Estimation of B-cells transformed by Epstein-Barr virus in patients with congenital agammaglobulinemia.
In vitro immunoglobulin synthesis was measured in lymphocytes from four patients with congenital agammaglobulinemia (cA gamma) stimulated by two different polyclonal B-cell activators, pokeweed mitogen (PWM) and Epstein-Barr virus (EBV). In PWM-stimulated cultures, patient T-cells treated with mitomycin C (MMC) were able to help the immunoglobulin (Ig) synthesis of normal B-cells. Patient B-cell-enriched fraction not containing surface Ig positive cells did not produce Ig in combination with MMC-treated autologous or allogeneic T-cells. Patient lymphocytes were infected with EBV and the subsequent production of Ig was measured. In lymphocytes from control subjects, exponential growth of the cells having EBV-associated nuclear antigen (EBNA) was shown to be associated with an exponential increase in Ig secretion within 1 week after EBV infection. However, in lymphocytes from three of the four patients, it took 2, 4 and 10 weeks, respectively, until lymphocyte-transformation and subsequent Ig-secretion were observed. Lymphocytes from one patient were not transformed nor did they secrete Ig after EBV infection. These results may imply that a small number of B-cells are present in peripheral blood of most of patients with cA gamma, and that they are able to produce the Ig after transformation by EBV which takes a much longer time than in controls.
D000361:Agammaglobulinemia / Q000151:congenital / Q000276:immunology*; D000956:Antigens, Viral / Q000032:analysis; D001402:B-Lymphocytes / Q000276:immunology*; D004854:Herpesvirus 4, Human / Q000276:immunology / Q000472:pathogenicity*; D006801:Humans; D007136:Immunoglobulins / Q000096:biosynthesis; D066298:In Vitro Techniques; D008213:Lymphocyte Activation; D008214:Lymphocytes / Q000378:metabolism
D000956:Antigens, Viral; D007136:Immunoglobulins