title: Behçet's-like syndrome associated with idiopathic CD4+ T-lymphocytopenia, opportunistic infections, and a large population of TCR alpha beta+ CD4- CD8- T cells.
abstract: Herein we report a patient with Behçet's like syndrome, idiopathic CD4+ T-lymphocytopenia, opportunistic infections, and a large polyclonal population of TCR alpha beta + CD4- CD8- T cells. Microfluorimetric analysis of peripheral blood mononuclear cells revealed CD4+ T-cell counts of 10 +/- 5/mm3. The CD3+ T cells were 99% TCR alpha beta +, of which 74 +/- 5% were CD4- CD8-. No clonal populations were detected by southern analysis for T-cell receptor V beta gene rearrangements. No evidence of human immunodeficiency virus infection was present, although nocardia, candida, pneumocystis, cytomegalovirus, and herpes infections were documented. The concomitant presence of opportunistic infections and a large population of TCR alpha beta + CD4- CD8- T cells suggests a pathogenic association and an intense immune response to microbial lipid or lipoglycan antigens presented in the context of CD1 molecules. This case demonstrates the potential for idiopathic CD4+ T-lymphocytopenia to occur in Behçet's-like syndrome with lethal consequences.
authors.affiliation: Department of Medicine, Baylor College of Medicine, Houston, Texas 77030, USA.
authors.forename: J
authors.initials: J
authors.lastname: Venzor
authors.forename: Q
authors.initials: Q
authors.lastname: Hua
authors.forename: R B
authors.initials: RB
authors.lastname: Bressler
authors.forename: C H
authors.initials: CH
authors.lastname: Miranda
authors.forename: D P
authors.initials: DP
authors.lastname: Huston
mesh_terms: D001528:Behcet Syndrome / Q000150:complications* / Q000175:diagnosis / Q000276:immunology*; D002177:Candidiasis / Q000150:complications / Q000276:immunology; D003586:Cytomegalovirus Infections / Q000150:complications / Q000276:immunology; D017809:Fatal Outcome; D018023:HIV Seronegativity; D006566:Herpesviridae Infections / Q000150:complications / Q000276:immunology; D006801:Humans; D008297:Male; D008875:Middle Aged; D009617:Nocardia Infections / Q000150:complications / Q000276:immunology; D009616:Nocardia asteroides; D009894:Opportunistic Infections / Q000150:complications* / Q000276:immunology*; D011020:Pneumonia, Pneumocystis / Q000150:complications / Q000276:immunology; D016693:Receptors, Antigen, T-Cell, alpha-beta / Q000378:metabolism; D016176:T-Lymphocyte Subsets / Q000276:immunology; D018344:T-Lymphocytopenia, Idiopathic CD4-Positive / Q000150:complications* / Q000276:immunology*
chemical_list: D016693:Receptors, Antigen, T-Cell, alpha-beta