Biology and therapy of mantle cell lymphoma

Mantle cell lymphoma is an aggressive non-Hodgkin's lymphoma characterized by the t(11;14) chromosomal translocation and overexpression of cyclin D1. Constituting approximately 5 to 8% of all non-Hodgkin's lymphomas, it carries the poorest prognosis among non-Hodgkin's lymphoma subtypes. Standard and effective treatment approaches have yet to be established.

Several recent insights regarding the molecular pathogenesis and prognostic biomarkers have been realized by way of comparative genomic hybridization, cDNA microarray, and proteomic analysis. Clinical trials using chemotherapy plus rituximab have shown improved response rates, including complete remissions, but without cure in most cases, indicating a clear need for new treatment approaches. Novel therapies for relapsed disease using the proteasome inhibitor bortezomib, thalidomide plus rituximab, the cyclin inhibitor flavopiridol, or inhibitors of the mammalian target of rapamycin (mTOR) have shown encouraging clinical responses. Stem cell transplantation, including nonmyeloablative approaches, are being incorporated into therapeutic regimens and show promise in both the front-line and relapsed/refractory settings.

This review summarizes recent advances in the biology, pathogenesis, and therapy of mantle cell lymphoma and identifies ongoing areas of clinical investigation and new treatment approaches.