Marginal zone lymphoma (MZL)

Extra nodal MZL sometimes known as mucosa-associated lymphoid tissue lymphoma (MALT). It is the most common type of MZL. It is found outside the lymph nodes in places such as; the stomach, small intestine, skin, salivary gland, thyroid, eyes and lungs.

This type pf MZL is divided into 2 categories. Gastric MZL, which develops in the stomach and non-gastric, which develops outside the stomach.

Treatment depends on the stage, location and if there are any underlying infections contributing to the lymphoma.

Nodal MZL is found in the lymph nodes and accounts for about 10% of all MZL cases. Treatment depends on your symptoms and stage of disease at diagnosis.

Splenic MZL occurs most often in the spleen and blood. It can be associated with a previous hepatitis C infection and accounts for approximately 20% of all MZL cases. Treatment depends on your symptoms and stage of disease at diagnosis.

The best test to diagnose MZL is a biopsy. A biopsy is a procedure that involves taking a sample of affected tissue and looking at it under the microscope. The type of biopsy you have will depend on your disease subtype.

A lymph node biopsy involves removing part or all of a suspicious lymph node. You will have this type of biopsy if you have nodal MZL.

An endoscopic biopsy might be required if the lymphoma is suspected in areas like the stomach or lungs. A thin, flexible camera (endoscope) is passed through your mouth or nose to reach the area. A sample of the affected area is taken for testing.

A surgical biopsy might be required if the tissue is hard to reach, or if the needle doesn’t provide enough of a sample. This is often a day procedure, where a piece of the tissue or lump is removed for testing.

Other tests you may have include:

• medical history and physical exam
• blood tests including full blood count (FBC), kidney and liver function, electrolytes and sometimes thyroid function tests.
• genetic tests
• bone marrow biopsy
• lumbar puncture

These tests help your treatment team work out your stage and prognosis.

Imaging tests help stage your MZL and find where it has spread in the body.

• computed tomography (CT) scan
• positron emission tomography (PET) scan
• magnetic resonance imaging (MRI)
• endoscopy, a procedure where a small camera looks at parts of your stomach, bowel, or lungs.
• eye check with a specialist doctor.

When the disease is slow growing (indolent) and not causing symptoms, it may be safe to be monitored without needing treatment right away. This approach is called active monitoring.

Active monitoring may be recommended for:

• stage 2 MZL that can’t be treated with radiation
• advanced stage MZL (stage 3 or 4) without symptoms.

Active monitoring involves regular checkups to monitor your health and find any changes early.

The timing of checkups depends on:

• how fast the disease appears to be progressing
• how comfortable you are with delaying treatment
• your ability to notice and report new or worsening symptoms.

Waiting to start treatment can be stressful for many people. If this approach causes anxiety or emotional distress, it’s important to seek help. Talk with your treatment team or seek support from a Leukaemia Foundation Healthcare Professional.

Radiation therapy, also called radiotherapy, is a type of treatment that:

• uses high energy x-rays to kill cancer cells
• is a local therapy because it only destroys cancer cells in the area it treats.

In early-stage MZL, radiation may offer a chance to cure the disease if it is limited to one or a few areas. In advanced stage MZL radiation therapy may be used to relieve symptoms and improve quality of life, even if it doesn’t cure the disease.

If you have gastric MALT lymphoma caused by Helicobacter pylori (H. pylori) infection, you might be prescribed antibiotics as a treatment. This can sometimes cure the MZL.

You will also be given other supportive medications while you are taking antibiotics. This could include a proton pump inhibitor (PPI) like esomeprazole to help protect the stomach and manage any reflux.

People with hepatitis C virus (HCV)-associated MZL can access treatments through the Pharmaceutical Benefits Scheme (PBS) that target both the hepatitis C infection and the lymphoma at the same time. These treatments can be very effective, with a strong chance that the lymphoma will shrink or even disappear as hepatitis C is treated.

Immunotherapy is a type of biological therapy. It uses the immune system to recognise cancer cells and destroy them. Your treatment team will give this to you via a drip into a vein.

Immunotherapy works by:

• stimulating the immune system to find and attack cancer cells
• removing barriers that are stopping the immune system from killing cancer cells.

Rituximab is a monoclonal antibody used to treat some types of MZL. It works by targeting CD20 positive B cells so the immune system can destroy that cell and help control cancer. Rituximab is normally given as an intravenous infusion in a day hospital.

Targeted therapy is a cancer treatment that finds and blocks parts of cancer cells that help them grow and spread.

Bruton’s tyrosine kinase inhibitors (BTKis) are a type of targeted therapy that blocks a protein inside B cells that helps them survive and grow. This can slow the growth of or destroy cancerous B cells.

Zanubrutinib is a BTKi that can be used to treat relapsed MZL. This treatment is not covered by the Pharmaceutical Benefits Scheme (PBS) and may result in out-of-pocket costs.

Chemotherapy (chemo) is used to treat MZL if multiple areas of the body are affected. Chemo destroys cancer cells and stops them from growing. It also damages healthy cells and causes side effects. There is information on chemotherapy side effects and how to manage them on our chemotherapy webpage.

The type of chemo recommended will depend on your subtype of MZL, the treatment goal, your age, and medical history. Your haematologist will provide you with information on your plan, including when and how often you will have it. 

There are several treatment plans for MZL. They can be found on the Cancer Institute NSW website.

Clinical trials may be available for some types of MZL. They often compare new or combination therapies with current treatments, including side effects.

Many are randomised, meaning some patients receive the new treatment, and others receive standard care. Trials help improve future treatments and may offer access to costly new therapies not on the Pharmaceutical Benefits Scheme (PBS) in Australia.

If you are considering a clinical trial, your doctor will explain:

• the possible risks and benefits
• how it compares to standard treatment
• what is involved.

You will need to give informed consent before taking part.