Mantle Cell Lymphoma: Current and Emerging Treatment Strategies and Unanswered Questions

Abstract

Mantle cell lymphoma (MCL) is biologically and clinically heterogeneous with no clear standard of care. Overexpression of cyclin D1 is a hallmark of MCL. Evolving characterization of other molecular drivers explain a variety of disease phenotypes. These molecular profiles challenge risk stratification techniques. TP53-deleted disease is associated with adverse outcomes. Frontline treatment programs include intensive chemoimmunotherapy and autologous stem cell transplantation. Minimal residual disease may change management of MCL and guide therapy. As commonly dysregulated pathways become enumerated, novel biologically targeted agents and their combinations have been developed that will increasingly replace older, more toxic, and less efficacious regimens.