The pathologic diagnosis of mantle cell lymphoma

Mantle cell lymphoma (MCL) is a mature B-cell non-Hodgkin lymphoma usually characterized by t(11;14) (q13;q32), or CCND1 translocation and Cyclin D1 over expression. A very small subset of MCL may lack the t(11;14) (q13;q32) translocation and Cyclin D1 over expression, but show alternative translocations involving CCND2 and CCND3, and over expression of SOX11. In general, MCL has been considered a very aggressive and incurable lymphoma and patients with MCL usually have a poor prognosis. However, indolent variants, including in situ mantle cell neoplasm and the recently recognized leukemic non-nodal MCL do exist. In recent years, genome-wide molecular genetic studies have revealed a characteristic MCL genetic profile. This review will focus on the pathologic diagnosis of MCL using the traditional morphological and immunophenotypic strategies combined with cytogenetic characteristics and recently identified molecular profile. Morphological subtypes, immunophenotypic variants, recently recognized indolent variants, as well as MCL risk stratification will also be discussed.