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Circulating activated B cells in primary biliary cirrhosis

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Abstract

Since patients with primary biliary cirrhosis (PBC) have evidence of abnormal function of the humoral immune system, we determined if B cells from patients with this disease show evidence of activation and can be stimulated by polyclonal activators. Using a reverse hemolytic plaque assay, it was found that patients with PBC had a significant increase in the number of circulating immunoglobulin-secreting cells, compared to normal controls and patients with chronic type B hepatitis virus (HBV) infection. However, the total number of activated cells was less than 1% of the total circulating B-cell population. Furthermore, we were unable to detect an increase in the expression of transferrin receptors, a membrane receptor associated with B-cell activation, in the majority of B cells in patients with PBC. In other studies, immunoglobulin production by lymphocytes from patients with PBC, when stimulated with the polyclonal activators pokeweed mitogen and Epstein-Barr virus (EBV), was reduced. This hyporesponsiveness was not due to a decrease in the number of B cells, as determined by staining with the monoclonal antibody anti-Leu 12. Furthermore, the decreased response of B cells to polyclonal activation in PBC patients was not due increased suppressor T-cell function, since EBV-stimulated cultures of lymphocytes from patients with PBC demonstrated diminished suppression of immunoglobulin-secreting cells after 14 days of culture compared to controls. These findings suggest that the humoral abnormalities in PBC are due to the activation of a small subpopulation of B cells rather than to generalized B-cell hyperactivity.

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Authors and Affiliations

  1. Mucosal Immunity Section, Laboratory of Clinical Investigation, National Institute of Allergy and Infectious Diseases, USA

    S. P. James & W. Strober

  2. Liver Diseases Section, Digestive Diseases Branch, National Institute of Arthritis, Diabetes, and Digestive and Kidney Diseases, National Institutes of Health, 20205, Bethesda, Maryland

    E. A. Jones & J. H. Hoofnagle

Authors
  1. S. P. James
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  2. E. A. Jones
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  3. J. H. Hoofnagle
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  4. W. Strober
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James, S.P., Jones, E.A., Hoofnagle, J.H. et al. Circulating activated B cells in primary biliary cirrhosis. J Clin Immunol 5, 254–260 (1985). https://doi.org/10.1007/BF00929460

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  • DOI: https://doi.org/10.1007/BF00929460

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