Important note: Information in this article was accurate in 1981. The state of the art may have changed since the publication date.
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.
Keywords: Adolescence Adult Agammaglobulinemia/IMMUNOLOGY/*PHYSIOPATHOLOGY B-Lymphocytes/IMMUNOLOGY Child Dysgammaglobulinemia/IMMUNOLOGY/*PHYSIOPATHOLOGY DNA/BIOSYNTHESIS Herpesvirus 4, Human/IMMUNOLOGY Human IgA/*DEFICIENCY Immunoglobulins/*BIOSYNTHESIS Immunoglobulins, Surface/BIOSYNTHESIS Lymphocytes/IMMUNOLOGY Middle Age Pokeweed Mitogens/PHARMACOLOGY Support, Non-U.S. Gov't JOURNAL ARTICLE
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