B-Cell and T-Cell Phenotypes in CVID Patients Correlate with the Clinical Phenotype of the Disease

Autor: Gaël, Mouillot, Maryvonnick, Carmagnat, Laurence, Gérard, Jean-Luc, Garnier, Claire, Fieschi, Nicolas, Vince, Lionel, Karlin, Jean-François, Viallard, Roland, Jaussaud, Julien, Boileau, Jean, Donadieu, Martine, Gardembas, Nicolas, Schleinitz, Felipe, Suarez, Eric, Hachulla, Karen, Delavigne, Martine, Morisset, Serge, Jacquot, Nicolas, Just, Lionel, Galicier, Dominique, Charron, Patrice, Debré, Eric, Oksenhendler, Claire, Rabian, L, Gérard
Rok vydání: 2010
Předmět:
Zdroj: Journal of Clinical Immunology. 30:746-755
ISSN: 1573-2592
0271-9142
DOI: 10.1007/s10875-010-9424-3
Popis: Common variable immunodeficiency (CVID) is a heterogeneous disorder characterized by recurrent infections and defective immunoglobulin production.The DEFI French national prospective study investigated peripheral T-cell and B-cell compartments in 313 CVID patients grouped according to their clinical phenotype, using flow cytometry.In patients developing infection only (IO), the main B-cell or T-cell abnormalities were a defect in switched memory B cells and a decrease in naive CD4(+) T cells associated with an increase in CD4(+)CD95(+) cells. These abnormalities were more pronounced in patients developing lymphoproliferation (LP), autoimmune cytopenia (AC), or chronic enteropathy (CE). Moreover, LP and AC patients presented an increase in CD21(low) B cells and CD4(+)HLA-DR(+) T cells and a decrease in regulatory T cells.In these large series of CVID patients, the major abnormalities of the B-cell and T-cell compartments, although a hallmark of CVID, were only observed in half of the IO patients and were more frequent and severe in patients with additional lymphoproliferative, autoimmune, and digestive complications.
Databáze: OpenAIRE