Marginal zone lymphomas (MZLs) are a rare slow-growing type of B-cell non-Hodgkin lymphoma. B cells (B lymphocytes) are a type of white blood cell. There are different MZL types, but all MZL starts in lymphoid tissue including skin, lymph nodes and spleen. Healthcare providers can treat and sometimes cure some types of MZL.
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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.
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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.
There are three types of marginal zone lymphomas:
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:
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Extranodal marginal zone lymphoma symptoms vary based on the lymphoma location. For example, MALT lymphoma in your belly may cause:
Non-gastric MALT symptoms may include changes in your eye’s surface (conjunctiva) or tear (lacrimal) glands.
Nodal marginal zone lymphoma symptoms may include:
Splenic marginal zone lymphoma symptoms may include:
In general, people with marginal zone lymphoma have a family history of lymphoma, frequent infections or autoimmune diseases. The subtypes have specific causes:
Risk factors include having a family history of lymphoma and having certain infections and autoimmune disorders.
Healthcare providers diagnose the condition by asking questions about your symptoms, your medical history and your family medical history.
Tests vary based on the sub-type. In general, tests may include:
Healthcare providers use cancer staging systems to plan treatment and develop prognoses. MZL stages are:
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:
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This condition happens for several reasons, including autoimmune diseases and genetic issues you can’t control.
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.
Survival rates vary based on the type of marginal zone lymphoma. One study suggests the following:
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.
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If you have marginal zone lymphoma, your healthcare provider is your best resource for information about your prognosis.
If you’ve been diagnosed with marginal zone lymphoma, contact your provider if:
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:
You may want to ask the following questions:
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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.
Last reviewed on 04/17/2023.
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