Polyclonal immunoglobulin secretion in patients with common variable immunodeficiency using monoclonal B cell differentiation factors.
B cells from 25 patients with common variable immunodeficiency (CVI) were tested for their ability to differentiate under the influence of B cell differentiation factors (BCDF), derived from T cell hybridomas or T cell clones. 11 patients generated Ig plaque-forming cells in the range comparable to that of normal controls with supernatant from the T cell hybrid MOP 1L. With various hybrid or clone supernatants, differing response patterns emerged. Four patients who failed to respond to MOP 1L responded to T cell clone supernatant RAC. Another who failed to respond to both MOP 1L and RAC responded to T cell hybrid supernatant MTP 7. These results indicate that these supernatants contain different BCDFs and suggest heterogeneity in the differentiation states of B cells in CVI. In addition, three patients demonstrated exaggerated responses to BCDF, and evidence was obtained from B cells of these patients for increased BCDF receptor density. Thus, the accumulated evidence indicates that T cell defects may be a primary pathogenetic mechanism in common variable immunodeficiency, and purified BCDF may be of therapeutic value.
D000944:Antigens, Differentiation, B-Lymphocyte; D000954:Antigens, Surface / Q000276:immunology*; D001402:B-Lymphocytes / Q000276:immunology*; D002454:Cell Differentiation; D006801:Humans; D007153:Immunologic Deficiency Syndromes / Q000276:immunology*; D008213:Lymphocyte Activation; D011043:Pokeweed Mitogens / Q000494:pharmacology; D013997:Time Factors
D000944:Antigens, Differentiation, B-Lymphocyte; D000954:Antigens, Surface; D011043:Pokeweed Mitogens