From the School of Medicine and Public Health and the Paul P. Carbone Comprehensive Cancer Center, University of Wisconsin, Madison, WI
Author's disclosure of potential conflicts of interest is found at the end of this article.
Address reprint requests to Brad S. Kahl, MD, Associate Professor, 600 Highland Ave, H4/534 CSC, Madison, WI 53792; e-mail: bsk{at}medicine.wisc.edu
Overview: Mantle cell lymphoma is a unique biologic, pathologic, and clinical entity. It is neither common nor rare, comprising 6% to 8% of new lymphoma diagnoses. The prognosis is historically reported to be unfavorable, but more recent data suggest the prognosis is improving with a median overall survival of 5 years in some series. Using a combination of morphology and immunohistochemistry, the diagnosis is relatively straightforward, although some misclassifications still occur. The biology of mantle cell lymphoma is reasonably well understood and targeted therapies based on this knowledge are in development. The optimal front-line treatment strategy is not defined and is a source of controversy. Several clinical trials attempting to incorporate new agents into standard therapies are underway and will be discussed in this review.
