title: Pathophysiologic analysis of peripheral blood lymphocytes from patients with primary immunodeficiency. I. Ig synthesis by peripheral blood lymphocytes stimulated with either pokeweed mitogen or Epstein-Barr virus in vitro.
abstract: In the present study 5 patients with common variable hypogammaglobulinemia (CVH) and 4 patients with selective IgA deficiency (IgA-D) were analyzed for the cellular defects responsible for impaired Ig synthesis with use of peripheral blood lymphocytes stimulated with either PWM or EBV in vitro. By the use of co-culture with PWM, all the patients examined had intrinsic B cell defects restricted to the synthesis of Ig class corresponding to the low or absent Ig class(es) in the sera. Two types of excessive suppressor T activity were found, which were abrogated by irradiation. One was isotype-nonspecific and the other was IgA-specific. Moreover, failure of IgA-specific helper T activity was demonstrated. The use of EBV as an agent that polyclonally activates B cells independently of T cells and monocytes should allow a clearer delineation of the level of the B cell defects. When co-cultured with EBV, B cells from 3 patients with CVH produced normal to subnormal quantities of IgM although they could produce no IgM upon co-culturing with normal T cells and PWM. B cells from 2 patients with CVH could produce IgM normally by stimulation with either PWM or EBV; however, there was no restoration to produce IgG or IgA in these patients. In addition, B cells from 2 patients with IgA-D produced not only IgG and IgM but also IgA almost normally at 4 days after in vitro stimulation with EBV.
mesh_terms: D000293:Adolescent; D000328:Adult; D000361:Agammaglobulinemia / Q000276:immunology / Q000503:physiopathology*; D001402:B-Lymphocytes / Q000276:immunology; D002648:Child; D004247:DNA / Q000096:biosynthesis; D004406:Dysgammaglobulinemia / Q000276:immunology / Q000503:physiopathology*; D004854:Herpesvirus 4, Human / Q000276:immunology; D006801:Humans; D017098:IgA Deficiency; D007136:Immunoglobulins / Q000096:biosynthesis*; D008214:Lymphocytes / Q000276:immunology; D008875:Middle Aged; D011043:Pokeweed Mitogens / Q000494:pharmacology; D011947:Receptors, Antigen, B-Cell / Q000096:biosynthesis
chemical_list: D007136:Immunoglobulins; D011043:Pokeweed Mitogens; D011947:Receptors, Antigen, B-Cell; D004247:DNA