Journal of Clinical Oncology, Vol 14, 1269-1274, Copyright © 1996 by American Society of Clinical Oncology
Mantle-cell lymphoma: a population-based clinical study
GA Velders, JC Kluin-Nelemans, CJ De Boer, J Hermans, EM Noordijk, E Schuuring, MH Kramer, WA Van Deijk, JB Rahder, PM Kluin and JH Van Krieken
Department of Hematology, Leiden University Hospital, Leiden, The Netherlands.
PURPOSE: From a population-based non-Hodgkin's lymphoma (NHL) registry, 41
patients with mantle cell lymphoma (MCL) -- a recently defined distinct
B-cell NHL -- were selected and compared with patients with low- or
intermediate-grade NHL from the same registry. PATIENTS AND METHODS: The
incidence and behavior of MCL in the area of the Comprehensive Cancer
Center West (CCCW) from 1981 to 1989 were analyzed. Age, performance, tumor
bulk, extranodal localization, stage, response to therapy, and survival
were registered. Expression of cyclin D1 protein and Ki-67 were measured in
29 patients. RESULTS: MCL made up 3.7% of NHLs. The median age was 68
years, and the male-to-female ratio was 1.6:1. Seventy-eight percent
presented with stage IV, with the majority having bone 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 five patients with a low-risk
score and a median survival time of 93+ months. In 23 of 29 patients,
cyclin D1 overexpression was present, without any relation to overall or
disease- free survival. In contrast, a proliferative index less than 10%
was significantly related to a better overall survival time (50 v 24
months). CONCLUSION: MCL is a disease of the elderly, who present with
widespread disease and with a poor response to therapy. Although it harbors
features of an indolent NHL, it behaves clinically as an aggressive NHL
with a short overall survival time.
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