title: Combined decrease of defined B and T cell subsets in a group of common variable immunodeficiency patients.
abstract: Common variable immunodeficiency disease (CVID) is a primary immune disorder affecting B cells and characterized by hypogammaglobulinemia and recurrent infections. To elucidate the clinical and immunological heterogeneity of this condition, we have studied B and T cell subsets in 25 CVID patients. In eleven of them, we observed a remarkable relative expansion of a B cell subpopulation (CD19(hi)/CD21(lo) cells) characterized by the absence of CD23 and the reduced expression of the chemokine receptors CXCR5 and CCR7. Our analyses demonstrated in these patients that the expansion of CD19(hi)/CD21(lo) cells correlates with a selective decrease of circulating naïve and CD21(hi) memory B lymphocytes. The same group of patients displayed a simultaneous severe reduction of naïve CD4+ T cells associated with decreased levels of T cell receptor excision circles. These observations suggest that a combined defect in generation of B and T subpopulations may account for the abnormal immunophenotype characterizing this subgroup of CVID patients.
authors.affiliation: Istituto di Medicina Molecolare "Angelo Nocivelli", Clinica Pediatrica, Universita' di Brescia, Spedali Civili, Piazzale Spedali Civili 1, 25123 Brescia, Italy. daniele.moratto@gmail.com
authors.forename: Daniele
authors.initials: D
authors.lastname: Moratto
authors.forename: Anna Virginia
authors.initials: AV
authors.lastname: Gulino
authors.forename: Stefania
authors.initials: S
authors.lastname: Fontana
authors.forename: Luigi
authors.initials: L
authors.lastname: Mori
authors.forename: Silvia
authors.initials: S
authors.lastname: Pirovano
authors.forename: Annarosa
authors.initials: A
authors.lastname: Soresina
authors.forename: Antonella
authors.initials: A
authors.lastname: Meini
authors.forename: Luisa
authors.initials: L
authors.lastname: Imberti
authors.forename: Luigi Daniele
authors.initials: LD
authors.lastname: Notarangelo
authors.forename: Alessandro
authors.initials: A
authors.lastname: Plebani
authors.forename: Raffaele
authors.initials: R
authors.lastname: Badolato
mesh_terms: D000293:Adolescent; D000328:Adult; D000361:Agammaglobulinemia / Q000097:blood / Q000276:immunology*; D018941:Antigens, CD19 / Q000097:blood; D016175:B-Lymphocyte Subsets / Q000276:immunology*; D016022:Case-Control Studies; D002648:Child; D002675:Child, Preschool; D017074:Common Variable Immunodeficiency / Q000276:immunology*; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D008875:Middle Aged; D054400:Receptors, CCR7; D054380:Receptors, CXCR5; D019707:Receptors, Chemokine / Q000097:blood; D017464:Receptors, Complement 3d / Q000097:blood; D016176:T-Lymphocyte Subsets / Q000276:immunology*
chemical_list: D018941:Antigens, CD19; C515370:CCR7 protein, human; C095085:CXCR5 protein, human; D054400:Receptors, CCR7; D054380:Receptors, CXCR5; D019707:Receptors, Chemokine; D017464:Receptors, Complement 3d