Splenic marginal zone lymphoma

Splenic marginal zone lymphoma (SMZL) is a type of indolent (slow-growing) non-Hodgkin lymphoma (NHL) that starts in B cells. It belongs to a group of NHLs called marginal zone lymphomas, along with extranodal marginal zone lymphoma of mucosa-associated lymphatic tissue (MALT) and nodal marginal zone lymphoma (NMZL). They are called marginal zone lymphomas because they develop from the marginal zone, which is a specific area found in lymph nodes or the spleen.

SMZL usually develops in the spleen. It can also affect the bone marrow, the blood and the lymph nodes in the abdomen.

Sometimes SMZL can change into an aggressive (fast-growing) type of NHL called diffuse large B-cell lymphoma (DLBCL). If it does, it is treated like DLBCL. Find out more about treatments for DLBCL.

The following are treatment options for SMZL. Your healthcare team will suggest treatments based on your needs and work with you to develop a treatment plan.

SMZL is linked with hepatitis C virus (HCV) infection. If you have an HCV infection, you will be treated with antiviral drugs before you start cancer treatment. Treating the HCV first can sometimes reduce the symptoms of SMZL and you may not need any more treatment.

If SMZL is developing slowly and not causing any symptoms, you may be offered watchful waiting. The healthcare team will use tests to carefully monitor the SMZL. When symptoms appear or there are signs that the disease is progressing more quickly, you will be offered other treatments.

Find out more about watchful waiting.

Targeted therapy uses drugs to target specific molecules (such as proteins) on cancer cells or inside them. These molecules help send signals that tell cells to grow or divide. By targeting these molecules, the drugs stop the growth and spread of cancer cells while limiting harm to normal cells. Targeted therapy may also be called molecular targeted therapy.

Targeted therapy can be used alone or in combination with chemotherapy or immunotherapy. The most common targeted therapy drug used to treat SMZL is rituximab (Rituxan and biosimilars).

If SMZL comes back (relapses) after treatment or stops responding to treatment (called refractory disease), these targeted therapy drugs may be used:

• ibrutinib (Imbruvica)
• zanubrutinib (Brukinsa)
• lenalidomide (Revlimid)

These targeted therapy drugs may not be covered by all provincial or territorial health plans.

Find out more about targeted therapy for NHL.

Chemotherapy uses drugs to destroy cancer cells. It is often combined with a targeted therapy drug to treat SMZL. The most common drug combinations used are:

• BR – bendamustine (Treanda, Benvyon, Esamuze) and rituximab
• R-CHOP – rituximab, cyclophosphamide (Procytox), doxorubicin, vincristine and prednisone
• R-CVP – rituximab, cyclophosphamide, vincristine and prednisone
• chlorambucil (Leukeran) and rituximab
• cyclophosphamide and rituximab

Find out more about chemotherapy for NHL.

Surgery to remove the spleen (called a splenectomy) may be offered for SMZL. It is done to improve red blood cell or platelet counts. It is also done to relieve discomfort if the spleen is larger than normal and is putting pressure on other organs.

Find out more about surgery for NHL.

Radiation therapy uses high-energy rays or particles to destroy cancer cells. If you are not well enough to have other treatments, radiation therapy may be used to shrink an enlarged spleen and relieve symptoms.

Find out more about radiation therapy for NHL.

Talk to your doctor about clinical trials open to people with NHL in Canada. Clinical trials look at new ways to prevent, find and treat cancer. Find out more about clinical trials.