[Current treatment strategy in mantle cell lymphoma]

Mantle cell lymphoma (MCL) is a well-recognized lymphoma subtype that accounts for about 3-6% of all patients with non-Hodgkin lymphoma. The clinical course of MCL ranges from an indolent disease to a rapidly progressive malignancy, with a poor prognosis and a median overall survival (OS) of about 3-5 years reported in earlier data sets. Recent improvement has been achieved by the successful introduction of rituximab and dose-intensified approaches including high-dose cytarabine and autologous stem cell transplantation strategies especially in younger patients of age less than 66 years. In elderly patients, rituximab maintenance therapy after R-CHOP therapy was demonstrated to prolong survival. However, with the exception of allogeneic hematopoietic stem cell transplantation, current treatment approaches are non-curative, and the corresponding survival curves are characterized by a delayed but continuous decline and a median survival of 4-6 years. In recent years, new insights into the biology of MCL have been obtained which have provided the rationale for the development of novel therapeutic strategies. Emerging new drugs such as bendamustine, proteasome inhibitors, ibrutinib and others are based on the dysregulated control of cell cycle machinery and impaired apoptotic pathways. The efficacy of these agents as monotherapy was demonstrated to be comparable to conventional chemotherapy in relapsed MCL, and combination strategies are currently being investigated in clinical trials.