What is splenic marginal zone lymphoma?

Splenic marginal zone lymphoma (SMZL) is a rare type of non-Hodgkin lymphoma (NHL).

It belongs to a group of non-Hodgkin lymphomas called marginal zone lymphomas (MZL). There are 3 types:

splenic marginal zone lymphoma (SMZL)
extranodal MZL known as mucosa-associated lymphoid tissue (MALT) lymphoma
nodal marginal zone lymphoma (NMZL).

Your doctor may talk to you about the other marginal zone lymphomas before it is clear which type of MZL you have.

SMZL develops when B-cells become abnormal (cancerous). B-cells are white blood cells that normally help fight infection. They are sometimes called B-lymphocytes.

The abnormal B-cells (lymphoma cells) usually build up in the spleen, bone marrow and blood.

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Symptoms of splenic marginal zone lymphoma

Many people will have no symptoms when SMZL is first diagnosed. It is sometimes discovered during an examination of your tummy (abdomen). It can also be discovered when blood tests are done for some other reason.

The spleen may become bigger if lymphoma cells build up there. This is called splenomegaly. It can cause discomfort or pain in the upper part of the tummy.

Some people with SMZL will have a low number of blood cells in their blood. This is usually because an enlarged spleen may remove too many blood cells. It can also happen if lymphoma cells build up in the bone marrow. These cells take up space, which makes it difficult for the bone marrow to make enough normal blood cells.

If you have too few blood cells, you may:

feel tired
become breathless when moving around
bruise or bleed easily
be more at risk of infection.

Sometimes the lymphoma cells make an abnormal protein called a globulin or a paraprotein. This is found in the bloodstream. This protein can make the blood thicker and more slow-moving than normal. This can cause:

headaches
confusion
nosebleeds
blurred vision.

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Causes and risk factors of splenic marginal zone lymphoma

The causes of splenic marginal zone lymphoma (SMZL) are mostly unknown. It is sometimes linked to long-term infection with the hepatitis C virus (HCV). But it is important to remember that most people with HCV will not develop lymphoma.

SMZL can affect people of all ages, but is most common in people over 70.

Like other cancers, SMZL is not infectious. It cannot be passed on to other people.

We have more information about causes and risk factors of lymphoma.

Diagnosis of splenic marginal zone lymphoma

SMZL can be diagnosed through a combination of:

Rarely, SMZL is diagnosed after an operation to remove the spleen.

Other tests may be done to check for signs of infection with hepatitis C virus.

You can read more about further tests you may have in our information about non-Hodgkin lymphoma.

Waiting for test results can be difficult. You may find it helpful to talk to your family, friends or specialist nurse.

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Booklets and resources

The stages and grades of splenic marginal zone lymphoma

Your test results will help your doctors find out how many areas of your body are affected by lymphoma and where these areas are. This is called staging.

Lymphomas are also grouped as either low-grade or high-grade. Low-grade lymphomas are usually slow growing. High-grade lymphomas usually grow more quickly. Splenic marginal zone lymphoma (SMZL) is a low-grade lymphoma and normally develops slowly. Sometimes it can change (transform) to a high-grade lymphoma.

Knowing the stage and grade of the lymphoma helps your doctor plan the right treatment for you.

We have more information about the stages and grades of non-Hodgkin lymphoma.

Treatment for splenic marginal zone lymphoma

A team of specialists will meet to discuss the best possible treatment for you. They are called a multidisciplinary team (MDT).

Your doctor, cancer specialist or nurse will explain the different treatments and their side effects to you. They will also talk to you about things to consider when making treatment decisions.

Treatment for splenic marginal zone lymphoma (SMZL) depends on the stage of the lymphoma and whether you have symptoms that are causing problems.

SMZL usually develops slowly. Some people may not need treatment for months, or years. During this time, they will have regular check-ups to monitor the lymphoma. This is called watch and wait.

If treatment is needed, the aim is to control the lymphoma for as long as possible. This is called remission.

The most common treatments for SMZL are:

Immunotherapy and chemotherapy
SMZL is often treated with the immunotherapy drug rituximab in combination with chemotherapy. This is called chemoimmunotherapy. Sometimes rituximab is used on its own. Chemoimmunotherapy can often get the lymphoma into remission. Commonly used combinations of chemoimmunotherapy include:

R-CVP

R-CHOP

R-chlorambucil – rituximab and chlorambucil

R-bendamustine – rituximab and bendamustine.
Steroids

Steroids are drugs that are often given with chemotherapy to treat lymphomas. They help make chemotherapy more effective.
Surgery to remove the spleen (splenectomy)

Surgery to remove the spleen may sometimes be useful in this type of lymphoma. If the spleen is not too large, this operation can be done using laparoscopic surgery. This is sometimes called keyhole surgery.
Radiotherapy

Radiotherapy uses high-energy rays to destroy cancer cells, while doing as little harm as possible to normal cells. You may have low doses of radiotherapy to the spleen. This treatment may be used if the enlarged spleen is causing symptoms, but you are not well enough to have surgery or chemotherapy.

You may have some treatments as part of a clinical trial.

If SMZL comes back after treatment it is called a recurrence or relapse. When this happens, you can usually have more treatment to help control it again. We have more information about treating lymphoma that has come back.

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After splenic marginal zone lymphoma treatment

Supporting someone with splenic marginal zone lymphoma

You can:

ask your GP or someone from your cancer team for advice about support in your area
find cancer support services near you
call the Macmillan Support Line for free on 0808 808 00 00.
chat to our cancer information specialists online.
visit our non-Hodgkin lymphoma forum to talk to people who have been affected by lymphoma, share your experience, and ask your questions.

You may also find the HOPE programme helpful. The programme is a free 6 week self-management course designed to help you develop techniques and strategies when living with or after cancer. Topics include goal setting, fatigue management, and wellbeing.

You can sign up for the online HOPE programme. Or to find out more about face to face programmes in your area, email ServiceOpsSupport@macmillan.org.uk

Other organisations who offer information and support

Blood Cancer UK offers support and information to people affected by blood cancers, including lymphoma.

Lymphoma Action provides high quality information and support for people affected by lymphoma. It provides helpline services and a range of peer support including online support meetings, educational events and webinars. Its website includes TrialsLink, a database of lymphoma clinical trials.

More information and advice

We know cancer can affect you physically, emotionally and financially. We have information and advice about different ways cancer might impact you, such as help with: