Patient education: Splenic marginal zone lymphoma (The Basics)

What is splenic marginal zone lymphoma? — 

Splenic marginal zone lymphoma, or "SMZL," is an uncommon type of lymphoma.

Lymphoma is a cancer of lymphocytes, which are infection-fighting cells of the body's lymphatic system. The lymphatic system is made up of organs all over the body that make and store cells that fight infection (figure 1). "Splenic" means involving the spleen, which is an organ in the upper left part of the belly (figure 2).

When a person has SMZL, their spleen is usually larger than normal. They often also have abnormal numbers of certain types of blood cells.

SMZL is usually diagnosed in older adults. It rarely happens in people younger than 50 years old.

SMZL is more common in people with certain health conditions. These include "autoimmune disorders" (such as lupus), asthma, or chronic hepatitis C.

What are the symptoms of SMZL? — 

Many people do not have symptoms. They might find out that they have SMZL after a routine physical exam or getting blood tests done for another reason. When symptoms do happen, they can be related to:

●An enlarged spleen – Your doctor might notice this when feeling your belly during a routine physical exam. It can also cause belly pain, pain in the left shoulder, or feeling full after eating a small amount.

●A low red blood cell count – This is often found when a person gets routine blood tests. Symptoms can include feeling tired or weak, shortness of breath, headaches, and irritability.

●A low platelet count – This is often found when a person gets routine blood tests. Platelets are blood cells that help blood to clot. Having too few platelets can cause easy bruising or bleeding.

Unlike other types of lymphoma, it is unusual for people with SMZL to have symptoms like swollen lymph nodes, night sweats, or weight loss.

Is there a test for SMZL? — 

Yes. Your doctor or nurse will do an exam and ask about your symptoms. They will also order tests, such as:

●Blood tests – These include a "complete blood count," or "CBC," and other tests. The doctor will also look at your blood cells under a microscope.

●Bone marrow biopsy – A doctor uses a needle to remove a small sample of bone marrow, which is the spongy tissue in the center of some bones. The sample is then examined under a microscope.

●Tests on a sample of tissue from the spleen – Doctors sometimes use these tests to diagnose SMZL.

●CT scan, PET scan, or other imaging tests – These create pictures of the inside of the body.

Your doctor might also do tests to check for other health problems.

What is lymphoma staging? — 

This is a way in which doctors find out how far lymphoma has spread within the lymphatic system or within the body.

The right treatment for you depends, in part, on the stage of your lymphoma.

How is SMZL treated? — 

Often, it is not treated right away. Your doctor might not treat your SMZL right away if it is not causing any symptoms and your blood counts are normal. But they will watch your cancer closely by doing exams and blood tests until treatment is needed. SMZL usually grows slowly, and you might not need treatment for years.

If you do have symptoms or low blood cell counts, you will probably need treatment. This can relieve symptoms and slow down the disease. Treatment does not cure the cancer, but many people live for 10 years or more with SMZL. Your options for treatment will depend on how severe your symptoms are as well as your other health conditions. They might include:

●A medicine called rituximab – This medicine blocks the immune system. It is given through a thin tube that goes into a vein, called an "IV."

●Surgery to remove the spleen, called "splenectomy"

●Chemotherapy – These are medicines that kill cancer cells or stop them from growing.

If you have hepatitis C infection, you will probably get an "antiviral" medicine. This will treat the infection and might help with your SMZL symptoms, too.

If your SMZL continues to get worse, your doctor might recommend trying a different treatment or combination of treatments.

What else should I do? — 

Follow all of your doctor's instructions about visits and tests. It's also important to talk to your doctor about any side effects or problems you have during treatment.

Getting treated for SMZL involves making many choices, such as what treatment to have and when.

Always let your doctors and nurses know how you feel about a treatment. Any time you are offered a treatment, ask:

●What are the benefits of this treatment? Is it likely to help me live longer? Will it reduce or prevent symptoms?

●What are the risks to this treatment?

●Are there other options besides this treatment?

●What happens if I do not have this treatment?

More on this topic

Patient education: Lymphocytosis (The Basics)
Patient education: Lymphoma (The Basics)
Patient education: Complete blood count (CBC) (The Basics)
Patient education: White blood cell differential (The Basics)
Patient education: Bone marrow aspiration and biopsy (The Basics)
Patient education: Platelet count (The Basics)
Patient education: Splenectomy (The Basics)
Patient education: Hepatitis C (The Basics)

Patient education: Bone marrow aspiration and biopsy (The Basics)
Patient education: Hepatitis C (Beyond the Basics)