Document Detail

Importance of B cell co-stimulation in CD4(+) T cell differentiation: X-linked agammaglobulinaemia, a human model.
MedLine Citation:
PMID:  21488866     Owner:  NLM     Status:  MEDLINE    
We were interested in the question of whether the congenital lack of B cells actually had any influence on the development of the T cell compartment in patients with agammaglobulinaemia. Sixteen patients with X-linked agammaglobulinaemia (XLA) due to mutations in Btk, nine patients affected by common variable immune deficiency (CVID) with <2% of peripheral B cells and 20 healthy volunteers were enrolled. The T cell phenotype was determined with FACSCalibur and CellQuest Pro software. Mann-Whitney two-tailed analysis was used for statistical analysis. The CD4 T cell memory compartment was reduced in patients with XLA of all ages. This T cell subset encompasses both CD4(+)CD45RO(+) and CD4(+)CD45RO(+)CXCR5(+) cells and both subsets were decreased significantly when compared to healthy controls: P = 0·001 and P < 0·0001, respectively. This observation was confirmed in patients with CVID who had <2% B cells, suggesting that not the lack of Bruton's tyrosine kinase but the lack of B cells is most probably the cause of the impaired CD4 T cell maturation. We postulate that this defect is a correlate of the observed paucity of germinal centres in XLA. Our results support the importance of the interplay between B and T cells in the germinal centre for the activation of CD4 T cells in humans.
H Martini; V Enright; M Perro; S Workman; J Birmelin; E Giorda; I Quinti; V Lougaris; M Baronio; K Warnatz; B Grimbacher
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Publication Detail:
Type:  Comparative Study; Journal Article; Research Support, Non-U.S. Gov't     Date:  2011-04-13
Journal Detail:
Title:  Clinical and experimental immunology     Volume:  164     ISSN:  1365-2249     ISO Abbreviation:  Clin. Exp. Immunol.     Publication Date:  2011 Jun 
Date Detail:
Created Date:  2011-05-03     Completed Date:  2011-07-18     Revised Date:  2013-06-30    
Medline Journal Info:
Nlm Unique ID:  0057202     Medline TA:  Clin Exp Immunol     Country:  England    
Other Details:
Languages:  eng     Pagination:  381-7     Citation Subset:  IM    
Copyright Information:
© 2011 The Authors. Clinical and Experimental Immunology © 2011 British Society for Immunology.
Department of Immunology, Royal Free Hospital and University College London, UK.
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MeSH Terms
Agammaglobulinemia / diagnosis,  genetics,  immunology
Antigens, CD45 / biosynthesis
B-Lymphocytes / immunology,  metabolism*,  pathology
CD4-Positive T-Lymphocytes / immunology,  metabolism*,  pathology
Cell Differentiation
Cell Separation
Common Variable Immunodeficiency / diagnosis,  genetics,  immunology*
Flow Cytometry
Genetic Diseases, X-Linked / diagnosis,  genetics,  immunology
Immunologic Memory
Lymphocyte Depletion
Middle Aged
Mutation / genetics
Protein-Tyrosine Kinases / genetics
Receptors, CXCR5 / biosynthesis
Reg. No./Substance:
0/CXCR5 protein, human; 0/Receptors, CXCR5; EC tyrosine kinase; EC Kinases; EC, CD45

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