Mantle cell lymphoma is a rare and aggressive type of non-Hodgkin’s lymphoma.

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

Read on to learn about the causes and symptoms of MCL and how doctors diagnose and treat this type of cancer.

There are two forms of lymphoma: Hodgkin’s and non-Hodgkin’s. MCL is a type of non-Hodgkin’s lymphoma.

More than 80,600 people are diagnosed with non-Hodgkin’s lymphoma each year in the United States. Only about 5% of non-Hodgkin’s lymphomas are MCL.

While some forms of MCL are slow-growing, MCL is usually aggressive, and doctors often don’t diagnose it until it has spread throughout the body. It may cause symptoms based on the location of the spread, such as intestinal blockage if the tumor spreads to the digestive tract.

Causes and risk factors

MCL results from a genetic mutation, but the cause is usually unknown. A family history may increase your risk of developing it.

It occurs more often in males and adults ages 60–70 years.

You may experience certain symptoms depending on where the cancer has spread. Blood cancers can also cause systemic symptoms, like feeling generally unwell.

Signs and symptoms of MCL may include:

  • swollen lymph nodes, such as in the neck, armpits, or groin
  • fever or night sweats
  • fatigue
  • weight loss or loss of appetite
  • nausea or vomiting
  • discomfort or fullness from enlarged tonsils, liver (hepatomegaly), or spleen (splenomegaly)
  • gastrointestinal problems, such as indigestion (heartburn) 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.

Doctors diagnose MCL based on the results of a biopsy, such as a lymph node biopsy.

A doctor typically diagnoses MCL by performing the following tests and procedures:

  • Biopsy: During a biopsy, a doctor takes a small sample of tissue from the tumor. They then send it to a lab where scientists examine it for specific genetic changes. This helps doctors diagnose the type of cancer you have and determine how best to treat it.
  • Body scans: A doctor may recommend imaging tests, such as magnetic resonance imaging (MRI), positron emission computed tomography (PET-CT) scan, chest X-ray, or CT scan. These tests help the doctor see where the cancer is in your body.
  • Additional imaging tests: A doctor may recommend other imaging tests, such as a colonoscopy or esophagogastroduodenoscopy (EGD) if they suspect the cancer has spread to your digestive tract.
  • Blood tests: This can include a complete blood count (CBC) with differential, comprehensive metabolic panel (CMP), and uric acid level test. They typically also test for specific proteins, such as beta2 microglobulin and lactate dehydrogenase (LDH), which can indicate when treatment needs to start.

How do doctors stage mantle cell lymphoma?

Doctors stage MCL like they do other types of non-Hodgkin’s lymphoma.

Stage 1involves 1 lymph node or group of nearby lymph nodes
Stage 2involves 2 or more lymph node groups on the same side of the diaphragm
Stage 3involves lymph nodes on either side of the diaphragm or on 1 side of the diaphragm with spleen involvement
Stage 4involves multiple organs, with or without lymph node involvement

However, non-Hodgkin’s lymphoma does not necessarily start at stage 1 and progress to each of the other stages. The stage indicates the location of the disease.

Treatment will depend on the severity of your condition and how aggressive your lymphoma is.

If your cancer is indolent (slow growing), a doctor might suggest monitoring the cancer (watchful waiting) instead of undergoing immediate treatment. However, most people with MCL have aggressive cancers that require immediate treatment.

Treatment usually involves a combination of medications. This can include:

  • Chemotherapy: Doctors treat MCL with different types of chemotherapy. They may also combine with other treatments for better results.
  • Monoclonal antibodies: Rituximab is a monoclonal antibody that targets and destroys harmful cells. Doctors often use it alongside chemotherapy or other therapies in people with MCL.
  • Immunomodulatory medications: Lenalidomide (Revlimid) is an oral immunomodulatory medication that works by destroying abnormal cells in the bone marrow and helping the bone marrow create normal blood cells. Doctors may also prescribe steroids, which suppress the activity of the immune system.
  • Targeted therapy: Bortezomib (Velcade) is a targeted therapy that works by killing cancer cells. Acalabrutinib (Calquence) is an FDA-approved medication for people with MCL that works by blocking an enzyme that cancer needs to multiply and spread.
  • Stem cell transplant: Doctors sometimes recommend stem cell transplants for people with MCL. 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 MCL.
    • Allogeneic transplants use healthy stem cells from a donor. They carry more risks than autologous transplants but may offer a better chance for a cure.

Treatment side effects

Some people may experience side effects from MCL treatment. Side effects can include:

  • fever
  • chills
  • fatigue
  • numbness in the hands or feet
  • neutropenia (low white blood cell count that increases your risk of infection)
  • nausea and vomiting
  • rash
  • diarrhea
  • shortness of breath
  • hair loss

If you have side effects from treatment, it’s best to discuss them with your healthcare team. They may be able to help you manage or prevent them.

MCL is an aggressive form of cancer that’s difficult to treat. By the time doctors diagnose the cancer, it has often spread to other areas of the body. There is no curative treatment for all people with MCL, though some people may experience complete remission with an allogeneic stem cell transplant.

The median life expectancy is 1.8 to 9.4 years from diagnosis, depending on how quickly it progresses.

It’s important to remember that each person is different, and the survival rates are based on averages of people diagnosed with MCL. As researchers discover newer treatments, the outlook for those with this type of lymphoma is likely to improve.

Complications

People with MCL 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 cancer grows in your arteries and veins.
  • Gastrointestinal problems: In many people, doctors diagnose MCL 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.

Recovery from MCL can depend on individual factors and how advanced your disease is.

Most people go into remission after the 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.

What is the survival rate of mantle cell lymphoma?

According to a 2024 study that divided participants into under and over age 65 years, researchers found that 82% of people with MCL under 65 years were alive 5 years later, compared with 55% of those older than 65 years.

How curable is mantle cell lymphoma?

There is currently no cure for all people with MCL. But treatment can lead to a longer-term remission (reduction or disappearance of disease activity) in some people. Some people may experience complete remission with an allogeneic stem cell transplant.

Is mantle cell lymphoma an aggressive cancer?

MCL is usually aggressive and requires immediate treatment.

What are the signs of mantle cell lymphoma?

Some people may not have signs of MCL until the cancer has spread throughout the body. You may have symptoms that include fatigue, night sweats, and swollen lymph nodes. You may have additional symptoms depending on where the cancer has spread.

MCL is a type of non-Hodgkin’s lymphoma that is typically aggressive. It may not cause symptoms until it has spread throughout the body. Diagnosis requires various blood and imaging tests.

Treatment typically includes a combination of medications, such as chemotherapy and targeted therapies.