title: Diminished T cell numbers in patients with chronic granulomatous disease.
abstract: 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.
authors.affiliation: St. Christopher's Hospital for Children, Philadelphia, Pennsylvania 19134, USA.
authors.forename: Meredith
authors.initials: M
authors.lastname: Heltzer
authors.forename: Abbas F
authors.initials: AF
authors.lastname: Jawad
authors.forename: Julie
authors.initials: J
authors.lastname: Rae
authors.forename: John T
authors.initials: JT
authors.lastname: Curnutte
authors.forename: Kathleen E
authors.initials: KE
authors.lastname: Sullivan
mesh_terms: D000163:Acquired Immunodeficiency Syndrome; D000293:Adolescent; D000328:Adult; D000367:Age Factors; D002648:Child; D002675:Child, Preschool; D005434:Flow Cytometry; D006105:Granulomatous Disease, Chronic / Q000235:genetics / Q000276:immunology*; D006801:Humans; D007223:Infant; D007958:Leukocyte Count; D012189:Retrospective Studies; D013601:T-Lymphocytes / Q000276:immunology*