INTRODUCTION

Mantle cell lymphoma (MCL) is a unique subtype of non-Hodgkin's lymphoma (NHL) characterized in almost all cases by the chromosomal translocation t(11;14)(q13;q32) and nuclear cyclin D1 overexpression. Most patients present with advanced-stage disease, often with extranodal dissemination, and typically pursue an aggressive clinical course, with median survival historically averaging 3–4 years. No standard curative therapy exists, even with intensive induction regimens followed by autologous stem-cell transplantation. However, a number of recent insights into the molecular and cellular biology of the disease, as well as combined immunochemotherapy and novel therapeutic approaches, hold promise for improved outcomes. Importantly, MCL provides a paradigm for therapeutic targeting in neoplasms with dysregulated cell cycle and apoptotic pathways.

MCL comprises approximately 4–8% of all NHL, with a preponderance of older males relative to other lymphoma subtypes. The male-to-female ratio is 2–3 : 1 and median age at presentation is 60–65 years. No specific etiologic factors have been identified for this disease. An increased risk of lymphoid neoplasms has been reported in first-degree relatives of MCL patients, although MCL occurrence among multiple family members appears to be quite rare.

CLINICAL PRESENTATION

MCL typically presents in advanced stage; over 90% of patients are stage III–IV at diagnosis, frequently with B symptoms. Splenomegaly is seen in half or more of patients, often in association with a leukemic phase.