Marginal zone lymphoma
Marginal zone lymphoma is a type of non-Hodgkin lymphoma (NHL).
NHLs are a group of blood cancers that affect white blood cells called 
. They are cancers of the . There are many different sub-types of NHL.
There are different types of marginal zone lymphoma (MZL). These include:
- extranodal marginal zone lymphomas (EMZL), these are also called MALT lymphomas
- nodal marginal zone lymphomas (nodal MZL)
- splenic marginal zone lymphomas (splenic MZL)
This page is about nodal MZL and splenic MZL. We have a separate page about MALT lymphomas.
What is marginal zone lymphoma?
Marginal zone lymphoma is a type of B cell non-Hodgkin lymphoma (NHL).
Lymphoma develops when white blood cells called lymphocytes grow out of control. They move around your body in your blood and your lymphatic system .
The lymphatic system is an important part of our immune system. It has tubes that branch through all parts of the body.
These tubes are called lymph vessels or lymphatic vessels and they carry a straw coloured liquid called lymph. This liquid circulates around the body tissues. It contains a high number of white blood cells (lymphocytes) which fight infection.
There are different types of lymphocytes. These include T lymphocytes (T cells) and B lymphocytes (B cells).
MZL develops when the body makes abnormal B lymphocytes. So it is a type of B cell lymphoma. It is usually slow growing and is a .
MZL develops in an area at the edge of lymphoid tissue called the marginal zone. There are different types. MALT lymphomas are the most common type of MZL.
How common is it?
Each year around 2, 600 people are diagnosed with MZL in the UK.
Types of marginal zone lymphoma
There are different types of MZL.
Mucosa associated lymphoid tissue (MALT) lymphoma
This is the most common type of MZL. Doctors also call it extranodal marginal zone lymphoma (EMZL). Extranodal means it is outside the lymph nodes.
More than 60 out of 100 (more than 60%) of marginal zone lymphomas are MALT lymphomas.
MALT lymphoma does not start in the lymph nodes. It starts in the mucosa. This is a soft, moist tissue layer that protects and covers organs in different parts of your body.
The most common place for it to develop is the stomach. This is called gastric MALT lymphoma.
Less often it can develop in the small bowel, salivary gland, thyroid gland, tear (lacrimal) glands or lungs.
Nodal marginal zone lymphoma (nodal MZL)
This type of MZL starts within the . It is sometimes called monocytoid B cell lymphoma. Less than 10 in 100 MZLs (less than 10%) are this type.
Splenic marginal zone lymphoma (splenic MZL)
This type of MZL starts in the but can also be found in the bloodstream. Around 20 out of 100 MZLs (around 20%) are splenic MZL.
The spleen is part of your . It is an organ on the upper left side of your tummy (abdomen). It lies just under your rib cage.
Primary cutaneous marginal zone lymphoma
This type of MZL starts in the skin. We have a separate page of information about skin lymphomas.
Symptoms of marginal zone lymphoma
Some people with MZL have these symptoms. But some people don't have any symptoms.
General symptoms (B symptoms)
Nodal MZL and splenic MZL can both cause general symptoms such as:
- heavy sweating at night
- high temperatures that come and go with no obvious cause
- losing a lot of weight (more than one tenth of your weight)
Doctors call this group of symptoms B symptoms. It is important to tell your doctor about any symptoms like this.
Other symptoms depend on the type of MZL.
Symptoms of nodal MZL
The most common symptom is one or more painless swellings in the:
- neck
- armpit
- groin
These swellings are enlarged lymph nodes.
Feeling very tired (fatigue) can also be a symptom.
Symptoms of splenic MZL
Splenic MZL doesn't usually cause swollen lymph nodes.
Your spleen may become swollen. Doctors call this splenomegaly. It can cause discomfort or pain in the upper part of your tummy. It can also reduce your appetite and lead to weight loss.
Splenic MZL can affect your bone marrow. This can cause symptoms such as:
- low
red blood cells (anaemia), making you feel tired or breathless - low
platelets , causing bruising or bleeding problems
See your GP if you have any of these symptoms. They may not be related to lymphoma, but it's important to get checked out.
Tests to diagnose marginal zone lymphoma
The main test to diagnose nodal MZL is a . A doctor removes part or all of the swollen lymph node. They send it to a laboratory for a specialist to look at it under a microscope.
Doctors can usually diagnose splenic MZL using a sample of blood or . Rarely, they might need to remove your spleen to diagnose splenic MZL. This operation is called a splenectomy.
For all types of MZL you have blood tests.
If your doctor diagnoses lymphoma, you might have further tests. These might include:
- a
CT scan - a
bone marrow test - this is to check if you have lymphoma cells in your bone marrow, you might not need this test - a
PET-CT scan - these are less common for diagnosing MZL compared to other types of lymphoma
Stages and grades of marginal zone lymphoma
Grade
Grade describes how the cells look under a microscope. Your grade tells the doctor how quickly the lymphoma is likely to grow and spread. Doctors put NHL into 2 grades:
- low grade (slow growing)
- high grade (fast growing)
MZL is usually slow growing and called a low grade lymphoma. Doctors also call this indolent lymphoma.
Transformation
Low grade lymphomas can sometimes change into a faster growing (high grade) lymphoma. Doctors call this transformation.
Treatment when a low grade lymphoma transforms is usually the same treatment as for high grade lymphoma. This is usually a combination of anti cancer drugs.
Stage
You have tests to find out where the lymphoma is in your body. This is your stage. Doctors use your stage to plan your treatment.
There are different systems for staging NHL. Your doctor can tell you more about your stage, and what it means.
Generally, for NHL there are 4 main stages. Or your doctors might talk about:
- early or limited stage lymphoma
- advanced stage lymphoma
Treatment for marginal zone lymphoma
Treatment for MALT lymphoma usually works well. Your treatment depends on:
- what type of MZL you have
- where your lymphoma is (the stage)
- your symptoms
- your general health
The main treatments
The main treatments are:
radiotherapy immunotherapy on its ownchemotherapy combined with a targeted immunotherapy drug (chemoimmunotherapy)- surgery to remove your spleen, if you have splenic MZL
targeted cancer drugs
Splenic MZL and nodal MZL are more common in people with a viral liver infection called hepatitis C. If you have hepatitis C virus, your doctor will give you some anti viral treatment.
Most cases of gastric MALT lymphoma are linked to the bacteria Helicobacter pylori (H. pylori). You take antibiotics and a treatment to reduce the amount of acid made by the stomach. This works well for many people with gastric MALT lymphoma. You often don't need any other treatment.
Active monitoring (watch and wait)
You might not have treatment straight away. You have regular check ups instead. This is called active monitoring, or watch and wait.
How often you have check ups depends on your situation. You might have appointments every few months at first. These might become less often if you are well and nothing changes. At your appointment:
- the doctor exams you
- you have blood tests
You might start treatment if you have symptoms such as:
- low blood cell levels
- a very large spleen, if you have splenic MZL
- lymph nodes that are increasing in size if you have nodal MZL
- B symptoms
Treatment for nodal marginal zone lymphoma (MZL)
Radiotherapy
Radiotherapy uses high energy waves similar to x-rays to kill cancer cells.
You might have radiotherapy to treat early stage nodal MZL. Early stage MZL is when you have lymphoma in only 1 or 2 groups of lymph nodes in one part of your body. You have radiotherapy to the affected lymph nodes.
Chemoimmunotherapy
Chemoimmunotherapy is when you have a combination of anti cancer drug treatment. This combination includes:
- chemotherapy medicines which kill cancer cells
- immunotherapy medicines called monocloncal antibodies which use our immune system to fight cancer
The most common monoclonal antibody for treating MZL is rituximab.
Your doctor might offer you a combination of drug treatments if you have advanced nodal MZL. This combination is called chemoimmunotherapy.
Common chemoimmunotherapy treatments for nodal MZL include:
- rituximab and bendamustine
- rituximab, cyclophosphamide, doxorubicin, vincristine and prednisolone (R-CHOP)
- rituximab, cyclophosphamide, vincristine and prednisolone (R-CVP)
Prednisolone is a drug.
If nodal MZL comes back
Nodal MZL often comes back at some point. You might not need treatment straight away if you don't have any symptoms.
If you do need treatment, there are lots of options. You might have one of the following:
- a combination of 3 or 4 chemotherapy drugs with rituximab - you might have the same treatment you had before if you were in remission for a long time
- a clinical trial looking at a
targeted cancer drug - high dose chemotherapy and a
stem cell transplant - you must be fit and well to have this treatment - a targeted cancer drug called zanubrutinib - you take this as a tablet
Treatment for splenic marginal zone lymphoma (MZL)
Rituximab
You might have rituximab on its own. Rituximab is a type of immunotherapy called a moncolonal antibody. Immunotherapy uses our immune system to fight cancer.
Chemoimmunotherapy
Your doctor might offer you a combination of drug treatments for advanced splenic MZL that is causing symptoms. You usually have a course of chemotherapy together with a monoclonal antibody drug. This combination is called chemoimmunotherapy.
Common chemoimmunotherapy treatments for splenic MZL include:
- rituximab and bendamustine
- rituximab, cyclophosphamide, doxorubicin, vincristine and prednisolone (R-CHOP)
- rituximab, cyclophosphamide, vincristine and prednisolone (R-CVP)
Prednisolone is a steroid drug.
Surgery to remove your spleen
Sometimes your doctor might suggest you have an operation to remove your spleen. This is quite big surgery and you need to be well enough.
Radiotherapy
Radiotherapy uses high energy waves similar to x-rays to kill cancer cells.
You might have radiotherapy to your spleen if you aren't well enough for surgery or chemoimmunotherapy.
If splenic MZL comes back
Splenic MZL often comes back. You might not need treatment straight away if you don't have any symptoms.
If you do need treatment, there are lots of options. You might have one of the following:
- rituximab on its own
- surgery to remove your spleen (splenectomy)
- a combination of 3 or 4 chemotherapy drugs with rituximab - you might have the same treatment you had before if you were in remission for a long time
- a clinical trial looking at a targeted cancer drug
- high dose chemotherapy and a
stem cell transplant - you must be fit and well to have this treatment - a targeted cancer drug called zanubrutinib - you take this as a tablet
Follow up
You have regular follow up appointments after finishing treatment. These are appointments to check how you are. And to check there are no signs of the lymphoma coming back.
Your doctor will examine you and ask about any symptoms or side effects. You usually have blood tests.
You can ask any questions at these appointments. Contact your health care team between appointments if you have any symptoms or concerns. You don't need to wait for a booked appointment.
Survival (prognosis) for marginal zone lymphoma
MZL develops slowly and treatment often works well. But, as with other low grade NHLs, the lymphoma often comes back (relapses). At relapse you can usually have more treatment to keep it under control.
The best person to talk to you about your outlook or prognosis is your doctor or specialist nurse. Not everyone wants to know. People cope differently with their lymphoma and want different information.
Survival depends on many factors. So no one can tell you exactly how long you will live. Your doctor might be able to give you some guide, based on their knowledge and experience.
Research
Researchers around the world are looking at the treatments for NHL. Your doctor might suggest you take part in a clinical trial.
Coping with marginal zone lymphoma
It can be very difficult coping with a diagnosis of low grade lymphoma. For many, it is a chronic condition that you live with.
You might have periods of time when you are in remission and are well. Then times when your lymphoma has relapsed and you need to start treatment again.
It can help to talk to friends and family. Or join a support group to meet people in a similar situation.
Some people find that a watch and wait approach makes them anxious. It can be particularly worrying in the run up to check up appointments. But other people feel relieved that they don't need treatment just yet. And they feel able to carry on with work or carry on with other plans.
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