01

What is Marginal Zone Lymphoma?

Marginal zone lymphoma (MZL) is a type of slow-growing, non-Hodgkin lymphoma. MZL develops from a type of abnormal white blood cell called B cells. The precise cause of marginal zone lymphoma is unknown. It is, however, possibly linked to the bacteria Helicobacter pylori. Risk of developing MZL increases with age or with a compromised immune system.

02

Initial Marginal Zone Lymphoma Treatment

Medical providers must cover a few initial bases for patients diagnosed with marginal zone lymphoma. A treating physician should order a complete blood count, metabolic panel, and testing for the Helicobacter pylori bacteria. Imaging the chest, pelvis, and abdomen is also crucial to determine the most effective treatment strategy.

03

Types of Marginal Zone Lymphoma

There are three main types of marginal zone lymphoma:

Extranodal MZL or mucosa-associated lymphoid tissue (MALT)

MALT is the most common form of MZL. It will appear outside the lymph nodes in places like the stomach, lung, skin, or salivary gland. MALT tends to remain localized for longer periods of time. However, it also has a greater potential to progress into a more aggressive form of cancer.

Nodal MZL

This is the second most common type of MZL. It appears in the lymph nodes. Nodal MZL can also progress into a more aggressive form.

Splenic MZL

Splenic MZL is the least common presentation of the cancer. It appears in the spleen and blood.

04

Strategies for Marginal Zone Lymphoma Management

There is no single treatment method for marginal zone lymphoma. Appropriate care depends upon the stage of disease and the area in the body where the lymphoma appears. In general, care should commence upon detection of tumors or at the onset of symptoms.

Understand the appropriate treatment strategy for your MZL case

05

Treatment for Early Stage MZL

For MZL diagnosed in its early stages, radiation therapy at the disease site is the main treatment option. Moderate radiotherapy in a localized approach has shown a very good response rate in MZL patients. Surgery may be an initial option as well for cancers in locations not eligible for radiotherapy.

06

Treatment for Advanced Stage MZL

For more advanced MZL, the recommended treatment is immunotherapy using a rituximab-based regimen. Rituximab is an antibody that targets an antigen present in cancerous B cells. In more aggressive cases, R-CHOP is another option. R-CHOP is a combination of five drugs (rituximab, cyclophosphamide, doxorubicin, vincristine, prednisone) used as a chemotherapy regimen. R-CHOP should be reserved only for more extreme clinical scenarios.

07

MZL Treatment Toxicity Risks

Several complications exist in relation to common marginal zone lymphoma treatments. For MZL in the parotid gland, radiotherapy and R-CHOP may be used in instances of relapse. However, these courses of treatment are linked to a delayed complication called xerostomia. This is a severe dry mouth condition that can cause issues with swallowing and appropriate nutrition intake.
R-CHOP chemotherapy is also linked to an array of toxic side effects. These include increased risk of infection, congestive heart failure, and anemia. When utilized over a longer period of time for maintenance treatment, R-CHOP may also cause delayed onset toxic side effects. These include respiratory issues, progressive multifocal leukoencephalopathy, and neutropenia.

08

Marginal Zone Lymphoma Prognosis

MZL patients generally have a good outlook with appropriate treatment. The median marginal zone lymphoma survival rate is greater than 10 years. The long-term impacts of the current therapeutic options, however, are still undergoing clinical study.

This Litigation Guide was medically reviewed by Amy Chang, M.D.