Marginal Zone Lymphoma (MZL)

What is marginal zone lymphoma?

Marginal zone lymphoma (MZL) refers to a group of rare, slow-growing non-Hodgkin lymphomas. They typically develop in lymphoid tissue. This tissue contains B-cells, a type of white blood cell that’s in parts of your immune system like your lymph nodes and spleen.

Your lymph nodes contain lymphoid follicles. Lymphoid follicles have two zones, or sections — mantle zones and marginal zones. Marginal zones wrap around mantle zones. Marginal zone lymphoma happens when B-cells in the marginal zone mutate (change), becoming abnormal cells that multiply excessively.

Marginal zone lymphomas usually affect people aged 60 and older. They tend to be more common in men and people assigned male at birth than in women and people assigned female at birth.

What are marginal cell lymphoma types?

There are three types of marginal zone lymphomas:

• Mucosa-assisted lymphoid tissue (MALT) lymphoma: This is the most common type of MZL. Healthcare providers may use the term “extranodal marginal cell lymphoma.” This type of marginal zone lymphoma may develop in the lining of your belly (gastric MALT) or in your lungs, skin, thyroid, salivary gland, bowels or near your eye (non-gastric MALT).
• Nodal marginal zone lymphoma: This type affects your lymph nodes but can appear in your bone marrow.
• Splenic marginal zone lymphoma: This rare type of marginal zone lymphoma affects your spleen, blood and bone marrow.

What are marginal zone lymphoma symptoms?

Marginal zone lymphoma typically grows very slowly. You may have this condition without having symptoms. Symptoms vary depending on the condition type. In general, marginal zone lymphomas cause the following symptoms:

• Fever.
• Night sweats.
• Unexplained weight loss.

MALT lymphoma symptoms

Extranodal marginal zone lymphoma symptoms vary based on the lymphoma location. For example, MALT lymphoma in your belly may cause:

• Nausea and vomiting.
• Belly pain.
• Feeling full even when you haven’t eaten.

Non-gastric MALT symptoms may include changes in your eye’s surface (conjunctiva) or tear (lacrimal) glands.

Nodal marginal zone lymphoma symptoms

Nodal marginal zone lymphoma symptoms may include:

• Fever.
• Night sweats.
• Unexplained weight loss.

Splenic marginal zone lymphoma symptoms

Splenic marginal zone lymphoma symptoms may include:

• Fatigue.
• Enlarged spleen.
• Night sweats.
• Unexplained weight loss.

What causes marginal zone lymphoma?

In general, people with marginal zone lymphoma have a family history of lymphoma, frequent infections or autoimmune diseases. The subtypes have specific causes:

• MALT lymphoma causes include bacterial infections, specifically H. pylori infections, autoimmune diseases like Hashimoto’s disease or Sjӧgren’s syndrome, or having a family history of lymphoma.
• Splenic marginal zone lymphoma is linked to hepatitis C and autoimmune diseases.
• Nodal marginal zone lymphoma is associated with hepatitis C.

What are marginal zone lymphoma risk factors?

Risk factors include having a family history of lymphoma and having certain infections and autoimmune disorders.

How is marginal zone lymphoma diagnosed?

Healthcare providers diagnose the condition by asking questions about your symptoms, your medical history and your family medical history.

What tests do providers use to diagnose marginal zone lymphoma?

Tests vary based on the sub-type. In general, tests may include:

• CBC.
• LDH test.
• Beta-2 microglobulin, a tumor marker for blood cancers.
• Liver function tests.
• Renal (kidney) function tests.
• Serum protein immunofixation (IFX). This test checks for changes in your blood.
• Certain bacterial and viral infections like H. pylori, hepatitis C and hepatitis.
• CT scan.
• Needle biopsy.
• Bone marrow biopsy.
• Biopsy to obtain samples of cells or tissue from various parts of your body.

What are MZL stages?

Healthcare providers use cancer staging systems to plan treatment and develop prognoses. MZL stages are:

• Stage I: Cancer in one lymphatic area.
• Stage II: Cancer in two more lymph nodes located above or below your diaphragm.
• Stage II: Cancer in several lymph nodes above and below your diaphragm.
• Stage IV: Cancer that’s has spread to multiple organs.

What are marginal zone lymphoma treatments?

Marginal zone lymphoma grows very slowly. People with this condition may not need immediate treatment. Healthcare providers instead may monitor people’s health until they determine that treatment is necessary. This is “watchful waiting” or active surveillance.

Treatments for MZL vary depending on the subtype but may include:

• Antibiotics to treat bacterial infections, specifically H. pylori.
• Chemotherapy.
• Radiation therapy.

Can marginal zone lymphoma be prevented?

This condition happens for several reasons, including autoimmune diseases and genetic issues you can’t control.

Is marginal zone lymphoma curable?

That depends on the condition type. For example, antibiotic treatment that eliminates H. pylori may cure extranodal marginal zone lymphoma. Other treatments put the condition into remission. Remission happens when cancer treatment eliminates symptoms and tests show no signs of disease. But marginal zone lymphoma may recur (come back) after treatment.

What is the survival rate for marginal zone lymphoma?

Survival rates vary based on the type of marginal zone lymphoma. One study suggests the following:

• An estimated 88% of people with extranodal marginal zone lymphoma were alive five years after diagnosis.
• An estimated 79% of people with splenic marginal zone lymphoma were alive five years after diagnosis.
• An estimated 76.5% of people with nodal marginal zone lymphoma were alive five years after diagnosis.

When you think about survival rates, it’s important to remember these are estimates based on other people’s experiences and data collected over time. Your experience may be different.

Marginal zone lymphoma affects people aged 60 and older. In many cases, people with this condition ultimately die from causes other than marginal zone lymphoma.

If you have marginal zone lymphoma, your healthcare provider is your best resource for information about your prognosis.

When should I see my healthcare provider?

If you’ve been diagnosed with marginal zone lymphoma, contact your provider if:

• You notice changes in your body that may be marginal zone lymphoma symptoms.
• You’re getting treatment and your symptoms get worse.
• You’re in remission, and notice changes that could indicate your condition is coming back (recurring).

What can I do to take care of myself?

Self-care is an important part of living with lymphoma, including rare lymphomas such as marginal zone lymphoma. Here are some steps you can take:

• Manage your stress. If you have marginal zone lymphoma, you may not have symptoms that require treatment. While that’s good news, it can be stressful wondering if you’ll have symptoms. Exercise, listening to music or activities like yoga may help.
• Drink enough fluids. Cancer treatment and side effects may cause dehydration.
• Eat a well-balanced diet. Focus on small meals that include fruit, vegetables, nuts and full-fat dairy products.

What questions should I ask my healthcare provider?

You may want to ask the following questions:

• What type of marginal zone lymphoma do I have?
• What’s my condition stage?
• Do I need treatment?
• If I need treatment, what treatment do you recommend?
• Are there clinical trials I should consider?

A note from Cleveland Clinic

Marginal zone lymphomas (MZLs) are a type of non-Hodgkin lymphoma. They’re rare and grow very slowly. There are different marginal zone lymphoma types with different symptoms, treatments and prognoses. Some people live with marginal zone lymphoma for years before needing treatment. Other people may get treatment that puts the condition into remission. If you have this condition, ask your healthcare provider what you can expect. They’ll be glad to explain how the condition affects your body and your treatment options.