title: Utility of peripheral blood B cell subsets analysis in common variable immunodeficiency.
abstract: Abnormalities in peripheral blood B cell subsets have been identified in common variable immunodeficiency (CVID) patients and classification systems based upon their numbers have been proposed to predict the clinical features. We analysed B lymphocyte subsets by multi-colour flow cytometry (MFC) in a cohort of well-characterized CVID patients to look at their clinical relevance and validate the published association of different classification criteria (Freiburg, Paris and Euroclass) with clinical manifestations. CVID patients had a reduced proportion of total and switched memory B cells (MBC, swMBC) compared to normal controls (P < 0·0006). Patients classified in Freiburg Ia had a higher prevalence of granulomatous diseases (P = 0·0034). The previously published associations with autoimmune diseases could not be confirmed. The Euroclass classification was not predictive of clinical phenotypes. The absolute numbers of all B cell subsets were reduced in CVID patients compared to controls. There was a significant linear correlation between low absolute total B cells and MBC with granulomatous disease (P < 0·05) and a trend towards lower B cells in patients with autoimmune diseases (P = 0·07). Absolute number of different B cell subsets may be more meaningful than their relative percentages in assessing the risk of granulomatous diseases and possibly autoimmunity.
authors.affiliation: Division of Human Immunology, SA Pathology, University of Adelaide, Adelaide, Australia.
authors.forename: M
authors.initials: M
authors.lastname: Al Kindi
authors.forename: J
authors.initials: J
authors.lastname: Mundy
authors.forename: T
authors.initials: T
authors.lastname: Sullivan
authors.forename: W
authors.initials: W
authors.lastname: Smith
authors.forename: F
authors.initials: F
authors.lastname: Kette
authors.forename: A
authors.initials: A
authors.lastname: Smith
authors.forename: R
authors.initials: R
authors.lastname: Heddle
authors.forename: P
authors.initials: P
authors.lastname: Hissaria
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D001327:Autoimmune Diseases / Q000097:blood / Q000209:etiology / Q000276:immunology; D016175:B-Lymphocyte Subsets / Q000276:immunology*; D002648:Child; D002675:Child, Preschool; D017074:Common Variable Immunodeficiency / Q000097:blood / Q000145:classification / Q000209:etiology / Q000276:immunology*; D015897:Comorbidity; D003430:Cross-Sectional Studies; D005260:Female; D005434:Flow Cytometry; D006099:Granuloma / Q000209:etiology; D006801:Humans; D006967:Hypersensitivity / Q000209:etiology; D007156:Immunologic Memory; D016130:Immunophenotyping; D007239:Infections / Q000209:etiology; D018655:Lymphocyte Count; D008297:Male; D008875:Middle Aged; D022242:Pneumococcal Vaccines / Q000276:immunology; D012008:Recurrence; D013163:Splenomegaly / Q000209:etiology; D055815:Young Adult
chemical_list: C414006:23-valent pneumococcal capsular polysaccharide vaccine; D022242:Pneumococcal Vaccines