Patients with
angioimmunoblastic lymphadenopathy (AILD)-type
T-cell lymphoma may develop
hypergammaglobulinemia. Among four cases of AILD-type
T-cell lymphoma that we have studied, we detected a correlation between the number of plasma cells in tissue and the extent of
interleukin-6 (IL-6) expression in
lymphoma cells. We did not detect
IL-6 in three patients who had no
hypergammaglobulinemia and whose tissues showed only minimal plasma cell infiltration. In the fourth patient we observed an abundant
IL-6 production by
lymphoma cells, which accounted for a B-cell plasmacytic tissue response and for
hypergammaglobulinemia. The pathogenic significance of
IL-6 was substantiated by a concomitant decrease in the serum
IL-6 level, measurable
tumor mass, and
immunoglobulin levels, as well as by a decline in the proportion of plasmacytoid cells in peripheral blood promptly on administration of
chemotherapy. Plasmacytoid B cells could be maintained in culture in the presence of
IL-6, but viability was lost on co-incubation with anti-IL-6.
Interleukin-1 and
tumor necrosis factor were not produced by T
lymphoma cells and were incapable of sustaining plasmacytoid B-cell viability in vitro. Small amounts of
IL-4 were noted in T
lymphoma cells. Thus, in this case of AILD-type
T-cell lymphoma,
tumor cells with a T-cell phenotype produced
IL-6 in large quantities, explaining the accompanying B-cell and plasmacytic histologic changes and humoral disease manifestations, including marked
hypergammaglobulinemia. Although not all cases of AILD-type
T-cell lymphoma have an accompanying plasma cell proliferation and
hypergammaglobulinemia, and although the
cytokine network in these patients may be more complex than has been recognized, this case with
IL-6 expression serves to illustrate the utility of
cytokine assays in the analysis of the histopathologic and clinical heterogeneities of
peripheral T-cell lymphomas.