Marginal Zone Lymphoma: Clinicopathologic Variations and Approaches to Therapy.

PURPOSE OF REVIEW: The purpose of the study is to summarize the current conundrums in the management of marginal zone lymphomas (MZL).

RECENT FINDINGS: In 2017, the US Food and Drug Administration (FDA) approved ibrutinib, a first in class Bruton Tyrosine Kinase inhibitor, for the treatment of relapsed/refractory MZL based on pivotal open-label phase II trial demonstrating an overall response rates of 48%. Clinical trials design utilizing chemotherapy-free regimens for relapsed/refractory disease are gaining popularity. Recent studies have identified multiple genetic biomarkers that helped characterize and prognosticate different subtypes of MZL. MZLs are heterogeneous, mostly indolent, malignancies derived from B lymphocytes. Three disease subtypes are recognized, extranodal, nodal, and splenic. The disease characteristics, clinical picture, and treatment algorithms vary considerably based on subtype and site of involvement. Recent discoveries have enhanced our knowledge of the pathogenesis of MZLs leading to development of more accurate prognostic models as well as novel targeted systemic therapies.