Functional Studies on Lymphocytes from Two Siblings with Congenital Hypogammaglobulinaemia

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Abstract
Two brothers with hypogammaglobulinemia 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 Ig (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; 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 hemolytic 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. A primary B cell defect was demonstrated in the patients and an impaired cooperation between patients'' B and T cells was indicated. Activation of patients'' B cell to Ig secretion requires the presence of proliferating T cells.