Marginal Zone Lymphoma

Marginal zone lymphoma explained briefly

Marginal zone lymphoma (MZL) is a rare form of non-Hodgkin lymphoma, a cancer of the lymphatic system. In it, certain white blood cells (B lymphocytes) grow uncontrollably in the marginal zone of the lymphatic tissue. Marginal zone lymphoma usually progresses slowly and often goes unnoticed for a long time. Each year, around 1,000 to 1,500 people in Germany develop a marginal zone lymphoma. The disease mostly occurs in older adults, but in rare cases can also affect younger people.

Marginal zone lymphoma in figures

New cases per year in Germany

approx. 1,000 to 1,500

Typically affected

Older adults

Course

Usually slow

What exactly is marginal zone lymphoma?

Marginal zone lymphoma comprises various subtypes, which are distinguished according to their site of origin in the body. The three main types are:

• Extranodal marginal zone lymphoma of the MALT type (MALT lymphoma): This lymphoma mostly occurs outside the lymph nodes, frequently in the stomach, but also in organs such as the lung, the skin or the thyroid gland. It is often triggered by chronic inflammation such as an infection with Helicobacter pylori in the stomach.
• Nodal marginal zone lymphoma: This form arises within the lymph nodes themselves. It is considerably rarer than MALT lymphoma and affects exclusively the lymph nodes, without other organs being involved.
• Splenic marginal zone lymphoma: In this form, mainly the spleen is affected. The bone marrow and the blood can also be affected. An enlarged spleen is often characteristic.

Why do I have marginal zone lymphoma?

The exact causes of the development of a marginal zone lymphoma are not fully understood. However, there are some risk factors that can increase the likelihood of developing this disease:

• Chronic inflammation or infections: MALT lymphoma in particular is associated with long-lasting inflammation. An infection with Helicobacter pylori is a known risk factor for MALT lymphomas in the stomach.
• Weak immune system: People with a weakened immune system, e.g. due to an autoimmune disease or HIV infection, have a higher risk.
• Genetic predisposition: In some cases, a family history can increase the likelihood of developing a lymphoma.

Symptoms of marginal zone lymphoma

The symptoms of a marginal zone lymphoma depend on the affected region of the body. Because this disease usually grows slowly, it often goes unnoticed for a long time. Typical symptoms are:

• Enlarged lymph nodes: Above all in nodal marginal zone lymphomas.
• Abdominal pain and a feeling of fullness: Especially in splenic marginal zone lymphoma due to an enlarged spleen.
• Tiredness and weakness: General exhaustion occurs frequently.
• Fever, night sweats and unintentional weight loss (B symptoms): These symptoms can occur in advanced stages.

When is marginal zone lymphoma treated?

Because marginal zone lymphomas usually grow slowly, treatment is not always started immediately. In patients without severe complaints or progressing disease, a strategy of “watchful waiting" is often followed. This means that regular check-ups are carried out without therapy being started right away.

Treatment usually begins when:

• Symptoms become stronger or the disease progresses.
• Organs are impaired (e.g. the spleen in splenic marginal zone lymphoma).
• The lymphoma continues to grow despite observation.

How is marginal zone lymphoma treated?

The treatment methods depend on the type of marginal zone lymphoma and the stage of the disease. Common therapeutic approaches include:

• Antibiotics: For a MALT lymphoma in the stomach, antibiotic treatment to fight the Helicobacter pylori infection can cure the lymphoma in some cases.
• Immunotherapy: Monoclonal antibodies such as rituximab are often used to target the cancer cells.
• Radiotherapy: Local irradiation can be very effective for isolated foci, above all in MALT lymphomas.
• Chemotherapy: Used above all in advanced stages, often in combination with immunotherapy.
• Removal of the spleen: In splenic marginal zone lymphomas, removal of the enlarged spleen can be considered.