MANTLE CELL LYMPHOMA IN THE CHINESE - CLINICOPATHOLOGICAL FEATURES AND TREATMENT OUTCOME

We report the clinical, molecular, and immunohistological findings of 20 Chinese patients with mantle cell lymphoma diagnosed over a 10-year period. The disease affected mainly elderly patients (median age, 65. 5 years) with a male predominance (M/F, 3/1), Eighty percent presented with advanced stage III/IV disease but only 25% had B symptoms. Eight y-five percent had extranodal disease at presentation. Complete remiss ion (CR) and partial remission (PR) were achieved in 45% and 40% of th e patients, respectively. There was no difference in the CR rate for p atients treated with anthracycline-containing or nonanthracycline-cont aining regimens (43% and 50%, P=0.67). Disease progression or relapse was observed after a median of 26 months in patients who initially res ponded to treatment. Extranodal relapse occurred in the central nervou s system (n = 1), bone marrow (n = 1), pleura (n = 2), orbit (n = 2), and the gastrointestinal tract (n = 3). The median overall survival (O S) was 52 months but there were no long-term survivors. This was not d ifferent from the median OS of 53 months of patients with diffuse larg e cell (DLC) lymphoma treated in the same center over the same period (log rank, P = 0.76), Of the 12 patients who were tested for bcl-1 rea rrangement by polymerase chain reaction (PCR), five (42%) were positiv e for rearrangement in the major translocation cluster (MTC) region. T he median OS rates were 45 months and 63 months for PCR positive and n egative patients, respectively (P = 0.97). In conclusion, MCL is a dis ease mainly of the elderly in the Chinese with a male predominance and most had advanced-stage disease and extranodal involvement at present ation. Clinicopathologic features and treatment outcome were similar t o Caucasian patients, in that the disease combined the aggressive natu re of DLC lymphoma and the incurability of low-grade lymphoma. (C) 199 8 Wiley-Liss, Inc.