B-cell and T-cell phenotypes in CVID patients correlate with the clinical phenotype of the disease.
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.
Mouillot
Gaël
G
Immunology Laboratory, INSERM UMR-S945, CIB Pitié-Salpêtrière, Assistance Publique-Hôpitaux de Paris, Paris, France. gael.mouillot@psl.aphp.fr
Carmagnat
Maryvonnick
M
Gérard
Laurence
L
Garnier
Jean-Luc
JL
Fieschi
Claire
C
Vince
Nicolas
N
Karlin
Lionel
L
Viallard
Jean-François
JF
Jaussaud
Roland
R
Boileau
Julien
J
Donadieu
Jean
J
Gardembas
Martine
M
Schleinitz
Nicolas
N
Suarez
Felipe
F
Hachulla
Eric
E
Delavigne
Karen
K
Morisset
Martine
M
Jacquot
Serge
S
Just
Nicolas
N
Galicier
Lionel
L
Charron
Dominique
D
Debré
Patrice
P
Oksenhendler
Eric
E
Rabian
Claire
C
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
D015704:CD4 Antigens; D019014:fas Receptor