Published in:
01-09-2010
B-Cell and T-Cell Phenotypes in CVID Patients Correlate with the Clinical Phenotype of the Disease
Authors:
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, for the DEFI Study Group
Published in:
Journal of Clinical Immunology
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Issue 5/2010
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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.