Mantle cell lymphoma

Abstract

Mantle cell lymphoma (MCL) is a unique subtype of non-Hodgkin lymphoma (NHL) characterized by the chromosomal translocation t(11;14)(q13;q32) and nuclear cyclin D1 overexpression (Swerdlow and Williams, Hum Pathol 33:7-20, 2002; Williams et al, Leukemia 7(9):1437-1440, 1993a; Williams et al, Ann Oncol 6(3):297-299, 1995; Perez-Galan et al, Blood 117:26-38, 2011). Based on the recognition of characteristic morphologies, phenotype, and the t(11;14), the term "mantle cell lymphoma” was adopted in 1992 to reflect the apparent derivation from mantle zone B cells (Dreyling and Hiddemann, Hematology 2009:542-555, 2009). MCL comprises approximately 4-6 % of all non-Hodgkin’s lymphomas, with a preponderance of older males relative to other lymphoma subtypes. The male-female ratio is 2-3:1 and median age at presentation is 65 years (Swerdlow and Williams, Hum Pathol 33:7-20, 2002; Williams et al, Ann Oncol 6(3):297-299, 1995; Williams et al, Leukemia 7(9):1437-1440, 1993a; Perez-Galan et al, Blood 117:26-38, 2011). No specific etiologic factors have been identified for this disease. As with lymphoproliferative disorders in general, an increased risk of other lymphoid neoplasms is recognized among first-degree relatives of MCL patients, although MCL occurrence among multiple family members is quite rare.