ABSTRACT

Mantle cell lymphoma (MCL) is derived from antigen-naïve pregerminal center B cells located in the mantle zone surrounding normal germinal centers. MCL is characterized by disseminated disease, with most patients presenting with stage III or IV disease, lymphadenopathy, hepatosplenomegaly, and bone marrow and blood involvement. The addition of rituximab to transplantation protocols appears to be a very promising strategy for patients with relapsed MCL after autologous stem cell transplantation. MCL appears to be largely resistant to complete eradication by conventional chemotherapy. The value of International Prognostic Index in MCL is not well established, partly because most of the patients are within high-risk categories. MCL with a mantle zone growth pattern may be difficult to diagnose without immunohistochemical staining for B-cell lymphoma 1. Apart from “classic” MCL with rather monotonous proliferation of small- to medium-sized cells with irregular nuclei and inconspicuous nucleoli, two other cytologic variants have been recognized: blastoid and pleomorphic.