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Mantle Cell Lymphoma

Mantle cell lymphoma (MCL) is a rare and often aggressive subtype of non-Hodgkin lymphoma (NHL). It develops when B lymphocytes on the outer ring of a lymph node — the “mantle zone” — begin to grow and divide quickly and abnormally.

MCL is usually aggressive and spreads quickly, with 70% of patients in stage IV by the time they are diagnosed. This type of cancer responds well to chemotherapy, although relapse is not uncommon. Other treatment options include immunotherapy, stem cell transplant and radiation. The U.S. Food and Drug Administration (FDA) recently approved CAR T-cell therapy for patients with MCL who have not responded to or have relapsed following treatment, and ongoing clinical trials are leading to promising new advancements.

Although rare, some patients will develop a less aggressive version of the disease known as indolent or smoldering MCL, which progresses slowly and may not need to be treated immediately.

 

Mantle Cell Lymphoma Definition

What Is the Definition of Mantle Cell Lymphoma?

Mantle cell lymphoma (MCL) is a rare blood cancer that develops when B lymphocytes on the outer ring of a lymph node begin to grow and divide quickly and abnormally. This outer ring is called the “mantle zone,” and it is where the disease gets its name.

While MCL is usually aggressive and spreads quickly, some patients will develop indolent or smoldering MCL, a form of the disease that progresses slowly and leaves patients with few to no symptoms.

Mantle cell lymphoma is one of the more than 60 subtypes of non-Hodgkin lymphoma (NHL).

Mantle Cell Lymphoma Symptoms and Causes

What Are the Symptoms of Mantle Cell Lymphoma?

Like most lymphomas, the symptoms of MCL are similar to those of other types of cancer, as well as other medical conditions. If you have questions or concerns about your health, please talk to your doctor.

The symptoms of MCL often include one or more of the following:

  • regular fevers
  • lack of appetite
  • weight loss
  • abdominal pain
  • a feeling of “fullness” in your tonsils, liver or spleen

Some patients also experience constant fatigue, which can be a sign of anemia — a condition where your body lacks enough healthy red blood cells — caused by abnormal lymphocytes crowding out healthy cells.

What Causes Mantle Cell Lymphoma?

While the exact cause of MCL is not yet fully known, researchers have discovered a significant link between a genetic translocation — the swapping of genetic material between two chromosomes — and the disease.

Approximately 85% of patients who develop MCL have a translocation between chromosomes 11 and 14 — referred to as t(11.14) — which primarily affects the cyclin D1 gene. This change leads to the overproduction of the protein cyclin D1, which often results in tumor cell growth and division. Even in patients without this specific translocation, most individuals diagnosed with MCL experience increased levels of cyclin D1.

Mantle Cell Lymphoma Risk Factors

What Are the Risk Factors for Mantle Cell Lymphoma?

Mantle Cell Lymphoma Risk Factors

Mantle Cell Lymphoma Risk Factors

There are several known risk factors for developing MCL:

  • Age: As is the case with many types of cancer, risk for developing MCL increases with age. The median onset age is 60-70 years.
  • Gender: Men are approximately three times more likely than women to develop MCL.
  • Race/Ethnicity: MCL is more common in Caucasians.
  • Family history: Like other lymphomas, MCL is not an inherited disease, meaning it is not directly passed down by a parent. However, research has shown that if a close relative has had MCL, you are more likely to develop the disease.

Mantle Cell Lymphoma Diagnosis and Treatment

How Is Mantle Cell Lymphoma Diagnosed?

If your doctor suspects you have mantle cell lymphoma, you will likely go through a series of tests to confirm or rule out a diagnosis.

Here is a list of what you might experience:

  • Physical exam: First, your doctor is likely to perform a physical exam, paying special attention to your lymph nodes. They will also likely ask about your symptoms and family medical history.
  • Blood tests: After a physical exam your doctor will likely order a complete blood count (CBC). This test measures the level of red blood cells, white blood cells and platelets in your blood. With MCL, the abnormal cells often crowd out healthy cells, so low counts of other blood cells can often be an indicator of MCL or another type of lymphoma.
  • Genetic testing: Since research has discovered a strong link between the cyclin D1 gene and MCL, your doctor may recommend testing for the overproduction of the protein cyclin D1 and the t(11.14) translocation.
  • Biopsy: If MCL is suspected, a biopsy is needed to confirm the diagnosis. A surgeon or interventional radiologist may perform a biopsy of an appropriate lymph node after discussion with your hematologist/oncologist. Your hematologist/oncologist may also perform a bone marrow biopsy. These samples are then examined under a microscope for signs of the disease. Your doctor may also send the cells to a lab for evaluation.
    To achieve the most accurate diagnosis, most biopsies of the lymph node are excisional (referring to the removal of the entire lymph node), and will have to be surgical because of this, or are incisional (where only a small portion of the node is removed). Your doctor will discuss the best approach with you prior to your biopsy.

I’ve Been Diagnosed with Mantle Cell Lymphoma, Now What?

Your healthcare provider will help you determine the best immediate course of action. With a rare disease like MCL it is important to consult a hematologist/oncologist who specializes in the disease. With telemedicine, you may even be able to speak to an expert from the comfort of your own home.

Ask your doctor what stage the cancer is and how aggressive it is. This discussion will help guide you in making informed decisions about your treatment. Even though MCL is aggressive in most cases, some patients will be diagnosed with a slower-moving or indolent form of the disease. In these cases, treatment may not be necessary right away, and instead, close observation may be the best course of action.

What Are the Mantle Cell Lymphoma Treatment Options?

There is a range of treatment options for mantle cell lymphoma, but most patients will start with chemoimmunotherapy.

  • Immunotherapy helps your body’s immune system become better equipped to fight off cancer. It uses man-made versions of antibodies that target specific cancer cells known as monoclonal antibodies, like rituximab (Rituxan). Studies have shown that rituximab given with chemotherapy offers better outcomes than chemotherapy alone.
  • Chemotherapy uses strong drugs that kill or damage cancer cells and is generally the first line of defense used against MCL. One commonly used chemo regimen is R-CHOP, a combination of the immunotherapy drug rituximab (R) plus chemotherapy drugs:
    • cyclophosphamide (C)
    • hydroxydaunorubicin (H), also called doxorubicin hydrochloride
    • oncovin (O)
    • prednisone (P)

R-CHOP is usually administered in cycles spaced three weeks apart.

  • Stem cell transplants and radiation therapy are sometimes used in combination with chemoimmunotherapy. Radiation damages the cancer cells in a localized area to stop them from spreading. Stem cell transplants help your body make new, healthy bone marrow, free of lymphoma.

In addition, CAR T-cell therapy was recently approved by the FDA for patients with MCL who have not responded to or have relapsed following treatment. Ongoing clinical trials are leading to promising new advancements.

If your MCL is indolent (slow to grow), you and your treating doctor may decide to take a watch and wait approach. This means delaying treatment until needed, while closely monitoring for disease progression.

Here are a few questions to ask when discussing treatment options with your doctor:

  • What stage of mantle cell lymphoma do I have?
  • Do I need to start treatment right away?
  • Which treatment option(s) do you recommend, and why?
  • How long will treatment last?
  • What are the risks or side effects?
  • How will we know the treatment is working?

Mantle Cell Lymphoma Stages and Survival

What Are the Stages of Mantle Cell Lymphoma?

The Ann Arbor Staging System is used to stage MCL. There are four stages, and each is defined by if and how far the disease has spread.

  • Stage I: The cancer is contained to a single lymph node or organ.
  • Stage II: There are two or more affected lymph nodes, but they sit on the same side of the diaphragm.
  • Stage III: There are two or more affected lymph nodes, and at least one of each is located above and below the diaphragm.
  • Stage IV: The cancer is spread throughout the body in multiple organs and lymph nodes.

To learn more about mantle cell lymphoma staging and grading, talk to your doctor. They can answer your questions and help you better understand your stage and prognosis.

What is the Survival Rate of Mantle Cell Lymphoma?

According to the Leukemia & Lymphoma Society, the median overall survival for MCL is between 5 and 7 years. However, research has made remarkable strides in treatment for this type of lymphoma in the last several decades, with a near doubling of overall survival rates.

Although this type of lymphoma is aggressive and commonly returns after periods of remission, most patients respond well to chemotherapy. Researchers continue to look for new and better therapies, and there are a number of promising clinical trials currently underway.

 

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