Nodal marginal zone lymphoma

Nodal marginal zone lymphoma (NMZL) 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 splenic marginal zone lymphoma (SMZL). 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.

NMZL usually develops in lymph nodes. It can also affect the bone marrow and blood.

Sometimes NMZL 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 NMZL. Your healthcare team will suggest treatments based on your needs and work with you to develop a treatment plan.

NMZL 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 NMZL and you may not need any more treatment.

If NMZL develops slowly and doesn't cause symptoms, you may be offered watchful waiting. The healthcare team will carefully monitor the cancer. When symptoms appear or there are signs that the disease is progressing more quickly, they will offer other treatments.

Find out more about watchful waiting.

Radiation therapy uses high-energy rays or particles to destroy cancer cells. It is commonly used to treat NMZL that is in specific areas of the body.

Find out more about radiation therapy for NHL.

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. The most common targeted therapy drug used to treat NMZL is rituximab (Rituxan and biosimilars).

If NMZL 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 NMZL. Chemotherapy may also be used alone or in combination with radiation therapy.

The most common drug combinations used to treat NMZL 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.

A stem cell transplant replaces stem cells. It may be used to treat NMZL that comes back after treatment or stops responding to treatment.

Find out more about stem cell transplant 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.