Defect in production of B cell differentiation factor-like activity by mononuclear cells from a boy with hypogammaglobulinemia.
The case history of a boy who presented at 6 mo of age with pneumocystis carinii pneumonia is described. Hypogammaglobulinemia was detected. His T lymphocytes and B lymphocytes proliferated with mitogens but no immunoglobulin was secreted secondary to polyclonal stimulation with pokeweed mitogen. His peripheral blood mononuclear cells secreted interleukin 2 and B cell growth factor (BCGF) but failed to secrete detectable levels of B cell differentiation factor (BCDF). Studies of his B lymphocytes showed that they would secrete immunoglobulin in vitro after exposure to a supernatant containing BCDF activity. Hence regulatory control of these lymphokines appears to be independent, and this case illustrates the pathologic sequellae of a defect in BCDF-like production.
D000361:Agammaglobulinemia / Q000150:complications / Q000276:immunology* / Q000473:pathology; D000917:Antibody Formation; D001402:B-Lymphocytes / Q000276:immunology / Q000473:pathology*; D002454:Cell Differentiation; D006133:Growth Substances / Q000172:deficiency* / Q000378:metabolism; D006801:Humans; D007223:Infant; D007376:Interleukin-2 / Q000378:metabolism; D015847:Interleukin-4; D008213:Lymphocyte Activation; D008222:Lymphokines / Q000172:deficiency* / Q000378:metabolism; D008297:Male; D009000:Monocytes / Q000378:metabolism*; D009894:Opportunistic Infections / Q000209:etiology; D011020:Pneumonia, Pneumocystis / Q000209:etiology
D006133:Growth Substances; D007376:Interleukin-2; D008222:Lymphokines; D015847:Interleukin-4