Marginal Zone Lymphoma (MZL)

Overview of marginal zone lymphoma (MZL)

Marginal Zone Lymphoma is a group of indolent (slow-growing) B-cell non-Hodgkin lymphomas (NHL), that accounts for around 8% of all NHL cases.  The average age at diagnosis is 65 years, and overall, it is slightly more common in women than in men.

It is called ‘marginal zone’ lymphoma because it mainly affects lymphocytes at the edges of lymphoid tissue or nodes, in what is called the marginal zone. This can be seen when the tissue sample is studied under the microscope in the laboratory.

The three main types of marginal zone lymphoma (MZL) include:

• Mucosa-associated lymphoid tissue (MALT) lymphoma or also known as ‘extranodal MZL’ this is the most common form of MZL, and accounts for around two-thirds of all MZL cases per year.  
  • Gastric MALT lymphoma develops in the stomach and is the most common subtype of MALT lymphoma
  • Non-gastric MALT lymphoma develops outside of the stomach
• Nodal MZL: develops in the lymph nodes and accounts for about 10% of all MZL cases.
• Splenic MZL occurs most often in the spleen, blood and bone marrow and accounts for around 20% of MZL

Symptoms of marginal zone lymphoma (MZL)

Marginal Zone lymphoma is an indolent lymphoma, meaning it is usually slow growing. Symptoms may develop gradually over time and sometimes there are no symptoms and a diagnosis of MZL is by chance following tests done for other reasons. Common symptoms may include: 

• Enlarged lymph nodes
• Fatigue 
• Unexplained loss of weight
• Skin rash or itching of the skin
• Pains in the chest, abdomen (stomach) or bones for no obvious reason
• Fever for no known reason eg. Infection
• Drenching night sweats

Prognosis of marginal zone lymphoma (MZL)

Marginal zone lymphoma (MZL) develops slowly and, in most cases, it responds well to treatment (remission). However, this is an indolent lymphoma, and this means that it often relapses (comes back). If the lymphoma does relapse it can usually be treated again to keep it under control. The doctor can give you more information about your prognosis based on the grade and stage of the lymphoma. 

Treatment of marginal zone lymphoma (MZL)

The treatment for MZL is dependent on the subtype. The links below have specific information about the treatment for the different subtypes 

• Mucosa-associated lymphoid tissue (MALT) lymphoma
• Nodal marginal zone lymphoma
• Splenic marginal zone lymphoma

Treatments under investigation

There are many treatments that are currently being tested in clinical trials around the world and in Australia for patients with both newly diagnosed and relapsed MZL.