Management of Marginal Zone Lymphoma: A Canadian Perspective

Abstract

Marginal zone lymphomas (MZL) are a rare, heterogenous group of lymphomas, accounting for 5–17% of indolent non-Hodgkin lymphomas in the western world. They can be further divided into three subtypes: extranodal MZL, splenic MZL, and nodal MZL. These subtypes differ in clinical presentation and behavior, which influences how they are managed. There is currently no standard of care for the treatment of MZL, owing to the difficulty in conducting phase 3 randomized trials in MZL, and the fact that there are limited data on the efficacy of therapy in individual subtypes. Treatment practices are thus largely borrowed from other indolent lymphomas and are based on patient and disease characteristics, as well as access to therapy. This review summarizes the Canadian treatment landscape for MZL and how these therapies may be sequenced in practice.

Keywords

• marginal zone lymphoma
• mucosa-associated lymphoid tissue lymphoma
• chemoimmunotherapy
• anti-CD20 monoclonal antibody
• Bruton’s tyrosine kinase inhibitor