Mantle-cell lymphoma: classification and therapeutic implications

Abstract

Mantle-cell lymphomas have been recognized in the new Revised European-American Lymphoma Classification as a peripheral B-cell neoplasm that has a distinct morphologic, immunologic, and genetic phenotype. Mantle-cell lymphomas have been subtyped into four categories, termed 'mantle zone', 'nodular', 'diffuse', or 'blastoid'. The incidence of the 'mantle-zone' pattern remains controversial. The fact that patients with the nodular, diffuse, or blastoid subtypes of mantle-cell lymphoma have a high proliferative rate resulting from overexpression of the cyclin D1 and a very short median survival demonstrates conclusively that these patients should be categorized as having an aggressive lymphoma. Most authorities believe that the 'mantle zone' variant pursues a more benign clinical course than the other subtypes. Trials of the new purine analogs are of great interest in these mantle-zone lymphoma patients.