title: B-cell and T-cell phenotypes in CVID patients correlate with the clinical phenotype of the disease.
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 CD21(low) B cells and CD4(+)HLA-DR(+) T cells and a decrease in regulatory T cells.
  
  
  CONCLUSIONS
  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.
authors.affiliation: Immunology Laboratory, INSERM UMR-S945, CIB Pitié-Salpêtrière, Assistance Publique-Hôpitaux de Paris, Paris, France. gael.mouillot@psl.aphp.fr
authors.forename: Gaël
authors.initials: G
authors.lastname: Mouillot
authors.forename: Maryvonnick
authors.initials: M
authors.lastname: Carmagnat
authors.forename: Laurence
authors.initials: L
authors.lastname: Gérard
authors.forename: Jean-Luc
authors.initials: JL
authors.lastname: Garnier
authors.forename: Claire
authors.initials: C
authors.lastname: Fieschi
authors.forename: Nicolas
authors.initials: N
authors.lastname: Vince
authors.forename: Lionel
authors.initials: L
authors.lastname: Karlin
authors.forename: Jean-François
authors.initials: JF
authors.lastname: Viallard
authors.forename: Roland
authors.initials: R
authors.lastname: Jaussaud
authors.forename: Julien
authors.initials: J
authors.lastname: Boileau
authors.forename: Jean
authors.initials: J
authors.lastname: Donadieu
authors.forename: Martine
authors.initials: M
authors.lastname: Gardembas
authors.forename: Nicolas
authors.initials: N
authors.lastname: Schleinitz
authors.forename: Felipe
authors.initials: F
authors.lastname: Suarez
authors.forename: Eric
authors.initials: E
authors.lastname: Hachulla
authors.forename: Karen
authors.initials: K
authors.lastname: Delavigne
authors.forename: Martine
authors.initials: M
authors.lastname: Morisset
authors.forename: Serge
authors.initials: S
authors.lastname: Jacquot
authors.forename: Nicolas
authors.initials: N
authors.lastname: Just
authors.forename: Lionel
authors.initials: L
authors.lastname: Galicier
authors.forename: Dominique
authors.initials: D
authors.lastname: Charron
authors.forename: Patrice
authors.initials: P
authors.lastname: Debré
authors.forename: Eric
authors.initials: E
authors.lastname: Oksenhendler
authors.forename: Claire
authors.initials: C
authors.lastname: Rabian
mesh_terms: D000328:Adult; D000368:Aged; D001402:B-Lymphocytes / Q000276:immunology* / Q000378:metabolism / Q000473:pathology; D015704:CD4 Antigens / Q000096:biosynthesis; D002469:Cell Separation; D017074:Common Variable Immunodeficiency / Q000150:complications / Q000276:immunology* / Q000473:pathology / Q000503:physiopathology; D018450:Disease Progression; D005260:Female; D005434:Flow Cytometry; D005602:France; D006801:Humans; D017578:Immunoglobulin Class Switching / Q000187:drug effects; D007156:Immunologic Memory / Q000187:drug effects; D016130:Immunophenotyping; D007239:Infections / Q000209:etiology / Q000276:immunology* / Q000473:pathology / Q000503:physiopathology; D016131:Lymphocyte Subsets / Q000276:immunology* / Q000378:metabolism; D008231:Lymphopenia; D008297:Male; D008875:Middle Aged; D011504:Protein-Losing Enteropathies; D013601:T-Lymphocytes / Q000276:immunology* / Q000378:metabolism / Q000473:pathology; D019014:fas Receptor / Q000096:biosynthesis
chemical_list: D015704:CD4 Antigens; D019014:fas Receptor