Adult T cell lymphoma with hypergammaglobulinemia

Abstract

Five cases of adult T cell lymphoma with hyperglobulinemia were reported. Two patients died of extension of the disease despite intensive chemotherapy. Three others are still under treatment. Histologically, focal proliferation of monotonous pale cells and foci composed of polymorphic large, medium, and small cells were characteristic of all five cases. Infiltration by eosinophilic leukocytes and/or small histiocytic aggregates were also recognized. In addition, proliferation of branching vessels with hyaline deposits, perivascular proliferation of "immunoblasts" and plasma cells, absence of residual follicles, and depletion of small lymphocytes were the findings, which were consistent with immunoblastic lymphoadenopathy (IBL). Varying degrees of polyclonal hypergammaglobulinemia also corresponded with those of IBL. Rosette assay of the lymph node cells revealed a mixed population of E receptor-positive large, medium, and small cells with atypical nuclei. They also had T cell antigens on the surface and revealed no terminal deoxynucleotidyl transferase activity. These findings suggested their peripheral T cell origin. The cellular characteristics of these five cases are discussed in relation to other adult T cell lymphomas and to the helper function of neoplastic T cells.

Angio-immunoblastic lymphadenopathy: A clinical, immunological and molecular study

“…Only in the last few years has the proposition been advanced that many cases of AILD may in fact be Tcell lymphomas from the outset. This was first suggested by a group of Japanese workers (Shimoyama et al, 1979;Watanabe et al, 1980) (Stansfeld, 1985) al., 1975;Frizzera et al, 1974;Newcom et al, 1979a) because AILD was originally considered to be a benign hyperimmune akin to a drug reaction (Lukes & Tindle, 1975). It soon became apparent that this form of therapy was ineffective in maintaining a disease-free state in most patients.…”

“…It was widely believed that AILD was pre-malignant, and not malignant. At the end of the decade, Shimoyama et al, 1979 andWatanabe et al, 1980, described a form of peripheral T-cell lymphoma which was clinically and pathologically indistinguishable from AILD, as it has been described in the American and European literature. Since that time, the theory that AILD is, at least in the majority of cases, a special type of T-cell lymphoma, has steadily gained ground, although many of the curious features of this disease remain unexplained.…”

Peripheral T-cell lymphoma

“…IBL-like T-cell lymphoma, Lennert's lymphoma, and T-zone lymphoma are examples of this [56][57][58][59].…”

Pathology of peripheral t‐cell lymphomas and leukemias

“…We reported five patients with neoplastic proliferation of peripheral T-lymphocytes and with varying degrees of polyclonal hypergammaglobulinemia and have added nine cases since (Watanabe et al, 1980a;Shimoyama et al, 1982). There were 13 males and one female.…”

“…Most IBL-like T-cell lymphomas revealed polyclonal hypergammaglobulinemia. We formerly considered that the IBL-like T-cell lymphoma was derived from helper T-cells (Watanabe et al, 1980a). However, since the ATL lymphoma cells which possessed an inducer/helper T-cell phenotype caused in vitro suppression of Ig synthesis by B-lymphocytes, there is an apparent discrepancy between the expression of suppressor/cytotoxic T-cell epitopes on the proliferating neoplastic cells and in vitro helper activity and clinical hypergammaglobulinemia.…”