Malignant lymphoma of small cleaved lymphocytes of the follicular mantle zone

We describe a highly unusual lymphocytic lymphoma. It appeared to originate in the mantle zones of hyperplastic follicles that had large reactive centers. The tumor cells in the mantle zone were small lymphocytes with cleaved or very irregular nuclei. They had coarse and abundant IgM, kappa surface immunoglobulin markers, and receptors for complement. The tumor involvement was generalized at the time of discovery. The diagnosis of a malignant lymphoma was initially made with difficulty because of the presence of reactive follicular centers as well as a polyclonal hypergammaglobulinemia and large numbers of interfollicular plasma cells and plasmacytoid lymphocytes containing all classes of immunoglobulin. However, 2 yr later, the follicular centers were replaced by tumor nodules composed of lymphocytes identical in appearance and immunologic type to those seen originally. This case illustrates that not all nodular lymphomas are follicular center cell (FCC) neoplasms and that morphological transformation from small round to small cleaved lymphocytes and a corresponding increase in surface immunoglobulins may take place in the follicular mantle zone. The patient had a high titer of antibody to Epstein-Barr virus (EBV) and a poor lymphocyte response to concanavalin A, but neither the tumor cells nor the plasmacytoid lymphocytes contained EBV DNA.