title: Correlation of bone marrow abnormalities, peripheral lymphocyte subsets and clinical features in uncomplicated common variable immunodeficiency (CVID) patients.
abstract: B cell developmental defects in CVID were recently described in a limited number of cases. To date, a detailed correlation between this maturational defect and the clinical presentation of affected patients has not been reported. In this study, we correlated bone marrow B cell evaluation, peripheral B and T lymphocyte subsets and clinical findings in 15 CVID patients. Early B cell developmental defects were observed in one third of patients. Combined bone marrow and peripheral lymphocytes evaluation allowed to further subdivide CVID patients in three groups with shared clinical features at diagnosis and during follow-up. These data broaden the number of CVID patients with early B cell developmental defects and, together with the peripheral lymphocytes evaluation, offer insight into the related clinical features in affected patients.
authors.affiliation: Pediatrics Clinic and Institute for Molecular Medicine A. Nocivelli, Department of Clinical and Experimental Sciences, University of Brescia, Spedali Civili, Brescia, Italy. Electronic address: vlougarisbs@yahoo.com.
authors.forename: Vassilios
authors.initials: V
authors.lastname: Lougaris
authors.affiliation: Pediatrics Clinic and Institute for Molecular Medicine A. Nocivelli, Department of Clinical and Experimental Sciences, University of Brescia, Spedali Civili, Brescia, Italy.
authors.forename: Manuela
authors.initials: M
authors.lastname: Baronio
authors.affiliation: Department of Molecular and Translational Medicine, University of Brescia, Italy.
authors.forename: Stefania
authors.initials: S
authors.lastname: Masneri
authors.affiliation: Pediatrics Clinic and Institute for Molecular Medicine A. Nocivelli, Department of Clinical and Experimental Sciences, University of Brescia, Spedali Civili, Brescia, Italy.
authors.forename: Tiziana
authors.initials: T
authors.lastname: Lorenzini
authors.affiliation: Pediatrics Clinic and Institute for Molecular Medicine A. Nocivelli, Department of Clinical and Experimental Sciences, University of Brescia, Spedali Civili, Brescia, Italy.
authors.forename: Kim
authors.initials: K
authors.lastname: Cattivelli
authors.affiliation: Pediatrics Clinic and Institute for Molecular Medicine A. Nocivelli, Department of Clinical and Experimental Sciences, University of Brescia, Spedali Civili, Brescia, Italy.
authors.forename: Giacomo
authors.initials: G
authors.lastname: Tampella
authors.affiliation: Pediatrics Clinic and Institute for Molecular Medicine A. Nocivelli, Department of Clinical and Experimental Sciences, University of Brescia, Spedali Civili, Brescia, Italy.
authors.forename: Annarosa
authors.initials: A
authors.lastname: Soresina
authors.affiliation: Laboratory of Genetic Disorders of Childhood, "Angelo Nocivelli" Institute for Molecular Medicine, Spedali Civili, Brescia, Italy.
authors.forename: Daniele
authors.initials: D
authors.lastname: Moratto
authors.affiliation: Pediatrics Clinic and Institute for Molecular Medicine A. Nocivelli, Department of Clinical and Experimental Sciences, University of Brescia, Spedali Civili, Brescia, Italy.
authors.forename: Alessandro
authors.initials: A
authors.lastname: Plebani
mesh_terms: D000293:Adolescent; D000328:Adult; D001327:Autoimmune Diseases / Q000209:etiology / Q000276:immunology; D016175:B-Lymphocyte Subsets / Q000276:immunology; D001402:B-Lymphocytes / Q000276:immunology*; D001853:Bone Marrow; D001854:Bone Marrow Cells / Q000276:immunology*; D001987:Bronchiectasis / Q000209:etiology / Q000276:immunology; D017074:Common Variable Immunodeficiency / Q000150:complications / Q000276:immunology*; D005260:Female; D005434:Flow Cytometry; D006801:Humans; D008213:Lymphocyte Activation / Q000276:immunology*; D008297:Male; D008875:Middle Aged; D012008:Recurrence; D012141:Respiratory Tract Infections / Q000209:etiology / Q000276:immunology; D013163:Splenomegaly / Q000209:etiology / Q000276:immunology; D016176:T-Lymphocyte Subsets / Q000276:immunology; D013601:T-Lymphocytes / Q000276:immunology*; D055815:Young Adult
keywords: B cells; Bone marrow; CVID