Current and Future Therapies for Marginal Zone Lymphoma

Abstract

Marginal zone lymphoma (MZL) is an indolent non-Hodgkin lymphoma with three subtypes: nodal, extranodal and splenic. Initial therapies can vary based on the subtype of MZL, location of disease and stage of disease. Treatment of MZL in the relapsed refractory (R/R) setting has evolved in recent years with the approvals of Bruton's tyrosine kinase inhibitors, phosphoinositide 3-kinase inhibitors and an immune modulatory drug, lenalidomide. Questions remain as to how best to use these agents to maximize efficacy and minimize toxicity. In this article, we focus on the management of MZL with currently available agents, particularly in the R/R setting. We also examine the therapies that may further change the treatment paradigm for MZL. Specifically, we discuss the available data for chimeric antigen receptor T-cell therapies and CD20–CD3 bispecific antibodies, and consider the limitations and potential benefits of these approaches.