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Understanding Marginal Zone Lymphoma

Marginal zone lymphoma (MZL) is a slow-growing type of non-Hodgkin lymphoma (NHL), or blood cancer.

Lymphocytes, a type of white blood cell in the immune system, has two types that can grow uncontrollably and become cancerous: B lymphocytes (B cells) and T lymphocytes (T cells). Marginal zone lymphoma is a slow-growing, B-cell cancer that can spread throughout the lymph nodes, spleen, bone marrow, blood and other organs. Sometimes the cells form a mass called a tumor.1

There are three subtypes of MZL: extranodal, nodal, and splenic.2

MZL accounts for about 8% of all non-Hodgkin type cancers diagnosed and is slightly more common in women than men.

How does marginal zone lymphoma develop?

The most common form of MZL is extranodal or mucosa-associated lymphoid tissue (MALT) and occurs in about two-thirds of all MZL cases. It is found outside the lymph nodes, most often in the stomach, though sometimes in the breast, small intestine, salivary gland, thyroid, around the eyes, and lungs.

MALT lymphoma comes in two forms:

  • Gastric, so named because it most often develops in the stomach
  • Non-gastric, which develops outside the stomach

Many times, MALT is preceded by a medical history of chronic infection, inflammation, or autoimmune disorders affecting the organ where the cancer is found. For example, Helicobacter pylori (h. pylori), a bacteria known to cause stomach ulcers, is often found in patients with gastric MALT lymphoma.1

Nodal MZL is found in the lymph nodes and accounts for about a third of all MZL cases.1

Splenic MZL is usually found in the spleen, blood, and bone marrow, often in people who also have hepatitis C virus (HCV) infection. It occurs in about 8% of all MZL cases.1

Risk factors

Risk factors for developing MZL are:

  • Having Sjögren syndrome, lupus, or any other autoimmune condition that activates B-cells
  • Hepatitis C infection
  • Having a peptic ulcer
  • Having asthma
  • A first-degree relative (parent, sibling, child) with any leukemia or lymphoma
  • Hair–dye use
  • Occupations such as general carpenter, general laborer, painter, and metalworker2

Symptoms

Not everyone with MZL experiences symptoms, but for those who do, symptoms can be vague and include:

Other signs that point to MZL (particularly MALT) are gastric pain, nausea, an enlarged spleen, and signs of gastric bleeding such as anemia.4

Stages of MZL

MZL is usually diagnosed through a combination of physical exam, blood tests, biopsy, and imaging tests such as X-rays, ultrasounds, CT, PET and MRI scans.1,3

Stages one and two are considered early stages.

  • Stage 1: MZL is found in only one lymphatic area.
  • Stage 2: MZL is found in two or more lymph nodes, above or below the diaphragm.

Stages three and four are considered advanced stages.

  • Stage 3: MZL is found in several lymph nodes above and below the diaphragm.
  • Stage 4: MZL has spread to multiple organs.

What is a common prognosis?

Survival rates vary depending on the type of MZL a person has, their age at diagnosis, where the cancer is located, and the presence of other health issues. One study found that five-year survival rates between 1995 and 2009 were:5

  • MALT – 88.7%
  • Splenic – 79.7%
  • Nodal – 76.5%5

However, the American Society of Hematology published a study at roughly the same time and found slightly different results:

  • MALT – 85%
  • Splenic – 50-88%, depending on other risk factors
  • Nodal – 60-70%4

Given that MZL is a slow-growing cancer, early detection and watchful monitoring can improve the chances of long-term survival. Many new drugs for MZL and other slow-growing lymphomas are being studied.1

This article represents the opinions, thoughts, and experiences of the author; none of this content has been paid for by any advertiser. The Blood-Cancer.com team does not recommend or endorse any products or treatments discussed herein. Learn more about how we maintain editorial integrity here.

  1. Getting the facts: Marginal zone lymphoma. Lymphoma Research Foundation. Available at https://www.lymphoma.org/wp-content/uploads/2018/07/MarginalZoneLymphomaF_Fact-Sheet_2018.pdf. Accessed 10/29/18.
  2. Bracci P, et al. Medical History, Lifestyle, Family History, and Occupational Risk Factors for Marginal Zone Lymphoma: The InterLymph Non-Hodgkin Lymphoma Subtypes Project. J Natl Cancer Inst Monogr. 2014 Aug; 2014(48): 52–65. doi: 10.1093/jncimonographs/lgu011. Accessed 10/29/18.
  3. Nodal marginal zone lymphoma. Lymphoma Action. Available at: https://lymphoma-action.org.uk/types-lymphoma-non-hodgkin-lymphoma/nodal-marginal-zone-lymphoma. Accessed 10/29/18.
  4. Zinzani PL. American Society of Hematology. The many faces of marginal zone lymphoma. ASH Education Book December 8, 2012 vol. 2012 no. 1 426-432. Available at http://asheducationbook.hematologylibrary.org/content/2012/1/426.full. Accessed 10/29/18.
  5. Olszewski AJ1, Castillo JJ. Survival of patients with marginal zone lymphoma: analysis of the Surveillance, Epidemiology, and End Results database. Cancer. 2013 Feb 1;119(3):629-38. doi: 10.1002/cncr.27773. Epub 2012 Aug 14. Available at https://www.ncbi.nlm.nih.gov/pubmed/22893605. Accessed 10/29/18.

    Comments

  6. Ann Harper moderator
    6 months ago

    There are so many different forms of cancer. We hear so much about breast and prostate but not so much about other types. Thank you for sharing.

  7. Poll