Marginal Zone Lymphoma

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Synonyms

MZL, marginal zone B-cell lymphoma, lymphoma of marginal zone B cell, MZBCL,

Overview

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 than in women.

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.

Causes

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.

Prevention

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

Diagnosis

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.

Prognosis

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.

Treatment

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.