Marginal Zone Lymphoma: Clinicopathologic Variations and Approaches to Therapy

  • Sabarish Ayyappan
  • Basem M. William
Lymphomas (MR Smith, Section Editor)
Part of the following topical collections:
  1. Topical Collection on Lymphomas


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.


Lymphoma Non-Hodgkin Marginal zone Ibrutinib Mucosal Splenic villous Indolent Rituximab Splenectomy 


Compliance with Ethical Standards

Conflict of Interest

Sabarish Ayyappan and Basem M. William declare they have no conflict of interest.

Human and Animal Rights and Informed Consent

This article does not contain any studies with human or animal subjects performed by any of the authors.


Papers of particular interest, published recently, have been highlighted as: • Of importance •• Of major importance

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© Springer Science+Business Media, LLC, part of Springer Nature 2018

Authors and Affiliations

  1. 1.Division of Hematology, Department of Internal MedicineThe Ohio State University Comprehensive Cancer Center (OSUCCC-James)ColumbusUSA

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