Adam Klocperk1, Ester Mejstříková2, Jana Kayserová3, Tomáš Kalina2, Anna Šedivá3. 1. Department of Immunology, 2nd Faculty of Medicine, Charles University and University Hospital Motol, Prague, Czech Republic. Electronic address: adam.klocperk@fnmotol.cz. 2. Childhood Leukemia Investigation Prague, Department of Paediatric Haematology and Oncology, Charles University and University Hospital Motol, Prague, Czech Republic. 3. Department of Immunology, 2nd Faculty of Medicine, Charles University and University Hospital Motol, Prague, Czech Republic.
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. 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. 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. 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.
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 syndromepatients. 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. 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. 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.
Authors: Jenny Lingman Framme; Christina Lundqvist; Anna-Carin Lundell; Pauline A van Schouwenburg; Andri L Lemarquis; Karolina Thörn; Susanne Lindgren; Judith Gudmundsdottir; Vanja Lundberg; Sofie Degerman; Rolf H Zetterström; Stephan Borte; Lennart Hammarström; Esbjörn Telemo; Magnus Hultdin; Mirjam van der Burg; Anders Fasth; Sólveig Oskarsdóttir; Olov Ekwall Journal: J Clin Immunol Date: 2022-01-26 Impact factor: 8.542
Authors: Adam Klocperk; Zuzana Paračková; Markéta Bloomfield; Michal Rataj; Jan Pokorný; Susanne Unger; Klaus Warnatz; Anna Šedivá Journal: Front Immunol Date: 2018-07-23 Impact factor: 7.561