What is nodal marginal zone lymphoma?
Lymphoma is a type of cancer. It can happen when growth of a lymphocyte (white blood cell) population goes out of control.
Marginal zone lymphomas develop from B lymphocytes (B cells) that are normally found in the ‘marginal zone’. The marginal zone is at the edge of the area of lymphoid tissue and is where B cells are normally found (lymphoid tissue is part of the immune system, for example the lymph nodes or spleen). Other types of marginal zone lymphomas are MALT lymphoma (extranodal marginal zone lymphoma) and splenic marginal zone lymphoma.
Nodal marginal zone lymphoma (nodal MZL) is a type of low-grade (slow-growing) non-Hodgkin lymphoma (NHL). It is a rare type of lymphoma – fewer than 2 in 100 cases of NHL are nodal MZL.
Who gets nodal marginal zone lymphoma and what causes it?
Nodal MZL can affect people of any age but is most common in people in their 60s.
In most cases, it is not known what causes nodal MZL. This type of lymphoma is more common in people who have been infected with hepatitis C virus. However, the vast majority of people who have had hepatitis C do not go on to develop lymphoma.
Nodal MZL commonly causes swollen lymph nodes (glands), usually in the neck or groin. They might be in a single area or in several areas. The lumps are not usually painful.
There are usually no other symptoms. In some cases, people feel very tired. A few people can also experience generalised symptoms like weight loss, fevers and night sweats. These are known as ‘B symptoms’ and often occur together.
Nodal MZL is diagnosed with a biopsy. A sample of tissue that is affected by lymphoma, such as a swollen lymph node, is removed during a small operation. It is usually done under local anaesthetic. The sample is examined by an expert lymphoma pathologist. The pathologist then does tests on the tissue to find out what type of lymphoma it is.
Nodal MZL can be difficult to diagnose because it can look similar to other types of lymphoma. Sometimes the lymphoma, or a part of it, can transform (change) into a faster-growing type of lymphoma, which makes it even more difficult to diagnose.
Although waiting for the results of your tests can be difficult, your doctor is collecting important information during this time. It is important that your doctor knows exactly what type of lymphoma you have so they can give you the most appropriate treatment.
You have other tests to find out more about your general health. Tests are also needed to find out which parts of your body are affected by lymphoma – this is called ‘staging’. These tests usually include:
- a physical examination
- blood tests to look at your general health, your blood cell counts and to detect infections, such as hepatitis C
- a scan – usually a CT scan.
A PET scan might be done if your specialist thinks it would be helpful in planning your treatment. You might have a bone marrow biopsy to see if the lymphoma is affecting your bone marrow.
Stage describes how much of your body is affected by lymphoma.
- Stage 1 and 2 lymphomas are described as ‘early’ stage.
- Stage 3 and 4 lymphomas are described as ‘advanced’ stage.
Most people have advanced stage lymphoma when they’re diagnosed – the lymphoma is in several places in the body. This is not uncommon as the lymphatic system runs throughout the body. There are good treatments for all stages of nodal MZL.
A letter might be added to your stage:
- ‘A’ means you are asymptomatic (have no ‘B symptoms’)
- ‘B’ means you have any or all of the ‘B symptoms’ (fevers, night sweats, weight loss).
Nodal MZL develops slowly. Treatment is often successful, but the lymphoma frequently relapses (comes back). Most people live with this type of lymphoma for many years and may need treatment from time-to-time.
Your doctor can give you more information about your prognosis (outlook) based on your individual circumstances.
Your treatment depends on whether the lymphoma is causing any problems and on your general health.
If the lymphoma is not causing any symptoms, your doctor might suggest monitoring you and saving treatment until it is needed. This is called active monitoring or ‘watch and wait’. This approach allows you to avoid the side effects of treatment for as long as possible. Delaying treatment has no effect on how well it works when you do need it.
If you test positive for hepatitis C, you may be offered anti-viral treatment. Clearing the infection can also clear the lymphoma. If this is the case, you may not need any more treatment.
If you need treatment, there are several options:
- radiotherapy if your lymphoma is localised (in a single or a few places)
- antibody therapy, for example rituximab.
Chemotherapy and antibody therapy are often used together as‘chemo-immunotherapy’.
The most common chemotherapy for nodal MZL includes:
- chemotherapy with a single drug, eg bendamustine or chlorambucil
- a combination of chemotherapy drugs called CVP: cyclophosphamide, vincristine and the steroid prednisolone.
Your doctor might recommend a different regimen (combination of drugs). For example, CHOP might be considered if you have widespread lymphoma.
If rituximab is given with chemotherapy, an ‘R’ is usually added to the name of the regimen, eg R-CVP.
The aim of treatment is to put you into remission (no evidence of lymphoma) for as long as possible. Your doctor should discuss with you the treatment they recommend.
Treatments affect people differently. Each type of treatment or drug has a different set of possible side effects. Your medical team should give you more information about any side effects associated with your treatment. Ask for more information if you are worried about potential side effects. Your medical team can also give advice and treatment if you experience troublesome side effects during your treatment.
Nodal MZL can transform (change) into a faster-growing or aggressive type of lymphoma. Overall, transformation happens in around 1 in 7 people during the course of their disease. Transformed nodal MZL is treated like a high-grade (fast-growing) non-Hodgkin lymphoma. The most common treatment is the chemo-immunotherapy regimen, R-CHOP.
When you are in remission (no evidence of lymphoma) after your treatment or during a period of active monitoring (‘watch and wait’), you have regular appointments in the clinic. Your doctor makes sure you are recovering well from treatment and that your lymphoma is still in remission.
You do not normally have scans at follow-up appointments unless you develop new symptoms.
It is common for nodal MZL to relapse (come back) after successful treatment. You can usually have more treatment to give another period of remission. You might be given the same treatment or a different type of treatment, depending on your response to your previous treatment and your general health. This approach can often control the lymphoma for many years.
New treatments are often tested first in people with relapsed or refractory lymphoma (lymphoma that didn’t respond to treatment). You may be able to enter a clinical trial of a newer targeted treatment.
A stem cell transplant is rarely considered for nodal MZL. It is usually only considered if your lymphoma relapses soon after chemo-immunotherapy. You must be fit enough to have this intensive form of treatment. A stem cell transplant allows you to have high doses of chemotherapy. It can give a better chance of a long-lasting remission (no evidence of lymphoma) than standard chemotherapy regimens.
Nodal MZL is rare so most clinical trials include people with nodal MZL alongside other types of low-grade lymphoma. There are several targeted treatments in clinical trials for marginal zone lymphoma, including drugs already approved for use in other types of lymphoma, eg:
Your doctor can advise whether there is a clinical trial that is suitable for you. You can also find out more about clinical trials and search for suitable clinical trials at Lymphoma TrialsLink.