MANTLE-CELL LYMPHOMA - A POPULATION-BASED CLINICAL-STUDY

Purpose: From a population-based non-Hodgkin's lymphoma (NHL) registry , 41 patients with mantle cell lymphoma (MCL)-a recently defined disti nct B-cell NHL-were selected and compared with patients with low- or i ntermediate-grade NHL from the same registry. Patients and Methods: Th e incidence and behavior of MCL in the area of the Comprehensive Cance r Center West (CCCW) from 1981 until 1989 were analyzed. Age, performa nce, tumor bulk, extranodal localization, stage, response to therapy, and survival were registered. Expression of cyclin D1 protein and Ki-6 7 were measured in 29 patients. Results: MCL made up 3.7% of NHLs. The median age was 68 years, and the merle-to-female ratio was 1.6:1. Sev enty-eight percent presented with stage IV, with the majority having b one marrow involvement. The complete response (CR) rate was 32% (13 of 41), with a median duration of 25 months. The median overall survival time was 31.5 months. The International Prognostic Index identified f ive patients with a low-risk score and a median survival time of 93+ m onths. In 23 of 29 patients, cyclin D1 overexpression was present, wit hout any relation to overall or disease-free survival. In contrast, a proliferative index less than 10% was significantly related to ct bett er overall survival time (50 v 24 months). (C) 1996 by American Societ y of Clinical Oncology. Conclusion: MCL is a disease of the elderly, w ho present with widespread disease and with a poor response to therapy . Although it harbors features of an indolent NHL, it behaves clinical ly as an aggressive NHL with a short overall survival time.