title: CD40/CD40L expression in leukocytes from chronic granulomatous disease patients.
abstract: Chronic granulomatous disease (CGD) is an inherited disorder caused by defects in the NADPH oxidase complex, which generates superoxide, the precursor of hydrogen peroxide (H(2)O(2)) and other reactive oxygen derivatives with microbicidal activity. Because CGD patients are at risk of chronic inflammatory manifestations, including inflammatory bowel disease and autoimmune diseases, and it is not clear whether these pathologies are exclusively secondary to altered superoxide production, or whether distinct immunologic defects are involved, we explored cell proliferation, lymphocyte cell counts, immunoglobulin levels, presence of autoimmune antibodies and expression of costimulatory molecules in leukocytes from CGD patients. We found that CGD patients have a diminished phytohemagglutinin-induced proliferation of blood mononuclear cells. Following stimulation with PMA plus ionomycin, a reduced percentage of CD40L expression in T lymphocytes and a diminished expression of CD40 molecules in neutrophils were observed on leukocytes from these patients. Our results suggest an altered interplay between elements of innate and adaptive immunity in CGD patients, which may be reflected in an increased susceptibility to opportunistic infections.
authors: Institute of Clinical Immunology, University of Los Andes, Mérida, Venezuela.
authors: Siham
authors: S
authors: Salmen
authors: Daniela
authors: D
authors: Corte
authors: Loredana
authors: L
authors: Goncalves
authors: Luisa
authors: L
authors: Barboza
authors: Henry
authors: H
authors: Montes
authors: Alí
authors: A
authors: Calderón
authors: Lisbeth
authors: L
authors: Berrueta
mesh_terms: D000293:Adolescent; D000328:Adult; D019013:CD40 Antigens / Q000097:blood*; D023201:CD40 Ligand / Q000097:blood*; D005260:Female; D006105:Granulomatous Disease, Chronic / Q000276:immunology*; D006801:Humans; D007962:Leukocytes / Q000276:immunology*; D008297:Male; D019255:NADPH Oxidases / Q000502:physiology; D009504:Neutrophils / Q000276:immunology; D017382:Reactive Oxygen Species / Q000378:metabolism
chemical_list: D019013:CD40 Antigens; D017382:Reactive Oxygen Species; D023201:CD40 Ligand; D019255:NADPH Oxidases