MANTLE CELL LYMPHOMA - PRESENTING FEATURES, RESPONSE TO THERAPY AND PROGNOSTIC FACTORS

BACKGROUND. The goal of this study was to analyze the presenting featu res, natural history, and prognostic factors in 59 patients with well characterized mantle cell lymphoma (MCL). METHODS. Cases were classifi ed as nodular or diffuse and as typical or blastic variants. Age, perf ormance status (PS), histologic variants, mitotic index (MI), hematolo gic parameters, tumor extension data, and International Prognostic Ind ex (IPI) were recorded and evaluated for prognosis. RESULTS. The media n age of the patients was 63 years (range, 39-83 years), and the male to female ratio was 3:1. Fifty-three patients had typical histology (3 nodular and 50 diffuse), and 6 had the blastic variant. Approximately 95% of patients presented with advanced stage disease (Ann Arbor Stag e III-IV). Leukemic expression was observed in 58%. Complete and parti al response rates were 19% and 46%, respectively. Parameters associate d with lower response rate were Stage IV, high/intermediate or high ri sk IPI, and increased lactate dehydrogenase (LDH) level. In the logist ic regression analysis, high LDH level and Stage IV disease were assoc iated independently with lower response rate. Median survival was 49 m onths. Parameters associated with a short survival were: poor PS, sple nomegaly, B-symptoms, MI > 2.5, leukocyte count > 10 X 10(9)/L, high L DH level, blastic variant, and high/intermediate or high risk IPI. In the Cox proportional hazards regression model, only poor PS (relative risk [RR] = 3.3; P = 0.002), splenomegaly (RR = 2.8; P = 0.007), and M I > 2.5 (RR = 2.4; P = 0.012) were associated with short survival. CON CLUSIONS. In this series, patients with MCL presented with advanced st age and extranodal involvement. Only a minority of patients achieved a complete response. The median survival was 4 years, with PS, splenome galy, and MI being the most important factors predicting survival. The se results show clearly that more effective therapies for MCL are need ed. (C) 1998 American Cancer Society.