MANTLE-CELL LYMPHOMA - CLASSIFICATION AND THERAPEUTIC IMPLICATIONS

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. Mantl e-cell lymphomas have been subtyped into four categories, termed 'mant le zone: 'nodular', 'diffuse', or 'blastoid'. The incidence of the 'ma ntle-zone' pattern remains controversial. The fact that patients with the nodular, diffuse, or blastoid subtypes of mantle-cell lymphoma hav e a high proliferative rate resulting from overexpression of the cycli n DI and a very short median survival demonstrates conclusively that t hese patients should be categorized as having an aggressive lymphoma. Most authorities believe that the 'mantle zone' variant pu sues a more benign clinical course than the other subtypes. Trials of the new pur ine analogs are of great interest in these mantle-zone lymphoma patien ts.