Splenic marginal zone lymphoma (SMZL)

SMZL usually does not cause many obvious symptoms to start with.

The spleen may become bigger if lymphoma cells build up there. This can cause discomfort or pain in the upper part of the tummy and a reduced appetite. An enlarged spleen is called splenomegaly.

Some people with SMZL have a low number of blood cells. This may cause the following symptoms:

• tiredness
• shortness of breath
• bruising or bleeding easily
• repeat infections.

A low number of blood cells is usually caused by an enlarged spleen removing too many blood cells. But it can also happen if the lymphoma affects the bone marrow and stops it making normal blood cells properly.

SMZL may also cause the following symptoms:

• drenching night sweats
• unexplained high temperatures
• unexplained weight loss.

These are called B symptoms. Along with staging and grading (below), B symptoms can help your cancer team understand:

• how the lymphoma might develop
• which treatments might help.

A change in how you feel or how your body works can be caused by conditions other than SMZL. But always ask your GP to check any ongoing, new or unexplained symptoms.

We are also here if you need someone to talk to. You can:

• call the Macmillan Support Line free on 0808 808 00 00
• chat to our specialists online.

Many people will have no symptoms to start with. The lymphoma is sometimes discovered during checks for other conditions – for example, during an examination of the tummy or a blood test for something else.

The main tests to diagnose SMZL are:

• blood tests
• bone marrow samples.

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

Waiting for test results can be a difficult time. It may take up to 2 weeks for the results to be ready.

You may find it helpful to talk to family members or a close friend. Your specialist nurse can also provide support.

You can talk to one of our cancer support specialists for free on 0808 808 0000, 7 days a week, 8am to 8pm.

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

After the MDT meeting, you will usually meet with your cancer doctor and specialist nurse. They will explain the different treatments and their side effects. They will also talk to you about things to consider when making treatment decisions.

Your treatment options will depend on:

• the stage of the lymphoma
• your general health
• your preferences.

If tests show that you have hepatitis C (HCV), you will have antiviral drugs to treat this infection. But SMZL usually develops slowly.

If you have no symptoms, you may not need any treatment. Instead, you may have regular tests and appointments to monitor the lymphoma and check for signs that you need to start treatment. This is called watch and wait. It may also be called active surveillance or active monitoring. It is a way of safely delaying treatment until you need it.

If you need to start treatment, it may include one of the following. You may have some treatments as part of a clinical trial. 

Treatment for SMZL is often very effective. It may make all signs of lymphoma disappear for a time. Some people will not need further treatment for many years. But SMZL often comes back. This is called recurrence or relapse.

Your treatment may depend on the treatment you have had and how long your remission lasted.

If you have a relapse with no symptoms, you may have ongoing monitoring with tests and scans. If you need to start treatment, this may involve the following:

• radiotherapy to treat an area of lymphoma that is causing symptoms
• rituximab
• rituximab with chemotherapy
• surgery to remove the spleen
• intensive treatment with a stem cell transplant
• targeted therapy drugs, such as zanubrutinib.

Your cancer team will talk to you about the best options for your situation.

Sometimes SMZL can change from low grade into high grade lymphoma. This is called transformation or transformed SMZL. It is not common. But your cancer team will do tests to check for this when you are diagnosed or have a relapse of SMZL.

High grade lymphoma grows more quickly and is treated differently. Transformed SMZL is treated in the same way as another type of lymphoma called DLBCL.

People often have many different feelings during and after SMZL treatment. If you have finished treatment, you may feel relieved it has ended, but worried about what might happen in the future.

It is important to know where to get support or information if you need it. People often need support even some time after SMZL treatment. But sometimes it is difficult to know who to ask for help.

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

• 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.

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:

• eating problems and your appetite
• fertility
• how you're feeling
• improving your sexual well-being
• managing pain and discomfort
• money and work
• tiredness (fatigue)
• trouble sleeping
• uncertainty
• walking and mobility problems.