Abstract
Using Epstein-Barr virus (EBV), we have established LCL from 15 patients with IDS, including patients with ataxia telangiectasia (3), Wiskott-Aldrich syndrome (1), severe combined immune deficiency (1), common variable immune deficiency (5), X-linked immune deficiency with increased IgM (1), immune deficiency with normal immunoglobulins (1), C4 deficiency (1), Job's syndrome (1). LCL could not be established from 4 patients with infantile X-linked agammaglobulinemia despite multiple attempts. All LCL have surface markers and functions characteristic of normal B-lymphocytes, including C3 receptors and surface immunoglobulins, and were potent stimulators in the mixed lymphocyte reaction. None of the LCL expressed T-cell characteristics. The inability to establish LCL from X-linked agammaglobulinemic patients might reflect absence of EBV receptors as well as absent B-lymphocytes. Most of the LCL appear to be polyclonal. Individual lines expressed more than one class of surface immunoglobulin. Indeed, LCL were established which expressed immunoglobulin classes markedly deficient or absent in the serum of the patients from which they were derived, suggesting the defect resulted from suppressor activity rather than defective synthesis. Further immunologic and metabolic studies of these lines may help to elucidate pathologic mechanisms responsible for primary IDS.
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Sullivan, J., Ochs, H., Dunsmoor, C. et al. ESTABLISHMENT AND CHARACTERIZATION OF LYMPHOBLASTOID CELL LINES (LCL) DERIVED FROM PATIENTS WITH IMMUNE DEFICIENCY SYNDROMES (IDS). Pediatr Res 11, 495 (1977). https://doi.org/10.1203/00006450-197704000-00753
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DOI: https://doi.org/10.1203/00006450-197704000-00753