title: Defect in B cell function in HTLV III/LAV positive hemophilia patients.
abstract: The capacity of the peripheral blood lymphocytes (PBL) to generate an antibody response in vitro T cell-dependent antigen ovalbumin was studied in 12 severe hemophilia patients who were otherwise in good health. PBL from four of 12 patients were not capable of generating such a response after stimulation in vitro, whereas all controls were normal. This negative plaque-forming cell (PFC) response coincided with the presence of antibodies directed toward human T-lymphotropic virus III/lymphadenopathy-associated virus (HTLV-III/LAV). Only one patient with antibodies against HTLV-III/LAV had a normal PFC response. The negative PFC response was not due to a deficient T helper cell activity, nor to an excessive T suppressor cell function. However, in the peripheral blood of these four patients, the presence of activated B cells that are refractory to antigen-specific T helper cell signals and secrete specific antibodies spontaneously could be demonstrated. Most of the patients showed a hyperimmunoglobulinemia. No correlation between the T4/T8 ratio and the level of the PFC response was demonstrable. From the data obtained in these investigations we raise the hypothesis that infection with HTLV-III/LAV in hemophilia patients will lead to in vivo (pre)activation of B cells that results in unresponsiveness or decreased response to antigen-specific signals.
mesh_terms: D000293:Adolescent; D000328:Adult; D000914:Antibodies, Viral / Q000032:analysis*; D001402:B-Lymphocytes / Q000145:classification / Q000378:metabolism / Q000502:physiology*; D002478:Cells, Cultured; D002648:Child; D015483:HIV Antibodies; D006467:Hemophilia A / Q000097:blood*; D002836:Hemophilia B / Q000097:blood; D006801:Humans; D007075:Immunoglobulin M / Q000378:metabolism; D007157:Immunologic Surveillance; D008875:Middle Aged; D010047:Ovalbumin / Q000494:pharmacology; D013601:T-Lymphocytes / Q000145:classification / Q000276:immunology; D006377:T-Lymphocytes, Helper-Inducer / Q000502:physiology; D050378:T-Lymphocytes, Regulatory / Q000502:physiology
chemical_list: D000914:Antibodies, Viral; D015483:HIV Antibodies; D007075:Immunoglobulin M; D010047:Ovalbumin