Combined decrease of defined B and T cell subsets in a group of common variable immunodeficiency patients.
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.
Moratto
Daniele
D
Istituto di Medicina Molecolare "Angelo Nocivelli", Clinica Pediatrica, Universita' di Brescia, Spedali Civili, Piazzale Spedali Civili 1, 25123 Brescia, Italy. daniele.moratto@gmail.com
Gulino
Anna Virginia
AV
Fontana
Stefania
S
Mori
Luigi
L
Pirovano
Silvia
S
Soresina
Annarosa
A
Meini
Antonella
A
Imberti
Luisa
L
Notarangelo
Luigi Daniele
LD
Plebani
Alessandro
A
Badolato
Raffaele
R
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*
D018941:Antigens, CD19; C515370:CCR7 protein, human; C095085:CXCR5 protein, human; D054400:Receptors, CCR7; D054380:Receptors, CXCR5; D019707:Receptors, Chemokine; D017464:Receptors, Complement 3d