Mantle Cell Lymphoma

What is mantle cell lymphoma?

Mantle cell lymphoma is a rare blood cancer that starts in white blood cells in your lymph nodes. This type of cancer often grows slowly before starting to grow more rapidly. Mantle cell lymphoma quickly spreads throughout your lymphatic system and to other parts of your body. There’s no cure for mantle cell lymphoma, but there are treatments that put the condition into remission. Remission means you don’t have symptoms and tests show no signs of cancer. Medical researchers are testing new treatments to make remission periods last longer.

How does this condition affect my body?

Mantle cell lymphoma is a subtype of non-Hodgkin lymphoma. It’s called mantle cell lymphoma because it starts in B lymphocytes (B cells) located in mantle zones of the lymph nodes. Mantle cell lymphoma often begins as a slow-growing (indolent) disease that later grows rapidly (aggressive). In advanced forms of this disease, cancer spreads from your lymph nodes to places like your bloodstream, bone marrow and digestive system.

How serious is mantle cell lymphoma?

This is a serious illness because it can spread very quickly to become an advanced form of cancer. Mantle cell lymphoma is not a curable lymphoma. In most cases, treatment can put the condition into remission.

Unfortunately, cancer in remission isn’t the same as cancer that’s been cured. Mantle cell lymphoma can come back (relapse) after being in remission for months or years.

Over time, the cycle of remission and relapse revolves more quickly until treatment can no longer put the disease into remission. With newer targeted treatments, remission periods for mantle cell lymphoma have significantly increased.

Who’s affected by mantle cell lymphoma?

Mantle cell lymphoma typically affects people age 60 to 70. Men and people designated male at birth (DMAB) are three times more likely to develop the condition than women and people designated female at birth (DFAB).

About 1 in 10 people with mantle cell lymphoma have a slow-growing version of the condition. But for most people, mantle cell lymphoma is an aggressive form of cancer that requires immediate treatment.

Is mantle cell lymphoma a common disease?

No, it’s rare, affecting 1 in 200,000 people.

What are the symptoms?

Mantle cell lymphoma has many symptoms. Some symptoms are common physical problems that people may not realize are cancer symptoms. That’s one reason why most people already have widespread mantle cell lymphoma at the time of diagnosis.

Healthcare providers refer to some symptoms as B symptoms because they’re clear signs of B-cell lymphoma. B symptoms include drenching night sweats, fevers that don’t go away with over-the-counter medication and unexplained weight loss. This is losing at least 10% of your weight within six months without diet or exercise. Other symptoms are:

• Nausea and vomiting or diarrhea.
• Swollen lymph nodes in your neck, armpits or groin.
• Heartburn, belly pain or bloating.
• A sense of fullness or discomfort from enlarged tonsils, enlarged liver (hepatomegaly) or enlarged spleen.
• Pressure or pain in your lower back, often going down one or both legs.
• Fatigue.

What causes mantle cell lymphoma?

Most of the time, mantle cell lymphoma happens when certain genes mutate and change how your B lymphocytes function. In mantle cell lymphoma, abnormal B cells make too much cyclin D1, a protein that helps B cells grow. The cyclin D1 overload makes B cells duplicate and multiply uncontrollably. The duplicate cells make tumors that become lymphoma.

Researchers don’t know what triggers this genetic change. Healthcare providers use genetic analysis to diagnose mantle cell lymphoma and develop treatment.

How do healthcare providers diagnose mantle cell lymphoma?

Healthcare providers diagnose this condition by doing a physical examination and asking about symptoms. They’ll typically focus on symptoms like swollen lymph nodes. They’ll also do the following laboratory tests:

• Complete blood count (CBC) with differential. A complete blood count measures the number of red blood cells, white blood cells and platelets in your blood. It also measures the amount of hemoglobin in your red blood cells. Hemoglobin is a protein that carries oxygen.
• Lactate dehydrogenase (LDH) test. This test checks for tissue damage caused by injury or disease.
• Comprehensive metabolic panel (CMP). Among other things, this blood sample test helps evaluate liver and kidney function and electrolytes in your blood that control nerve and muscle function.
• Uric acid level test. Uric acid is a waste product in your bloodstream.
• Bone marrow aspiration and biopsy. In this test, healthcare providers take a sample of your bone marrow to check for cancer cells.
• Lymph node biopsy. Providers may do a lymph node biopsy to establish a diagnosis.
• Computed tomography (CT) scan. This test uses a series of X-rays and a computer to produce a 3D image of soft tissues and bones.
• Positron emission tomography (PET) scan. This test produces images of your organs and tissues at work.
• Esophagogastroduodenoscopy (EGD). If you have gastrointestinal issues, healthcare providers may use this test to examine your upper gastrointestinal tract for signs of mantle cell lymphoma in your upper GI tract.
• Colonoscopy. Healthcare providers may do this test to check for signs of mantle cell lymphoma in your large intestine (colon and rectum)

Healthcare providers also analyze blood and tissue samples to obtain information about the disease’s genetic makeup. Understanding the mutations helps providers stage cancer and develop treatment plans.

How do healthcare providers stage mantle cell lymphoma?

Cancer staging systems lay the foundation for cancer treatment. Healthcare providers stage cancer by collecting information about specific conditions. Then they use specific staging systems to place conditions in certain categories or stages.

To stage mantle cell lymphoma, healthcare providers consider factors like:

• If the lymphoma has spread from the lymph nodes where it started to other lymph nodes.
• If it's spread to organs in your lymphatic system.
• If it's spread to areas outside your lymphatic system.

They also use a disease-specific staging system called the Mantle Cell Prognostic Index (MCPI). Healthcare providers use the index to estimate if mantle cell lymphoma is likely to come back. The index is based on the following factors:

• Age. Mantle cell lymphoma typically affects people between the ages of 60 and 80. People closer to age 60 may have fewer relapses.
• White blood cell count. A high white blood cell count means your immune system is under stress or fighting an infection.
• Lactate dehydrogenase (LDH). This is a measure of cellular damage.
• Ability to carry on your normal daily activities.
• How fast lymphoma cells are dividing.

How do healthcare providers treat mantle cell lymphoma?

Treatment varies based on your specific situation. You may have mantle cell lymphoma without having symptoms. In that case, your provider may recommend watchful waiting instead of starting treatment. Providers typically use chemoimmunotherapy (combined chemotherapy and immunotherapy) as initial or frontline treatment.

Here is information on treatment options:

• Watchful waiting. Healthcare providers monitor your overall health and watch for symptoms before starting treatment.
• Chemotherapy. This treatment uses drugs to destroy cancer. It’s a common initial treatment for mantle cell lymphoma.
• Monoclonal antibody therapy. This targeted therapy uses lab-created antibodies to find and kill specific cancer cells.
• Immunotherapy. This treatment fights cancer by boosting your immune system. CAR T-cell therapy is an example of immunotherapy for mantle cell lymphoma.
• Maintenance therapy. If initial treatment puts mantle cell lymphoma into remission, healthcare providers typically use monoclonal antibody therapy as ongoing therapy to keep the disease from coming back.
• Autologous stem cell transplantation. Healthcare providers may recommend this treatment for people able to go through intensive chemotherapy before stem cell transplantation. This treatment doesn’t cure mantle cell lymphoma.

Healthcare providers and medical researchers are investigating new treatments to increase remission time and the amount of time between remissions and relapses. Ask your provider for information about participating in clinical trials for new treatments.

How can I prevent this?

You can’t prevent this condition. Mantle cell lymphoma is a genetic disorder that happens when genes mutate or change. Researchers don’t know why these genes mutate so there’s nothing you can or should do to prevent this condition.

What is the prognosis for mantle cell lymphoma?

A prognosis is an estimate of what you may expect if you have treatment for a given illness. With an incurable disease like mantle cell lymphoma, a prognosis is an estimate of how long you may live.

In mantle cell lymphoma, healthcare providers base prognoses on factors including:

• The MCPI rank.
• The cancer stage.
• Your response to treatment.
• Your overall health.

If you have this condition, it’s important to remember a prognosis is an estimate based on other people’s experiences with mantle cell lymphoma. Your healthcare provider is your best resource for information about what you can expect.

Is mantle cell lymphoma curable?

No, there’s no cure for mantle cell lymphoma. There are treatments that put the condition into remission.

What is the survival rate of mantle cell lymphoma?

In general, people with mantle cell lymphoma live two to nine years after diagnosis. Survival rates vary depending on whether the condition is indolent (slow growing) or aggressive. Studies show people at high risk for relapse live about two years after diagnosis and people at low risk are alive five years after diagnosis.

What’s it like to live with mantle cell lymphoma?

If you have mantle cell lymphoma, you’re living with a disease that never really goes away. There are treatments that put this condition into remission for months. But mantle cell lymphoma always comes back (relapses). You may go through several cycles of remissions and relapses, and you’ll need new or different treatment when your symptoms come back.

You’re also living with uncertainty. You may spend a lot of time and energy worrying and wondering when a relapse will happen. You may worry about new treatment side effects and if the new treatment will send your condition back into remission. It’s natural to have these feelings. But if worrying about what’s next keeps you from enjoying life, ask your healthcare provider for help. Your provider knows you and your medical history. They’re your best resource for information about when your condition may come back. They’ll also explain what changes in your body may signal a relapse or simply be an everyday ache or pain. Here are other suggestions that may be helpful:

• Acknowledge your emotions. You may think hiding or ignoring negative feelings will make them go away. They won’t. Instead, feelings like fear, anxiety or anger may fester and grow. If you can find ways to accept negative feelings, you may be able to start sharing your feelings.
• Share your emotions. You may avoid talking to family and friends because you don’t want to burden them with negative feelings like fear and anxiety. That’s understandable. If you’re struggling, ask your healthcare provider for help. Sharing your worries with them may help you put your worries into perspective.
• Manage your stress. Anxiety boosts stress. You may find regular exercise or hobbies bring down your stress levels.
• Make healthy choices. The cycle of remission and relapse may make you feel like you’ve lost control of your health. Everyday decisions like creating healthy food plans or developing an exercise routine may help you regain a sense of control.

When should I see my healthcare provider?

If you’re receiving treatment, you’ll see your healthcare provider on a regular basis. They’ll help you manage treatment side effects, evaluate whether treatment is effective and monitor your overall health. If you’ve finished treatment and your condition is in remission, you’ll see your healthcare provider for maintenance therapy and follow-up appointments so your provider can check on your overall health and any signs your condition has come back.

What questions should I ask my healthcare provider?

Mantle cell lymphoma is a rare illness. Until you learned you had this condition, you may not have known it existed. You may have many questions for your healthcare provider. Here are a few for your consideration:

• What is this condition?
• What caused it?
• What treatments do you recommend?
• What can I expect?

A note from Cleveland Clinic

Healthcare providers can’t cure mantle cell lymphoma. They can help you to live with mantle cell lymphoma. They can provide treatments that eliminate cancer cells and cancer symptoms and put the condition into remission. Mantle cell lymphoma typically comes back after remission. When that happens, healthcare providers use different treatments to put the condition back into remission. Medical researchers are evaluating treatments to increase the amount of time between remissions and relapses.

If you have mantle cell lymphoma, you may be interested in clinical trials testing new ways to treat the condition. The new treatments may not cure mantle cell lymphoma, but they may give you more symptom-free time to enjoy life. Ask your healthcare provider if you could be a candidate for a clinical trial. They’ll be glad to help you.