About marginal zone lymphoma (MZL)
Marginal zone lymphoma (MZL) is a rare slow-growing type of non-Hodgkin lymphoma (NHL). It starts in the B lymphocytes, which are a type of white blood cell made in the bone marrow.
Who gets MZL
- MZL accounts for 10-15% of all NHL cases.
- It is estimated that there are 500 new cases of MZL diagnosed every year in Australia and New Zealand.
- More commonly diagnosed in women
Causes of marginal zone lymphoma (MZL)
In most cases, there is no specific reason for the genetic mutations that cause MZL. There are some risk factors that may increase your risk of developing MZL. Such as:
- Family history of lymphoma.
- Chronic or previous infections – bacterial or viral. Especially H. pylori, hepatitis C.
- Age – more frequently diagnosed in people 60 years or older.
- Gender – more commonly diagnosed in women.
- Autoimmune diseases such as Hashimoto disease or Sjogren syndrome.
Symptoms of marginal zone lymphoma (MZL)
Symptoms you may experience from your MZL will depend on where in your body the lymphoma is. General symptoms of MZL can include:
- Swollen lymph nodes
- Tired for no reason
- Breathlessness
- Bruising or bleeding more easily
- Drenching night sweats
- Weight-loss without trying
- Itchy skin
- Feeling full quickly
- Pain or discomfort in your upper abdomen
Diagnosis of marginal zone lymphoma (MZL)
MZL is diagnosed with a number of tests. You may have some or all of these tests before an accurate diagnosis can be made. The tests will vary based on the suspected subtype of MZL.
Medical history and physical exam
Your haematologist will check:
- How you’ve been feeling
- Your symptoms and how long you’ve been experiencing them
- If you have had any previous viral or bacterial illnesses like H. pylori or hepatitis
- Your health history and your family’s health history
.
You will have a physical exam to check for any swelling or pain. Tell your haematologist if you’ve noticed any changes in your body.
Blood tests
Blood tests are taken:
- At diagnosis (baseline bloods) and throughout treatment.
- To see if MZL is affecting your blood cells, electrolytes, or other organs such as the liver or kidneys.
- Blood tests that check for previous bacterial or viral infections like H. pylori, hepatitis C. These tests are important as they can help identify your subtype MZL.
Once you start treatment, future blood test results are compared with your baseline blood test results to see how you are responding to treatment.
Biopsy
A biopsy is a surgical procedure to remove some tissue from the body for testing. Depending on the area and how much tissue is taken for the biopsy you may be given local anaesthetic, or it may be performed under a general anaesthetic. The tissue is sent to the laboratory (pathology) for testing. The purpose of any of these biopsies is to confirm your diagnosis, identify your subtype of MZL and develop a treatment plan.
Lymph node biopsy
A lymph node biopsy involves removing part or all of a suspicious lymph node.
Endoscopic biopsy
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.
Surgical biopsy
If the tissue is hard to reach, or if the needle doesn’t give enough of a sample, you might need a small operation (often a day procedure) to remove a piece of the tissue or lump for testing.
Imaging scans
Your haematologist may ask you to have some imaging scans to see if MZL is in other parts of your body. These tests might include:
- Chest x-ray
- Computed tomography (CT) scan
- Magnetic resonance imaging (MRI) scan
- Positron emission tomography (PET) scan
These tests can also be used to see how you are responding to treatment. You can read more about these imaging tests in Non-Hodgkin lymphoma (NHL) – A guide for people with NHL
Types of MZL
The World Health Organization (WHO) has a system that defines MZL subtypes. Your haematologist will use the WHO system to work out your subtype and treatment.
Extranodal MZL
- Also known as mucosa-associated lymphoid tissue lymphoma (MALT)
- The most common type of MZL
- Occurs outside the lymph nodes in places such as; the stomach, small intestine, salivary gland, thyroid, eyes and lungs
- Divided into two categories – gastric, which develops in the stomach and nasogastric, which develops outside the stomach.
- Treatment depends on the stage, location and if there are any underlying infections contributing to the lymphoma.
Nodal MZL
- Happens within the lymph nodes
- Accounts for about 10% of all MZL cases
- Treatment depends on your symptoms and stage of disease
Splenic MZL
- Occurs most often in the spleen and blood
- Can be associated with a previous hepatitis C infection
- Accounts for approximately 20% of all MZL cases
- Treatment depends on your symptoms and stage of disease
Staging
Staging MZL describes how far the lymphoma has spread throughout the body. your stage of MZL will depend on
- How many areas of the body are affected by the lymphoma
- If the lymphoma is above and/or below the diaphragm
- Whether the lymphoma has spread to the bone marrow or other organs.
| Stage 1 – limited stage disease One lymph node area or a single extra nodal site. Either above or below the diaphragm. |
| Stage 2 – limited stage disease Two or more lymph node areas. On the same side of the diaphragm. |
| Stage 3 – advanced stage disease One or more lymph node areas and/or spleen. Both sides of the diaphragm. |
| Stage 4 – advanced stage disease Multiple lymph nodes are affected and it has spread to other parts of the body. (eg bones, lung, liver). |
Prognosis of marginal zone lymphoma (MZL)
A prognosis is an estimate your haematologist will make of the likely course and outcome of your disease. MZL isn’t always curable, but treatment can often achieve a long-term remission. Your haematologist will be able to talk to you about your prognosis, based on your subtype.
Treatment of marginal zone lymphoma (MZL)
Treatments for MZL vary depending on your subtype, stage of disease, and the symptoms you were experiencing at diagnosis.
Watch and wait
Many people with MZL may not need to start treatment straight way. This approach is called ‘watch and wait’. It may apply to you if you have early-stage disease with no symptoms. Watch and wait involves regular check-ups and monitoring by your haematologist and/or general practitioner (GP). Studies show that watch and wait offers no disadvantages compared to early treatment for low-risk MZL. In some cases, MZL can be managed with this approach for years before it progresses.
Living with marginal zone lymphoma (MZL)
How MZL affects your everyday life will depend on many factors. It could be that you are returning or managing work, trying to exercise or managing your nutrition. There are some helpful resources and information to guide you – Living well with blood cancer. The Online Blood Cancer Support Service – Leukaemia Foundation has learn modules on long term side effects, transition to the new normal, cancer related fatigue and more.
Caring for someone with marginal zone lymphoma (MZL)
We have a range of information and resources that may help when you are caring for someone with marginal zone lymphoma (MZL).
