CD5-negative mantle cell lymphoma: clinicopathologic features of an indolent variant that confers a survival advantage

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 We performed the most extensive review to-date of all reported cases of CD5-negative MCL and included unpublished cases diagnosed at our institutions to further characterize this disease subset.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.

Keywords:

• Lymphoma and Hodgkin disease
• lymphocytes
• prognostication

Acknowledgements

Dr. Elias Campo is acknowledged for his helpful advice.

Author contribution

AS and NSS designed the research, analyzed the results, made the figures, and wrote the manuscript. NSS, SEMH, DL, MR, WW, AW provided gene expression data on CD5+ MCL patients. AN, SSC, IS, MN, provided unreported data of SOX11 expression and/or IGHV mutational status on CD5-negative MCL patients. JL performed the bio-statistical analysis. HS and JS proofread the manuscript and provided critical feedback. All authors read and approved the final manuscript.

Disclosure statement

Authors declare no conflict of interest.