Document Detail

Changes in B cell immunophenotype in common variable immunodeficiency: cause or effect - is bronchiectasis indicative of undiagnosed immunodeficiency?
MedLine Citation:
PMID:  23286946     Owner:  NLM     Status:  MEDLINE    
Common variable immunodeficiency (CVID) is the most common severe primary immunodeficiency, but the pathology of this condition is poorly understood. CVID involves a defect in the production of immunoglobulin from B cells, with a subsequent predisposition to infections. Approximately 10-20% of cases are inherited, but even in families with a genetic defect the penetrance is far from complete. A classification system for CVID has been suggested (EUROclass) based on B cell immunophenotyping, but it has not been shown that altered B cell immunophenotype is not a consequence of the complications and treatment of CVID. This study compares the EUROclass B cell immunophenotype of CVID patients (n = 30) with suitable disease controls with bronchiectasis (n = 11), granulomatous disease (Crohn's disease) (n = 9) and neurological patients on immunoglobulin treatment (n = 6). The results of this study correlate with previous literature, that alterations in B cell immunophenotype are associated strongly with CVID. Interestingly, three of the 11 bronchiectasis patients without known immunodeficiency had an altered B cell immunophenotype, suggesting the possibility of undiagnosed immunodeficiency, or that bronchiectasis may cause a secondary alteration in B cell immunophenotype. This study showed a significant difference in B cell immunophenotype between CVID patients compared to disease control groups of granulomatous disease and immunoglobulin treatment. This suggests that granulomatous disease (in Crohn's disease) and immunoglobulin treatment (for chronic neurological conditions) are not causal of an altered B cell immunophenotype in these control populations.
P Bright; S Grigoriadou; P Kamperidis; M Buckland; A Hickey; H J Longhurst
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  Clinical and experimental immunology     Volume:  171     ISSN:  1365-2249     ISO Abbreviation:  Clin. Exp. Immunol.     Publication Date:  2013 Feb 
Date Detail:
Created Date:  2013-01-04     Completed Date:  2013-03-12     Revised Date:  2014-02-04    
Medline Journal Info:
Nlm Unique ID:  0057202     Medline TA:  Clin Exp Immunol     Country:  England    
Other Details:
Languages:  eng     Pagination:  195-200     Citation Subset:  IM    
Copyright Information:
© 2012 Crown copyright.
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MeSH Terms
Antigens, CD / immunology
B-Lymphocyte Subsets / immunology*
B-Lymphocytes / immunology*
Bronchiectasis / complications,  diagnosis*,  immunology
Case-Control Studies
Cell Differentiation / drug effects,  immunology
Cell Separation
Cells, Cultured
Common Variable Immunodeficiency / immunology*
Crohn Disease / complications,  diagnosis*,  immunology
Diagnosis, Differential
Disease Progression
Flow Cytometry
Immunologic Memory
Middle Aged
Nervous System Diseases / complications,  diagnosis*,  immunology
Young Adult
Reg. No./Substance:
0/Antigens, CD

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