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Low marginal zone-like B lymphocytes and natural antibodies characterize skewed B-lymphocyte subpopulations in del22q11 DiGeorge patients.
- Source :
-
Clinical immunology (Orlando, Fla.) [Clin Immunol] 2015 Dec; Vol. 161 (2), pp. 144-9. Date of Electronic Publication: 2015 Sep 02. - Publication Year :
- 2015
-
Abstract
- Purpose: Patients with DiGeorge syndrome suffer from T-lymphopenia. T-cells are important for the maturation and regulation of B-cell function. Our aim was to characterize the B-cell compartment in DiGeorge syndrome patients.<br />Methods: B-cell subset phenotypization using flow cytometry. Serum BAFF (B-cell activating factor) and serum anti-alpha-galactosyl IgM measurement using ELISA. Serum IgG measurement using nephelometry.<br />Results: We observed a significantly increased number of naïve B-cells and decreased number of switched memory B-cells in DiGeorge patients. Furthermore, we observed increased BAFF levels and a trend toward hypergammaglobulinemia later in life. Surprisingly, we detected a decrease in marginal zone-like (MZ-like) B-cells and natural antibodies in DiGeorge patients.<br />Conclusion: The maturation of B-cells is impaired in DiGeorge patients, with high naïve and low switched memory B-cell numbers being observed. There is a clear trend toward hypergammaglobulinemia later in life, coupled with increased serum BAFF levels. Surprisingly, the T-independent humoral response is also impaired, with low numbers of MZ-like B-cell and low levels of anti-alpha-galactosyl IgM natural antibodies being detected.<br /> (Copyright © 2015 Elsevier Inc. All rights reserved.)
- Subjects :
- Adolescent
Adult
B-Cell Activating Factor immunology
Case-Control Studies
Child
Child, Preschool
Female
Humans
Hypergammaglobulinemia immunology
Immunity, Humoral immunology
Immunoglobulin G immunology
Immunoglobulin M immunology
Immunologic Memory immunology
Infant
Infant, Newborn
Male
Young Adult
Antibodies immunology
B-Lymphocyte Subsets immunology
DiGeorge Syndrome immunology
Subjects
Details
- Language :
- English
- ISSN :
- 1521-7035
- Volume :
- 161
- Issue :
- 2
- Database :
- MEDLINE
- Journal :
- Clinical immunology (Orlando, Fla.)
- Publication Type :
- Academic Journal
- Accession number :
- 26341314
- Full Text :
- https://doi.org/10.1016/j.clim.2015.08.013