Abstract
Summary: We report the evaluation of in vitro immunoregulation in a 12-year-old untreated boy with severe combined immunodeficiency (SCID). Severely hypogammaglobulinemic, the patient was incapable of a specific antibody response to either natural substances or administered antigens. Ficoll-Hypaque-isolated peripheral blood mononuclear cells (MNL) from the patient failed to respond to pokeweed mitogen (PWM) with the normal increment in immunoglobulin-secreting cells, as measured by a reverse hemolytic plaque assay. Since the patient was lymphopenic, his MNL were relatively enriched for monocytes (range = 51–81%). Removal of phagocytic cells or the addition of unrelated irradiated helper T lymphocytes resulted in enhanced, but still suboptimal response to PWM, suggesting some intrinsic defect in B lymphocyte function. Co-culture of patient MNL with normal MNL resulted in marked suppression (12% of predicted) of PWM-induced Ig-secreting cells. Suppressor activity was unaffected by prior irradiation of patient MNL, but was substantially reversed (99% of predicted) by removal of his phagocytic cells, whereas the combination of the two procedures further reversed suppression (184% of predicted). The patient's MNL consistently demonstrated subnormal percentages of T3+ and T4+ cells and subnormal to low normal percentages of T8+ cells. These data suggest both an intrinsic defect in B lymphocyte function, and a relative excess of monocytes which could further inhibit Ig secretion by B lymphocytes. Natural killer (NK) cell function was characterized by normal target cell binding by NK cells but severely depressed NK cell cytotoxicity.
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Paschall, V., Brown, L., Lawrence, E. et al. Immunoregulation in an Isolated 12-year-old Boy with Congenital Severe Combined Immunodeficiency. Pediatr Res 18, 723–728 (1984). https://doi.org/10.1203/00006450-198408000-00009
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DOI: https://doi.org/10.1203/00006450-198408000-00009