IMMUNOBLASTIC SARCOMA OF T-CELL VERSUS B-CELL ORIGIN .1. CLINICAL-FEATURES

Abstract

Within the Lukes-Collins classification system of malignant lymphoma, a tumor of large transformed lymphocytes, termed immunoblastic sarcoma (IBS), is described. This morphological type would have been included within the histiocytic category of Rappaport. IBS may be of B-lymphocytic or T-lymphocytic origin. Since differences or similarities of these 2 variants have not yet been described, the case histories of 35 such patients who had immunologic marker studies performed were reviewed. Nineteen patients had T-cell IBS (T-IBS); 16 had B-cell IBS (B-IBS). Median age for both groups was .apprx. 50 yr. A history of prior immune disorder was found in 31% of B-IBS and 16% of T-IBS cases. Prior lymphoproliferative malignancy was noted in 21% of T-IBS and 13% of B-IBS patients. All T-IBS patients first presented because of lymphadenopathy: 56% of B-IBS cases initially presented because of extranodal disease. System B symptoms were common in both. Similarly, most patients had widespread disease (stage III or IV) at diagnosis. Clinically suspected hepatic (P = 0.05) and retroperitoneal node (P = 0.01) involvement were more often found in T-IBS. Of the T-IBS patients, 41% demonstrated polyclonal hypergammaglobulinemia, a finding not seen in B-IBS patients (P = 0.02). Although not statistically significant because of small numbers of patients, data on therapy and survival suggest that IBS of B-cell type may be successfully treated with aggressive, multiagent chemotherapy, while alternative approaches appear warranted in T-cell disease.