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.
There are three subtypes of MZL1:
- Extranodal MZL: also known as MZL of the mucosa-associated lymphoid tissue (MALT) and makes up approximately 70% of MZL5,6
- Splenic MZL: typically occurs in the spleen and blood and makes up about 20% of newly diagnosed MZL5
- 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).