Mantle cell lymphoma is a rare lymphoma. Lymphoma is a type of cancer that starts in your white blood cells.

There are two forms of lymphoma: Hodgkin’s and non-Hodgkin’s. Mantle cell is considered a non-Hodgkin’s lymphoma.

This type of cancer is usually aggressive and often isn’t diagnosed until it’s spread throughout your body.

Read on to learn more about how doctors diagnose mantle cell lymphoma and what types of treatments are available.

More than 72,000 people are diagnosed with non-Hodgkin’s lymphoma each year in the United States. Only about 6 percent of non-Hodgkin’s lymphomas are mantle cell lymphoma.

Men in their early 60s have the highest likelihood of developing mantle cell lymphoma. Caucasians are also at a greater risk for this cancer than people of other races.

Signs and symptoms of mantle cell lymphoma may include:

  • swollen lymph nodes
  • fever or night sweats
  • weight loss or loss of appetite
  • nausea or vomiting
  • fatigue
  • discomfort due to an enlarged tonsil, liver, or spleen
  • gastrointestinal problems, such as indigestion or abdominal pain
  • pressure or pain in the lower back

Some people with mantle cell lymphoma won’t have any obvious symptoms until their disease has spread throughout their body.

Your doctor can diagnose mantle cell lymphoma by performing the following tests and procedures:

  • Biopsy. During this procedure, doctors take a small sample of tissue from your tumor and examine it under a microscope.
  • Blood test. A blood draw might be done to check your white blood cell count.
  • Body scans. Imaging tests, such as computed axial tomography (CAT), may be performed, so your doctor can see where the cancer is in your body.

Treatment will depend on the severity of your condition and how far the lymphoma has spread.

Watch and wait

If your cancer grows slowly, your doctor might suggest that you watch the cancer instead of undergoing immediate treatment.

However, most people with mantle cell lymphoma have cancers that are more aggressive and require immediate treatment.

Medication

The following therapies are commonly used for treating mantle cell lymphoma:

  • Chemotherapy. Different types of chemo are used and are often combined with other treatments for better results.
  • Rituximab (Rituxan). Rituximab is a monoclonal antibody that targets and destroys harmful cells. It’s often used alongside chemo or other therapies in people with mantle cell lymphoma.
  • Lenalidomide (Revlimid). This is an oral immunomodulatory medication. Revlimid works by destroying abnormal cells in the bone marrow and helping the bone marrow create normal blood cells.
  • Bortezomib (Velcade). Velcade is a targeted therapy that works by killing cancer cells.
  • Acalabrutinib (Calquence). The FDA approved this new medicine for people with mantle cell lymphoma in October 2017. Acalabrutinib works by blocking an enzyme that cancer needs to multiply and spread.

You might experience side effects of treatment, such as:

  • fever
  • chills
  • numbness in the hands or feet
  • nausea
  • infection
  • rash
  • diarrhea
  • shortness of breath
  • hair loss
  • other issues

Talk to your doctor if your symptoms become severe.

Stem cell transplant

Stem cell transplants are sometimes recommended for people with mantle cell lymphoma. This procedure involves infusing healthy stem cells in your body to replace diseased bone marrow.

There are two types of stem cell transplants:

  • Autologous transplants involve using your own stem cells to treat your disease. These procedures are typically performed to extend remission in people with mantle cell lymphoma.
  • Allogeneic transplants use healthy stem cells from a donor. They’re considered more risky than autologous transplants, but may offer a better chance for a cure.

These procedures may carry many risks. Talk to your doctor about the benefits and dangers of having a stem cell transplant.

People with mantle cell lymphoma are likely to develop complications from their disease. Some of these include:

  • Low blood cell counts. Low white and red blood cell counts may happen when your disease progresses. Additionally, you may have a low number of platelets in your blood.
  • High white blood cell counts. You might develop high white blood cell counts if the cancer grows in your arteries and veins.
  • Gastrointestinal problems. In many people, mantle cell lymphoma is diagnosed when the disease has spread to other areas of the body, such as the gastrointestinal tract. This can cause stomach issues, polyps, or abdominal pain.

Your chances of recovery will depend on the type of mantle cell lymphoma you have and how advanced your disease is.

Most people respond well to initial treatment of chemotherapy with or without a stem cell transplant. However, the cancer usually comes back. If this happens, you can develop treatment resistance, which means the therapies that worked before might not be as effective.

Mantle cell lymphoma is considered an aggressive form of cancer that’s difficult to treat. By the time the cancer is diagnosed, it’s often spread to other areas of the body.

Over the last decades, overall survival rates have doubled, but relapses are still common. Today, the average overall survival time from diagnosis is between 5 and 7 years. The average progression-free period is 20 months.

It’s important to remember that each person is different, and the survival rates are just estimates. As researchers discover newer treatments, the outlook for mantle cell lymphoma is likely to improve.