What Is Marginal Zone Lymphoma (MZL)?

MZL is an indolent (slow-growing) non-Hodgkin lymphoma (iNHL) that arises from B cells in the marginal zone of the lymphoid follicle in the spleen, lymph nodes, or mucosa-associated lymphoid tissue (MALT).1,2

  • The marginal zone of the lymphoid follicle contains B cells and is found at the edge of lymphoid tissue3

MZL is thought to result from chronic immune stimulation due to infections as well as chromosomal rearrangements.1,4 See Pathogenesis of MZL.

MZL Subtypes

There are three subtypes of MZL1:

  1. Extranodal MZL: also known as MZL of the mucosa-associated lymphoid tissue (MALT) and makes up approximately 70% of MZL5,6
  2. Splenic MZL: typically occurs in the spleen and blood and makes up about 20% of newly diagnosed MZL5
  3. Nodal MZL: arises within the lymph nodes and makes up about 10% of all MZL5

Extranodal MZL, or MALT lymphoma, is the most common form of MZL and accounts for approximately 2/3 of all MZL.5 MALT lymphoma occurs outside the lymph nodes and is divided into two categories:5

Gastric MALT Non-gastric MALT
Develops in the stomach and is the most common site Develops outside of the stomach in places such as the small intestine, salivary gland, thyroid gland, eyes, and lungs

Clinical Presentation of MZL

MZL can involve many different tissues in the body, including the stomach, thoracic or abdominal lymph nodes, peripheral blood, spleen, and bone marrow. The presentation and symptoms of different types of MZL are often related to their sites of involvement. Patients also commonly present with peripheral lymphadenopathy (eg, lymph node disease, manifesting as small to medium superficial nodes) and splenomegaly (enlarged spleen).1 See Diagnosis of MZL.

US Statistics: MZL Incidence

MZL accounts for ~10% of all NHL1:

  • 7-8% gastric and non-gastric MALT
  • <2% nodal MZL
  • <1% splenic MZL

MZL is associated with an indolent disease course and relatively long survival.1 See Prognosis of MZL.


5-year relative survival rate (%)7*


*According to the National Cancer Institute Surveillance, Epidemiology, and End Results (SEER) Cancer Statistics Review 2008-2014 (n=9805).

References: 1. Referenced with permission from the NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®) for B-Cell Lymphomas V.4.2019. © National Comprehensive Cancer Network, Inc. 2019. All rights reserved. Accessed July 10, 2019. To view the most recent and complete version of the guideline, go online to NCCN.org. NCCN makes no warranties of any kind whatsoever regarding their content, use or application and disclaims any responsibility for their application or use in any way. 2. Leukemia & Lymphoma Society. NHL Subtypes. https://www.lls.org/lymphoma/non-hodgkin-lymphoma/diagnosis/nhl-subtypes. Accessed June 19, 2019. 3. Lymphoma Action. Nodal marginal zone lymphoma. https://lymphoma-action.org.uk/types-lymphoma-non-hodgkin-lymphoma/nodal-marginal-zone-lymphoma. Accessed May 16, 2019. 4. Zinzani PL, et al. ASH Ed Book. 2012:2012(1):426-432. 5. Lymphoma.org. Marginal Zone Lymphoma. https://www.lymphoma.org/aboutlymphoma/nhl/mzl/. Accessed January 20, 2019. 6. Denlinger NM, Epperla N, William BM. Cancer Manag Res 2018; 10: 615–624. 7. National Cancer Institute. SEER Cancer Statistics Review 2008-2014: Table 19.29. https://seer.cancer.gov/archive/csr/1975_2015/results_merged/sect_19_nhl.pdf. Accessed July 1, 2019. 8. Khalil MO, Morton LM, Devesa SS, et al. Incidence of marginal zone lymphoma in the United States, 2001–2009 with a focus on primary anatomic site. Br J Haematol. 2014;165(1):67–77.