Mantle Cell Lymphoma

Mantle cell lymphoma (MCL) is a rare blood cancer that starts in your lymphocytes (a type of white blood cell). It’s a subtype of non-Hodgkin lymphoma.

In most cases, MCL begins as a slow-growing cancer that later grows rapidly. It spreads throughout your lymphatic system. In its advanced stages, the cancer spreads from your lymph nodes to other areas, like your bloodstream, bone marrow and digestive system.

With mantle cell lymphoma, you might have periods of remission followed by periods of recurrence. That means the cancer can go away and come back, often several times. Treatment can’t cure MCL, but it can lengthen the amount of time you’re in remission.

Mantle cell lymphoma is rare, affecting about 1 in 200,000 people. Anyone can develop this type of cancer. People between the ages of 60 and 70 have a slightly higher risk.

Types of mantle cell lymphoma

The two types of mantle cell lymphoma are:

• Classical MCL. This type starts in your lymph nodes but usually spreads to other areas of your body. It’s usually more aggressive (fast-growing). Most MCL cases are the classical type.
• Leukaemic non-nodal MCL. This type usually causes a swollen spleen and lymphoma cells in your blood and bone marrow. It tends to grow more slowly than the classical type.

What are the symptoms of mantle cell lymphoma?

MCL symptoms vary from person to person and can look like ordinary illnesses. They may not always appear to be cancer-related. That’s one reason why mantle cell lymphoma is usually in the later stages at the time of diagnosis.

You might not have any symptoms with MCL. But if you do, they might include:

• Bruising
• Headaches
• Fatigue
• Fever
• Indigestion
• Loss of appetite
• Night sweats
• Swollen lymph nodes
• Weakness
• Unexplained weight loss

What causes mantle cell lymphoma?

Most of the time, mantle cell lymphoma happens when certain genes mutate (or change) and alter how your B cells function. Researchers don’t know what triggers this genetic change, but here’s how it works:

1. Abnormal B cells make too much cyclin D1 (a protein that helps B cells grow).
2. The cyclin D1 overload makes B cells duplicate and multiply uncontrollably.
3. The duplicate cells make tumors.

What are the complications of this condition?

As mantle cell lymphoma progresses, you might develop complications like:

• Gastrointestinal bleeding
• Ruptured spleen
• Tumor lysis syndrome

How is mantle cell lymphoma diagnosed?

Your healthcare provider may use many different tests to diagnose MCL, including:

• Lab tests. These may include a complete blood count (CBC), comprehensive metabolic panel (CMP), LDH test or a uric acid level test.
• Biopsies. Your healthcare provider may also recommend a lymph node biopsy or bone marrow biopsy. These procedures can help confirm the presence of cancer cells. Once a provider takes a tissue sample, they’ll send it to a pathologist for testing.
• Imaging tests. Your provider might take imaging tests to determine how far the cancer has spread. Depending on the location of the cancer, you might need a colonoscopy, CT scan, EGD (esophagogastroduodenoscopy) or PET scan.

Healthcare providers usually suspect mantle cell lymphoma after routine blood work shows a high lymphocyte count. If your lab results are abnormal, a healthcare provider will do an exam and ask you to describe your symptoms. They might gently press around your lymph nodes to see if they’re swollen.

What are the stages of mantle cell lymphoma?

Healthcare providers use a disease-specific staging system called the Mantle Cell Lymphoma International Prognostic Index (MIPI). It helps them predict survival rates. In general, late-stage MCL is more likely to come back than early-stage MCL.

Healthcare providers determine cancer stage by finding out how far the cancer has spread:

• Stage I. Localized to one lymph node or cluster of lymph nodes in the same area.
• Stage II. Cancer is in two or more lymph nodes or lymph node clusters on the same side of your diaphragm.
• Stage III. MCL has spread to both sides of your diaphragm. Or it’s in the lymph nodes in your spleen and above your diaphragm.
• Stage IV. Mantle cell lymphoma has spread to distant areas of your body or parts that aren’t lymph nodes.

How is mantle cell lymphoma treated?

MCL treatment is different for everyone. The option that’s right for you depends on factors like tumor location and growth.

Treatments for mantle cell lymphoma include:

• Chemotherapy
• Immunotherapy
• Radiation therapy
• Targeted therapy
• Stem cell transplant

You might qualify for some treatments but not others.

If you have MCL, you may want to consider a clinical trial. Clinical trials test new ways to treat cancer. The new treatments might 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.

Can mantle cell lymphoma be prevented?

You can’t prevent MCL. If you receive a diagnosis, it doesn’t mean you’ve done something wrong. Researchers are learning more about why genes mutate or change within our bodies to create cancers like MCL.

What is the outlook for mantle cell lymphoma?

Mantle cell lymphoma isn’t a curable lymphoma. But there are treatments that can send it into remission.

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.

The amount of time you experience remission may be shorter with MCL. But newer targeted treatments can increase the remission period. To learn more about your mantle cell lymphoma prognosis, ask your healthcare provider.

Survival rate

The five-year survival rate for mantle cell lymphoma is about 50%. That means around half of the people diagnosed with this disease are still alive five years after diagnosis.

Survival rates are just estimates. They can’t tell you how long you’ll live or how you’ll respond to a particular treatment. To learn what survival rates mean for you, talk to your healthcare provider.

How do I take care of myself?

Taking care of yourself — physically, emotionally and mentally — is essential with mantle cell lymphoma.

Here are some suggestions that might help:

• Acknowledge your emotions. Ignoring negative feelings won’t make them go away. Honoring your emotions is the first step in finding the support you need.
• Get support. Joining a support group is a great way to find others who are going through similar situations. Ask your healthcare provider about local or online resources.
• Manage your stress. Find ways to relax and bring intention into your daily life. Try meditation or other mindfulness exercises.
• Stick to a routine. The cycle of remission and relapse can make you feel out of sorts. Finding a daily routine and sticking to it can help you regain control.
• Talk to a counselor. It’s important to take care of your mental health. A counselor or therapist can help you sort through complicated emotions.

When should I see my healthcare provider?

If you’re receiving treatment for mantle cell lymphoma, you’ll see your healthcare provider regularly. They’ll monitor your progress and help you manage side effects.

If your condition is in remission, you’ll have occasional follow-ups. Ask your provider how frequently to schedule these appointments.