학술논문
B-Cell and T-Cell Phenotypes in CVID Patients Correlate with the Clinical Phenotype of the Disease
Document Type
Academic Journal
Author
Mouillot, Gaël; Carmagnat, Maryvonnick; Gérard, Laurence; Garnier, Jean-Luc; Fieschi, Claire; Vince, Nicolas; Karlin, Lionel; Viallard, Jean-François; Jaussaud, Roland; Boileau, Julien; Donadieu, Jean; Gardembas, Martine; Schleinitz, Nicolas; Suarez, Felipe; Hachulla, Eric; Delavigne, Karen; Morisset, Martine; Jacquot, Serge; Just, Nicolas; Galicier, Lionel; Charron, Dominique; Debré, Patrice; Oksenhendler, Eric; Rabian, Claire
Source
Journal of Clinical Immunology. Sep 01, 2010 30(5):746-755
Subject
Language
English
ISSN
0271-9142
Abstract
BACKGROUND: Common variable immunodeficiency (CVID) is a heterogeneous disorder characterized by recurrent infections and defective immunoglobulin production. Methods 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.Results 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 CD21low B cells and CD4+HLA-DR+ T cells and a decrease in regulatory T cells. Conclusion 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.