Mantle celllymphoma(MCL) is auniquesubtype of non-Hodgkin lymphoma (NHL) characterized by the chromosomal translocation t(11;14)(q13;q32) and nuclear cyclin D1 overexpression. It was first recognized as a distinct entity in the Kiel Classification in the 1970s, when it was designated “centrocytic lymphoma.” Subsequent descriptors included mantle zone lymphoma and intermediate lymphocytic lymphoma. In 1992, based on the recognition of characteristic morphologies, phenotype, and the t(11;14), the term “mantle cell lymphoma” was proposed to reflect the apparent derivation from mantle zone B-cells. Following this advance, focused clinical and pathogenesis studies became possible and have led to an evolving understanding of MCL, its clinical and biologic spectrum, and special therapeutic challenges.
Most patients present with advanced stage disease, often with extranodal dissemination, and, according to biological and clinical risk factors, may pursue either an indolent, steadily progressive, or an aggressive clinical course. No standard curative therapy exists aside from allogeneic transplantation. However, immunochemotherapy regimens with consolidative autologous stem cell transplantation in younger patients, an increasing number of effective therapies for sequential application, novel targeted agents, and maintenance therapy strategies are improving response durations and overall survival (OS).
MCL comprises approximately 4–6% of all NHL, 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. No specific etiologic factors have been identified for this disease.