CD5-negative mantle cell lymphoma: clinicopathologic features of an indolent variant that confers a survival advantage
Arshia Soleimani, Alba Navarro, Delong Liu, Sarah E. M. Herman, Shih‐Sung Chuang, Irma Slavutsky, Marina Narbaitz, Hana Safah, John Schmieg, John J. Lefante, Mark Roschewski, Wyndham H. Wilson, Adrian Wiestner, Nakhle S. Saba
Abstract
Conventionally, mantle cell lymphoma (MCL) is an aggressive CD5-positive B-cell malignancy with poor prognosis and limited survival. However, a small subset of patients presents with indolent disease and can be managed on a 'watch and wait' approach. CD5-negative MCL has recently been recognized as a more favorable variant of MCL, but its clinical and biological implications remain ill-defined. We performed the most extensive review to-date of all reported cases of CD5-negative MCL and included unpublished cases diagnosed at our institutions to further characterize this disease subset. Based on our analysis of 356 cases of CD5-negative MCL, we conclude that median overall survival exceeds 14 years and is independent of favorable prognostic markers such as leukemic non-nodal disease, absence of SOX11, and low Ki-67.