Mantle cell lymphoma

Mantle cell lymphoma is a type of non-Hodgkin lymphoma (NHL) that starts in B cells. It develops in the outer edge (called the mantle zone) of a lymph node.

Mantle cell lymphoma is most often aggressive (fast-growing). In rare cases, it is indolent (slow-growing).

The Mantle Cell Lymphoma International Prognostic Index (MIPI) was developed to determine the outcome for people with mantle cell lymphoma. Your healthcare team will use the MIPI to help determine if mantle cell lymphoma is likely to respond well to treatment and if it is likely to come back (relapse) after treatment.

The MIPI includes the following prognostic factors:

• 50 years of age or older
• high lactate dehydrogenase (LDH) levels
• white blood cell count is 6.7 × 10 ⁹/ L or greater
• performance status

Each factor is given a certain number of points. The points for each of the 4 factors are added together to assign a total point score. This number is used to assign the lymphoma to one of the following risk groups:

• low risk (0 to 3 points)
• intermediate risk (4 to 5 points)
• high risk (6 to 11 points)

The MIPI helps you and your healthcare team make treatment decisions that are right for you. This index is also used in clinical trials testing new drugs to treat mantle cell lymphoma.

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

If mantle cell lymphoma develops slowly and doesn't cause symptoms, you may be offered watchful waiting. Your healthcare team will use tests to monitor the cancer closely. When mantle cell lymphoma starts to cause symptoms or seems to be progressing, they will offer other treatments.

Find out more about watchful waiting for NHL.

Chemotherapy uses drugs to destroy cancer cells. It is a main treatment for all stages of mantle cell lymphoma. Chemotherapy is often given with the targeted therapy drug rituximab (Rituxan and biosimilars) .

The most common drug combinations used to treat mantle cell lymphoma are:

• BR – bendamustine (Treanda, Benvyon, Esamuze) and rituximab
• R-CHOP – rituximab, cyclophosphamide (Procytox), doxorubicin, vincristine and prednisone
• R-DHAP – rituximab, dexamethasone, cytarabine (Cytosar) and cisplatin

Find out more about chemotherapy 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.

Rituximab is a monoclonal antibody commonly used alone or in combination with chemotherapy to treat mantle cell lymphoma. It may also be used as maintenance therapy.

Other targeted therapy drugs that may be used to treat mantle cell lymphoma that comes back after treatment (relapses) or stops responding to other treatments (called refractory disease) include:

• ibrutinib (Imbruvica)
• acalabrutinib (Calquence)
• zanubrutinib (Brukinsa)
• bortezomib (Velcade)

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

Find out more about targeted therapy for NHL.

A stem cell transplant replaces stem cells. It may be an option for some people in first remission. It may also be used to treat mantle cell lymphoma that comes back after treatment or that stops responding to treatment.

Treatment options for people who can’t have a stem cell transplant or for people whose cancer comes back after a stem cell transplant include targeted therapy, immunotherapy and chemotherapy.

Find out more about stem cell transplant for NHL.

Immunotherapy helps to strengthen or restore the immune system’s ability to fight cancer. It is sometimes used to treat mantle cell lymphoma.

Brexucabtagene autoleucel (Tecartus) is a CAR T-cell therapy drug. CAR T-cell therapy takes millions of T cells from a person with cancer. In the lab, they are changed so they have chimeric antigen receptors (CARs) on their surface. These receptors recognize a specific antigen (protein) found on the type of cancer being treated. The T cells are then given back to the person where they multiply, attack and destroy the cancer cells.

Brexucabtagene autoleucel may be used to treat mantle cell lymphoma that comes back after treatment or stops responding to treatment. It is used if you have already received the targeted therapy drugs acalabrutinib or ibrutinib.

Brexucabtagene autoleucel may not be covered by all provincial and territorial health plans.

Find out more about immunotherapy for NHL.

Radiation therapy uses high-energy rays or particles to destroy cancer cells. It may be used to treat limited-stage (stage 1 or 2) mantle cell lymphoma or in combination with a stem cell transplant.

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.