title: Comparison of various classifications for patients with common variable immunodeficiency (CVID) using measurement of B-cell subsets.
abstract: BACKGROUND
  Common variable immunodeficiency (CVID) is a heterogeneous disease, characterised by hypogammaglobulinaemia leading to recurrent infections and various complications. The aim of this study was to classify CVID patients based on four known classifications (Paris, Freiburg, EUROclass, and B-cell patterns) by measurement of B-cell subsets and to assess the relation of each classification with clinical manifestations.
  
  
  METHODS
  We measured all B-cell subsets as both absolute count and percentage in 30 CVID patients and 30 healthy individuals using four-colour flow cytometry. Moreover, we evaluated antibody responses to pneumococcal vaccine in patients.
  
  
  RESULTS
  A significant reduction in percentage of terminal B-cell subsets (total, marginal zone-like, switched memory, IgM-only memory, total memory B-cells and plasmablast) and absolute count of all B-cell subsets along with a strong increase in CD21lowB-cells has been observed in patients. Patients with splenomegaly and hepatomegaly clustered in group Ia, smB+21lowand group 1 based on known classifications, and significantly tended to have a decreased transitional and marginal zone-like B-cells count, as well as an increase in CD21lowB-cell counts. Patients with lymphadenopathy, bronchiectasis and allergy had a significant decrease in absolute count of total memory, switched memory and total B-cells, respectively.
  
  
  CONCLUSIONS
  Classification of patients could provide useful information to guide clinicians in long-term follow-up of CVID patients. Our data demonstrate that it may be more accurate to use absolute counts of B-cell subpopulations in CVID patients because absolute counts of B-cell subsets are more associated with clinical manifestations compared with their percentage and also four known classifications.
authors.affiliation: Department of Immunology, School of Medicine, Isfahan University of Medical Sciences, Isfahan, Iran; Research Center for Immunodeficiencies, Pediatrics Center of Excellence, Children's Medical Center, Tehran University of Medical Sciences, Tehran, Iran; Molecular Immunology Interest Group (MIIG), Universal Scientific Education and Research Network (USERN), Isfahan, Iran.
authors.forename: R
authors.initials: R
authors.lastname: Yazdani
authors.affiliation: Department of Immunology, School of Medicine, Isfahan University of Medical Sciences, Isfahan, Iran.
authors.forename: R
authors.initials: R
authors.lastname: Seify
authors.affiliation: Department of Immunology, School of Medicine, Isfahan University of Medical Sciences, Isfahan, Iran. Electronic address: mghakemi@med.mui.ac.ir.
authors.forename: M
authors.initials: M
authors.lastname: Ganjalikhani-Hakemi
authors.affiliation: Research Center for Immunodeficiencies, Pediatrics Center of Excellence, Children's Medical Center, Tehran University of Medical Sciences, Tehran, Iran.
authors.forename: H
authors.initials: H
authors.lastname: Abolhassani
authors.affiliation: Department of Immunology, School of Medicine, Isfahan University of Medical Sciences, Isfahan, Iran.
authors.forename: N
authors.initials: N
authors.lastname: Eskandari
authors.affiliation: Department of Immunology, School of Public Health, Tehran University of Medical Sciences, Tehran, Iran.
authors.forename: F
authors.initials: F
authors.lastname: Golsaz-Shirazi
authors.affiliation: Department of Immunology, Tehran University of Medical Sciences, Tehran, Iran.
authors.forename: B
authors.initials: B
authors.lastname: Ansaripour
authors.affiliation: Department of Immunology, Tehran University of Medical Sciences, Tehran, Iran.
authors.forename: E
authors.initials: E
authors.lastname: Salehi
authors.affiliation: Department of Laboratory Medicine, Imam Hassan Mojtaba Hospital, Alborz University of Medical Sciences, Karaj, Iran.
authors.forename: G
authors.initials: G
authors.lastname: Azizi
authors.affiliation: Research Center for Immunodeficiencies, Pediatrics Center of Excellence, Children's Medical Center, Tehran University of Medical Sciences, Tehran, Iran; Department of Immunology, Tehran University of Medical Sciences, Tehran, Iran; Network of Immunity in Infection, Autoimmunity and Malignancy (NIIMA), Universal Scientific Education and Research Network (USERN), Tehran, Iran.
authors.forename: N
authors.initials: N
authors.lastname: Rezaei
authors.affiliation: Research Center for Immunodeficiencies, Pediatrics Center of Excellence, Children's Medical Center, Tehran University of Medical Sciences, Tehran, Iran. Electronic address: aghamohammadi@sina.tums.ac.ir.
authors.forename: A
authors.initials: A
authors.lastname: Aghamohammadi
mesh_terms: D000293:Adolescent; D000328:Adult; D016175:B-Lymphocyte Subsets / Q000276:immunology*; D001987:Bronchiectasis; D002648:Child; D017074:Common Variable Immunodeficiency / Q000145:classification / Q000276:immunology* / Q000503:physiopathology; D005260:Female; D006529:Hepatomegaly; D006801:Humans; D007156:Immunologic Memory; D016130:Immunophenotyping; D000072281:Lymphadenopathy; D018655:Lymphocyte Count; D008297:Male; D008875:Middle Aged; D017464:Receptors, Complement 3d / Q000378:metabolism; D013163:Splenomegaly; D055815:Young Adult
chemical_list: D017464:Receptors, Complement 3d
keywords: B-cell subsets; Classifications; Common variable immunodeficiency