor-kappa B-dependent transcription in T lymphocytes. , 1998, Journal of immunology.

[17]  K. Német,et al.  Regulation of Capacitative Ca2+ Influx in Human Neutrophil Granulocytes , 1997, The Journal of Biological Chemistry.

[18]  J. G. Scandalios,et al.  Oxidative stress and the molecular biology of antioxidant defenses. , 1997 .

[19]  D. Alling,et al.  Genotype-dependent variability in flow cytometric evaluation of reduced nicotinamide adenine dinucleotide phosphate oxidase function in patients with chronic granulomatous disease. , 1996, The Journal of pediatrics.

[20]  K. Schulze-Osthoff,et al.  IL‐2 gene expression and NF‐kappa B activation through CD28 requires reactive oxygen production by 5‐lipoxygenase. , 1995, The EMBO journal.

[21]  K. Schulze-Osthoff,et al.  Hydrogen peroxide as a potent activator of T lymphocyte functions , 1995, European journal of immunology.

[22]  N. Ganguly,et al.  Study of T-lymphocyte subpopulation in HBsAg-positive pregnant women. , 1993, Acta virologica.

[23]  S. Taniuchi,et al.  Decreased CD4+CD29+ (memory T) cells in patients with chronic granulomatous disease. , 1993, The Journal of infectious diseases.

[24]  S. Bhattacharya,et al.  PERIPHERAL T LYMPHOCYTES AND THEIR SUBSETS IN CUTANEOUS TUBERCULOSIS , 1992, International journal of dermatology.

[25]  C. Giaquinto,et al.  Age-related standards for T lymphocyte subsets based on uninfected children born to human immunodeficiency virus 1-infected women. The European Collaborative Study. , 1992, The Pediatric infectious disease journal.

[26]  A R Cross,et al.  Identification of a superoxide-generating NADPH oxidase system in human fibroblasts. , 1991, The Biochemical journal.

[27]  A. Bava,et al.  Lymphocyte Subpopulations and Cytokine Production in Paracoccidioidomycosis Patients , 1991, Microbiology and immunology.

[28]  John I. Gallin,et al.  A controlled trial of interferon gamma to prevent infection in chronic granulomatous disease. The International Chronic Granulomatous Disease Cooperative Study Group. , 1991, The New England journal of medicine.

[29]  W. Nauseef,et al.  Genetic variants of chronic granulomatous disease: prevalence of deficiencies of two cytosolic components of the NADPH oxidase system. , 1989, The New England journal of medicine.

[30]  A. D. de Weck,et al.  The superoxide generating system of B cell lines. Structural homology with the phagocytic oxidase and triggering via surface Ig. , 1988, Journal of immunology.

[31]  A. Monaco,et al.  Cloning the gene for the inherited disorder chronic granulomatous disease on the basis of its chromosomal location. , 1986, Cold Spring Harbor symposia on quantitative biology.

[32]  D. Roos,et al.  Continuous therapy with sulfamethoxazole-trimethoprim in patients with chronic granulomatous disease. , 1983, The Journal of pediatrics.