Mantle-Cell Lymphoma

For many years, pathologists were aware of a lymphoma of small lymphocytes that did not fit into existing classifications. Early names proposed for this lymphoma with a diffuse growth pattern were intermediate lymphocytic lymphoma and centrocytic lymphoma.^1,2 Mantle-zone lymphoma was the name for the same lymphoma in expanded mantle zones³; in addition to a typical morphologic appearance, this lymphoma had a characteristic immunophenotype (i.e., CD5+, CD10−, Bcl-2+, Bcl-6−, CD20+), with the t(11;14)(q13;q32) chromosomal translocation, and expression of cyclin D1. In 1990, t(11;14) was shown to be associated with intermediate lymphocytic lymphoma and centrocytic lymphoma.^4,5 In 1991, Raffeld and Jaffe first proposed the term mantle-cell lymphoma.⁶ Previously, mantle-cell lymphomas were given a variety of names based on the then-extant criteria for histologic classification; often they were confused with small lymphocytic lymphoma or were named diffuse small-cleaved-cell lymphoma. Other names they were given include lymphoblastic lymphoma, diffuse large-B-cell lymphoma, or follicular lymphoma. Initial studies of survival among patients treated for mantle-cell lymphoma showed a poor outcome after standard chemotherapy with cyclophosphamide, vincristine, and prednisone (CVP) and cyclophosphamide, doxorubicin, vincristine, and prednisone (CHOP), with a median survival of approximately 3 years, and long-term disease-free survival was rare.^7,8 However, with the development of rituximab and regimens specific for this disorder, survival has improved.