title: Memory switched B cell percentage and not serum immunoglobulin concentration is associated with clinical complications in children and adults with specific antibody deficiency and common variable immunodeficiency.
abstract: Although idiopathic humoral immunodeficiencies are arbitrarily classified into specific antibody deficiency (SAD) or common variable immunodeficiency (CVID), this distinction does not accurately predict the risk of the bronchiectasis, one of the major long-term clinical complications in these patients. In this study, clinical complications were compared with laboratory markers of cellular and humoral immunity in fifty-five consecutive patients (27 children and 28 adults) attending regional immunology clinics in Manchester, United Kingdom. Reduced CD19(+)CD27(+)IgD(-) B cell percentage but not serum immunoglobulin levels or classification of patients into SAD and CVID was associated with a significantly higher prevalence of bronchiectasis (OR 0.4 (0.2-0.8), P = 0.001), splenomegaly (OR 0.2 (0.1-0.5), P = 0.001) and autoimmunity (OR 0.4 (0.2-0.7), P = 0.003). We conclude that in patients with idiopathic humoral immunodeficiencies assessment of B cell switching more accurately predicts clinical prognosis than either classification of patients into SAD and CVID or serum immunoglobulin concentrations.
authors.affiliation: Department of Immunology, Hope Hospital, Manchester, UK.
authors.forename: Hana
authors.initials: H
authors.lastname: Alachkar
authors.forename: Nadine
authors.initials: N
authors.lastname: Taubenheim
authors.forename: Mansel R
authors.initials: MR
authors.lastname: Haeney
authors.forename: Anne
authors.initials: A
authors.lastname: Durandy
authors.forename: Peter D
authors.initials: PD
authors.lastname: Arkwright
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D016175:B-Lymphocyte Subsets / Q000276:immunology; D001402:B-Lymphocytes / Q000166:cytology / Q000276:immunology*; D001987:Bronchiectasis / Q000150:complications / Q000276:immunology; D002648:Child; D002675:Child, Preschool; D017074:Common Variable Immunodeficiency / Q000150:complications / Q000276:immunology*; D005260:Female; D006801:Humans; D017578:Immunoglobulin Class Switching / Q000276:immunology*; D007136:Immunoglobulins / Q000097:blood* / Q000276:immunology; D007153:Immunologic Deficiency Syndromes / Q000150:complications / Q000276:immunology*; D007156:Immunologic Memory / Q000276:immunology*; D016130:Immunophenotyping; D008213:Lymphocyte Activation; D008297:Male; D008875:Middle Aged; D009154:Mutation; D011237:Predictive Value of Tests; D013163:Splenomegaly / Q000276:immunology
chemical_list: D007136:Immunoglobulins