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.
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Acknowledgments
This study was supported by a national program on rare diseases (GIS-Maladies Rares), by the National Program for Clinical Research (PHRC 2005), and by the National Center on Hereditary Immune Deficiencies (CEREDIH).
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A complete list of the DEFI Study Group is presented in Appendix A.
Appendix A. DEFI Study Group
Appendix A. DEFI Study Group
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Coordination: Hôpital Saint Louis, Paris: E. Oksenhendler
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Clinical Centers: Hôpital Saint Louis, Paris: C. Fieschi, M. Malphettes, L. Galicier, S. Georgin, J.P. Fermand. Bordeaux: J.F. Viallard. Limoges: A. Jaccard. Tours: C. Hoarau, Y. Lebranchu. Hôpital Cochin, Paris: A. Bérezné, L. Mouthon. HEGP, Paris: M. Karmochkine. Marseille: N. Schleinitz. Lyon Sud: I. Durieu, R. Nove-Josserand. Clermont-Ferrand: V. Chanet. Montpellier: V. Le-Moing. Roubaix: N. Just. Hôtel-Dieu, Paris: C. Salanoubat. Reims: R. Jaussaud. Hôpital Necker, Paris: F. Suarez, O. Hermine. Le Mans: P. Solal-Celigny. Lille: E. Hachulla. Perpignan: L. Sanhes. Angers: M. Gardembas, I. Pellier. Troyes: P. Tisserant. Lyon Armée: M. Pavic. Dijon: B. Bonnotte. Pitié-Salpêtrière, Paris: J. Haroche, Z. Amoura. Toulouse: L. Alric, M.F. Thiercelin, L. Tetu, D. Adoue. Nancy Vandoeuvre: P. Bordigoni. Lyon Croix Rousse: T. Perpoint. Lyon Hotel-Dieu: P. Sève. Besançon: P. Rohrlich. Strasbourg: J.L. Pasquali, P. Soulas. Hôpital Foch, Suresnes: L.J. Couderc. Montauban: P. Giraud. Hôpital Saint-Louis, Pédiatrie, Paris: A. Baruchel. Clermont-Ferrand 2: I. Deleveau. Kremlin-Bicêtre: F. Chaix. Hôpital Trousseau, Paris: J. Donadieu. Rouen: F. Tron. Bobigny: C. Larroche. Aix: A.P. Blanc. Nantes: A. Masseau, M. Hamidou. Nancy: G. Kanny, M. Morisset. Poitiers: F. Millot. Bondy: O. Fain. Hôpital Bichat, Paris: R. Borie. Rennes: A. Perlat
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Laboratories: Pitié-Salpêtrière, INSERM U543, Paris: P. Debré, G. Mouillot, I. Théodorou. Saint-Louis, Immunologie, Paris: C. Rabian, M. Carmagnat. Saint-Louis, EA 3963, Paris: C. Fieschi, M. Malphettes, N. Vince, D. Boutboul
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Clinical research assistants: A. De Gouvello, A. Gardeur.
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Data management and statistics: L. Gérard.
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Mouillot, G., Carmagnat, M., Gérard, L. et al. B-Cell and T-Cell Phenotypes in CVID Patients Correlate with the Clinical Phenotype of the Disease. J Clin Immunol 30, 746–755 (2010). https://doi.org/10.1007/s10875-010-9424-3
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DOI: https://doi.org/10.1007/s10875-010-9424-3