title: Functional studies on lymphocytes from two siblings with congenital hypogammaglobulinaemia.
abstract: Two brothers with hypogammaglobulinaemia classified as common variable immunodeficiency (CVID) were investigated for distribution of peripheral blood lymphocyte (PBL) subpopulations, DNA synthesis and plaque-forming cell (PFC) capability of pokeweed mitogen (PWM) activated autologous and allogenic cocultures. Both patients had a decreased absolute number of T cells and normal or elevated levels of surface immunoglobulin (SmIg) bearing cells. Isolated B cells cocultured with autologous or allogeneic 4000 r irradiated T cells responded subnormally to PWM monitored by the 3H-thymidine incorporation in microcultures whereas B cells cocultured with allogeneic untreated normal T cells proliferated normally. PBL from parallel macrocultures of unfractionated or T/B separated patients' cells were not able to produce plaques using a reversed haemolytic protein A assay. Addition of glucocorticoid to unfractionated PBL did not reverse the unresponsiveness. In allogeneic cocultures patients' untreated or 2000 r irradiated T cells induced a normal PFC response. Normal untreated T cells induced a reduced number of IgM- and IgG-PFC from patients' B cells but this response was almost eliminated using irradiated normal T cells. These results demonstrate a primary B cell defect in the patients and indicate an impaired cooperation between patients' B and T cells. Activation of patients' B cells to Ig secretion requires the presence of proliferating T cells.
mesh_terms: D000361:Agammaglobulinemia / Q000235:genetics / Q000276:immunology*; D000921:Antibody-Producing Cells / Q000187:drug effects / Q000276:immunology; D001402:B-Lymphocytes / Q000276:immunology; D002648:Child; D003907:Dexamethasone / Q000494:pharmacology; D006462:Hemolytic Plaque Technique; D006801:Humans; D007958:Leukocyte Count; D008213:Lymphocyte Activation; D008214:Lymphocytes / Q000145:classification / Q000276:immunology*; D008297:Male; D012397:Rosette Formation; D013601:T-Lymphocytes / Q000276:immunology
chemical_list: D003907:Dexamethasone