Abstract
Functional and morphologic evaluation of the immune system was carried out on 2 females with sporadic agammaglobulinemia (SA) dating from infancy and a male with X-linked agammaglobulinemia (XLA). All 3 patients had profound hypogammaglobulinemia, lacked isoagglutinins, and failed to synthesize antibodies to Salmonella H and O antigens following immunization. All lacked plasma cells and cells exhibiting cytoplasmic fluorescence with fluoresclne-labeled antibodies to IgM, IgG, and IgA in biopsies of ileum and stimulated lymph node. In contrast to the patient with XLA, patients with SA had prominent germinal centers in stimulated nodes; one had the syndrome of nodular lymphoid hyperplasia (NLH). Using criteria established for identification of bursa-derived lymphocytes in chickens, B-lymphocytes were identified by electron microscopy in the lymph node of one patient with SA; only T-lymphocytes were found in the patient with XLA. Membrane-bound immunoglobulins (IgM, IgG, and IgA) were detected by immunofluorescent studies of living peripheral blood lymphocytes of both patients with SA, but were not found on cells from the patient with XLA. The results indicate that in these patients with SA, lymphocytes of the plasma cell line are able to synthesize surface immunoglobulins and to respond to antigenic stimulation by germinal center formation but fail to differentiate to the stage of antibody secretion; lack of antibody-mediated “feedback” inhibition of antigen-induced proliferation may account for the NLH often observed in SA. Our observations also provide preliminary evidence that B and T lymphocytes can be distinguished at an ultrastructural level in man.
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Lawton, A., Bockman, D. & Cooper, M. Further definition of two distinct types of congenital defects in plasma cell differentiation resulting in agammaglobulinemia. Pediatr Res 5, 377–378 (1971). https://doi.org/10.1203/00006450-197108000-00029
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DOI: https://doi.org/10.1203/00006450-197108000-00029