Novel therapies for relapsed/refractory mantle cell lymphoma

Mantle cell lymphoma is an aggressive Non-Hodgkin's lymphoma that is considered incurable with standard therapies. Most patients treated with frontline immunochemotherapy relapse within a few years and do not usually respond to salvage chemotherapy. Persistent activation of the B-cell receptor pathway is critical to the pathogenesis of mantle cell lymphoma. Inhibition of Bruton's tyrosine kinase, an essential B-cell receptor pathway component with ibrutinib has shown clinical activity and has changed how MCL is treated in the relapsed/refractory setting. However, resistance to ibrutinib is common and response is limited. Novel agents targeting the B-cell receptor pathway along with therapies outside of the pathway will be reviewed in this article. Ongoing and future studies will better define how these agents should be utilized in the ever-changing treatment landscape of mantle cell lymphoma.