Should There Be a Standard Therapy for Mantle Cell Lymphoma?

Mitchell R Smith

Disclosures

Future Oncol. 2011;7(2):227-237. 

In This Article

What is Mantle Cell Lymphoma?

Mantle cell lymphoma is a subtype of B-cell lymphomas initially defined as a clinicopathologic entity in the Revised European American Lymphoma (REAL) classification in 1994.[1] Prior to that, it comprised most of the diffuse poorly differentiated lymphoma in the Rappaport classification, centrocytic lymphoma in the Kiel classification and had also been termed 'diffuse intermediate lymphoma'. These descriptions reflect that, histopathologically, MCL demonstrates a diffuse pattern of infiltration with small-to-medium-sized lymphocytes with somewhat irregular shape and angulated nuclei, except in the blastoid variant. By cell surface phenotype, the cells are B cells (i.e., CD19, CD20 and CD22+) co-expressing CD5. In contrast to chronic lymphocytic leukemia (CLL), these CD5+ B cells in MCL generally have bright CD20 and immunoglobulins and do not express CD23. Clinically, the median age of diagnosis, as for other lymphomas, is the seventh decade, but there is a male predominance. MCL tends to present with advanced-stage disease and has a propensity to involve the bone marrow, spleen and GI tract. As with other indolent lymphomas, MCL responds to, but is not curable with, standard therapies. Thus, many European studies of indolent lymphoma include MCL. However, response duration and survival in MCL is significantly shorter than other indolent lymphomas in general, so in the USA, it is often considered an aggressive lymphoma. Such divergent views reflect the clinical heterogeneity of MCL.

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