Marginal Zone Lymphoma: State-of-the-Art Treatment

Ariel Sindel; Taha Al-Juhaishi; Victor Yazbeck

doi:10.1007/s11864-019-0687-5

Marginal Zone Lymphoma: State-of-the-Art Treatment

Curr Treat Options Oncol. 2019 Dec 5;20(12):90. doi: 10.1007/s11864-019-0687-5.

Authors

Ariel Sindel¹, Taha Al-Juhaishi¹, Victor Yazbeck^{2

3}

Affiliations

¹ Department of Internal Medicine, Virginia Commonwealth University, 401 College Street, Box 980035, Richmond, VA, 23298, USA.
² Department of Internal Medicine, Virginia Commonwealth University, 401 College Street, Box 980035, Richmond, VA, 23298, USA. vyyazbeck@vcu.edu.
³ Massey Cancer Center, Virginia Commonwealth University, Richmond, VA, USA. vyyazbeck@vcu.edu.

PMID: 31807935
DOI: 10.1007/s11864-019-0687-5

Abstract

Despite being the second most common indolent non-Hodgkin's lymphoma (iNHL), marginal zone lymphoma (MZL) remains largely understudied, and given its underlying disease heterogeneity, it is challenging to define a single treatment approach for these patients. For localized disease, local therapy is recommended such as triple therapy for H. pylori in gastric extranodal MZL, splenectomy for splenic MZL, and radiotherapy for nodal MZL. For disseminated disease with low tumor burden, a watch and wait or single-agent rituximab can be used. However, for symptomatic disease, a similar approach to follicular lymphoma (FL) can be used with chemoimmunotherapy approaches such as bendamustine and rituximab. High FDG uptake is not common in MZL and is not diagnostic by itself of transformation to high-grade lymphoma but informs the choice of the site to be biopsied. Transformation into a large B cell lymphoma is treated with R-CHOP-like regimens. Patients with relapsing disease after at least one CD20-based therapy have several recently approved chemotherapy-free options including B cell receptor inhibitors such ibrutinib (approved specifically in MZL) and immunomodulatory agents such as lenalidomide and rituximab (FDA approved in MZL and FL). Phosphoinositide 3-kinase (PI3K) inhibitors have shown excellent activity in iNHL, specifically in MZL, with breakthrough designation status for copanlisib and umbralisib, allowing off label use of this class of agents in clinical practice. With the availability of prospective clinical trials using chemo-free approaches, specifically those targeting abnormal signaling pathways activated in MZL tumors and its microenvironment, treating physicians are encouraged to enroll patients on these clinical trials in order to better understand the underlying biology, mechanisms of response, and resistance to current therapies and help design future rationale combination strategies.

Keywords: Immunotherapies; Indolent non-Hodgkin lymphoma; Marginal zone lymphoma; Targeted therapies; Transformed lymphomas; Treatment.

Publication types

Review

MeSH terms

Clinical Decision-Making
Combined Modality Therapy
Disease Management
Drug Resistance, Neoplasm
Humans
Lymphoma, B-Cell, Marginal Zone / diagnosis*
Lymphoma, B-Cell, Marginal Zone / etiology
Lymphoma, B-Cell, Marginal Zone / therapy*
Recurrence
Treatment Outcome