Update on the molecular biology of mantle cell lymphoma

Abstract

Mantle cell lymphoma (MCL) accounts for 5-10% of non-Hodgkin's lymphomas and it bears the worst prognosis among B cell lymphomas. Even more than in the other lymphomas, new insights in MCL biology are needed. In this review we will discuss the most recent published data. Immunoglobulin sequencing data suggest that the MCL cell of origin is a mature B cell which might have undergone an extra-follicular T-cell independent antigen maturation. The main aberrations that affect cell cycle and DNA repair pathways in MCL are summarized, also discussing the need of the t(11;14)(q13;q32) to make diagnosis of MCL. After the gene expression studies of the last years, now the research focus has moved towards the new opportunities provided by arrayCGH technique for gene discovery and for identification of therapeutic targets. Examples of genes identified because deleted or amplified at DNA level and that might be relevant for MCL pathogenesis are presented.