Mantle Cell Lymphoma

Mantle cell lymphoma (MCL) is a rare, aggressive form of non-Hodgkin lymphoma (NHL). Non-Hodgkin lymphoma is the name of a group of blood cancers that develop in the white blood cells (lymphocytes). MCL makes up about 5% of all non-Hodgkin lymphoma cases in the United States. 1

 

Understand MCL

MCL is a subtype of non-Hodgkin lymphoma known as B-cell lymphoma. B-cells are white blood cells found in your lymphatic system. The lymphatic system helps your body fight infections and remove waste. MCL usually affects the lymph nodes, but can also be found in blood, bone marrow, and other tissues throughout the body. 2

MCL occurs when there is an abnormal change found in the cells on the outer layer of the lymph nodes. This outer layer is called the “mantle zone.”

MCL can be treated over time to shrink the cancer and help contain it. For MCL, it is normal to have alternating periods of disease recurrence (MCL comes back) or remission (MCL is controlled or not detected). MCL requires lifelong symptom management and attention. Until a cure is found, MCL is managed like a chronic illness.

In cases where MCL either comes back or does not improve with treatment, some people may get treatments that can result in potential cure or long-term remission, such as a stem cell transplant or CAR T cell therapy.

• 1

  https://www.ncbi.nlm.nih.gov/books/NBK536985/

• 2

  https://www.mayoclinic.org/diseases-conditions/mantle-cell-lymphoma/symptoms-causes/syc-20584872

Types of MCL

There are 2 main subtypes of MCL. The subtype affects the symptoms you may have, how the disease should be treated, and how well treatment might work. The subtypes are:

1. Classical MCL – This is the most common form of MCL. It is also known as nodal MCL. This type of cancer is typically fast-growing and can be aggressive. Classical MCL starts in your lymph nodes and can impact other organs. Aggressive forms of classical MCL may spread to your GI (gastrointestinal) system, bones, liver, and spleen.
2. Leukemic non-nodal MCL – This type of MCL spreads through your blood, spleen, and bones. Leukemic, non-nodal MCL typically grows more slowly and does not involve your lymph nodes or GI system. 

There are other rare forms of MCL that may occur. Talk with your care team to learn more about your specific type.
 

Risk Factors

The cause of MCL is not fully understood. Most  of the time, MCL occurs when there is a change in your genes that affects how B-cells work. Abnormal B-cells make too much of a protein called cyclin D1. This excess protein causes your B-cells to multiply uncontrollably. This genetic change occurs over time, and scientists don’t know exactly what causes it. Lifestyle factors may play a role. The genetic variation that causes MCL cannot be passed on to your children.  

A risk factor is a behavior or factor that increases your risk of getting a disease. You may have cancer risk factors that you can change, like smoking or environmental exposure. There are some risk factors you cannot change, such as your age and sex. 

There are several factors that may increase your risk of developing MCL:

• Sex at birth – MCL occurs more often in people who are assigned male at birth.
• Age – Although MCL can be diagnosed at any age, people are often diagnosed when they are 60 or older.
• Race – White people are more likely to develop MCL than people of other races.
• Environmental exposures – Long-term exposure to certain chemicals, pesticides, or radiation may be linked to some MCL diagnoses. 

Just because someone has one or a few risk factors does not mean that they will get MCL. Your care team is available to answer your questions and help you understand your risk.
 

Signs & Symptoms

MCL may not cause symptoms at first, or symptoms may look like other common illnesses. The signs and symptoms of MCL may also be similar to those of other common blood cancers. People with MCL may experience a wide range of symptoms. MCL symptoms will vary from person to person. 

Common MCL signs and symptoms include:  

• Bruising – You may have a low platelet count (thrombocytopenia). This can cause bruising, tiny red dots on your skin (petechiae), or bleeding from any part of your body.
• Headaches – You may experience pain or discomfort in the head, face, or neck area.
• Fatigue/weakness – You may feel physically, emotionally, or mentally exhausted.
• Fever – You may have a high body temperature over several days or weeks.
• Unexplained weight loss/appetite changes – You may not feel hungry or may lose weight without dieting or attempting weight loss.
• Night sweats – You may wake up drenched in sweat.
• Swollen lymph nodes – You may find painless lumps or swollen lymph nodes in the neck, underarm, or groin area.

To diagnose MCL, your doctor will order several tests, including biopsies, blood tests, and imaging tests. These tests will confirm the exact type and stage of MCL. This will help you and your doctor decide the best treatment options for you.
 

Diagnosis

Blood tests  

• The complete blood count (CBC) counts the number and types of blood cells in a blood sample.
• The comprehensive metabolic panel (CMP) counts 14 substances in your blood; including substances like proteins, electrolytes, and enzymes.
• The lactate dehydrogenase (LDH) test measures the level of LDH in your blood. High LDH levels may signal a more advanced stage of the disease.
• The uric acid level test measures the level of uric acid in your blood.  
   

Imaging tests

• A colonoscopy is a procedure where a doctor looks inside your colon and rectum to check for signs of cancer. Even though colonoscopies are usually done to look for colorectal cancer, they can also find signs of MCL in your GI tract. Learn more about preparing for your colonoscopy and other cancer screening guidelines.
• A computed tomography (CT) scan is a detailed x-ray image of the inside of your body. During your scan, you will lie flat on a table while a series of images are taken. This scan is quick and painless.
• An upper endoscopy is a nonsurgical procedure where a doctor uses an endoscope to view your upper gastrointestinal (GI) tract. The endoscope is a thin tube with a camera and surgical tool attached to it.
• A positron emission tomography (PET) scan is a test that shows a detailed image of the inside of your body. Before the exam, you will receive an injection of  a sugar-like substance. Your doctor can see this substance on the scan and can use it to visualize your disease progression.

The type of imaging test you may need will depend on the location of your cancer. These tests help your doctor determine how far your cancer has spread. Ask your doctor which imaging test they recommend for you.   

Biopsies

• A lymph node biopsy is when your doctor takes a sample of your lymph node to be examined. Your doctor may take one or several of your lymph nodes  during this procedure.
• A bone marrow biopsy inserts a needle into your hipbone to get a sample of liquid or tissue from the bone marrow.  Bone marrow is where blood cells are made.
  • The removal of liquid is an aspiration.
  • The removal of tissue is a biopsy.

Your sample is sent to the lab to be tested by the pathologist. A pathologist is a doctor who looks at tissue or blood under a microscope to find any signs of disease. Your care team will use your biopsy to confirm your MCL diagnosis. A biopsy can also help determine the spread of your cancer. Speak with your care team to understand which biopsy type they recommend for you.

There are a number of treatments that work to manage MCL. Until a cure is found, MCL is managed like a chronic illness. Most people will need treatment on and off throughout their life. Your doctor will look at your age, overall health, and MCL type to work with you to make a treatment plan.
 

Watch and Wait

Slow-growing MCL may be monitored using a “watch and wait” approach. This involves watching for any signs that your disease may be progressing without any use of treatment. It may also be referred to as active surveillance. Some people can live for years without treatment. For this approach, you are still regularly seen by your care team and complete tests for any signs of progression.

Effective communication with your care team helps them understand how treatment is affecting you, both physically and mentally. Your care team will share the best ways to manage side effects from treatment. For people with rare cancers, like MCL, open communication is especially important. Your care team may need to connect you with specialists for MCL and other rare blood cancers. Communication is key when making informed decisions about your cancer care.

Managing Physical Side Effects

Every person's experience with cancer treatment is different. Side effects will vary depending on the type of treatment you receive. There are physical side effects that are common with certain treatment types. It is helpful to know the side effects of different treatment options when making your treatment plan with your care team.

MCL treatment  side effects are commonly caused by low blood cell counts. Common side effects of MCL treatment include:

• Fatigue/weakness
• Nausea & vomiting
• Weight & appetite changes
• Risk of anemia, infection, & bleeding
• Hair loss
• Changes in the mouth & esophagus (tube below the throat that carries food and drink to the stomach)
• Constipation & diarrhea
• Fever/chills

Treatment for MCL can affect your body in many ways. Different treatment options may come with different possible side effects. There are options available to support your side effect management. 

Consider this list of tips to help manage side effects of MCL treatment:

• Wash hands frequently and thoroughly with soap and water
• Consider asking your doctor if your treatment or dose can be adjusted if the side effects become too much
• Eat 5–6 small meals throughout the day, instead of 3 big meals
• Lean on plain, soft food to help manage eating concerns
• Avoid large crowds where there is a high risk of infection (and consider wearing a mask when in public)
• Learn about supportive care options available for you  

Managing side effects is an important part of your care, and your care team can help. Your doctor may refer you to a palliative and supportive care specialist. 

MCL is a chronic condition that requires long-term disease management. There may be periods of remission (when the cancer goes away) to periods of relapse (when the cancer returns). Managing the stress and anxiety of life with MCL can be difficult. It’s hard when you feel unsure of what the future holds. This can make coping with your diagnosis especially unique and challenging. 

Use these tips to help you cope with MCL: 

1. Lean on a strong support network. Your loved ones, friends, and members of your care team are here for you during this difficult time. It is important to be open and honest with them about how you are feeling. Support groups are a great way to manage your emotions and social interactions.
2. Stay flexible and understand that change is okay. Your disease status, treatment plan, side effects, and mental health may change from day to day. Learn how to adjust to these changes.
3. Continue psychosocial support throughout your survivorship period. Extended periods of stress and anxiety are commonly associated with MCL. It is important to talk with licensed mental health professionals during and after MCL treatment. A licensed professional is trained to help you manage any fears of recurrence or relapse.
4. Find financial assistance resources to support you. Lifelong disease management can be expensive and add up quickly. Some treatment options and supportive care options are more expensive than others. Reach out to your care team to learn about how they can help you manage financial concerns.
5. Learn about free support services offered by patient advocacy organizations. Many organizations offer free educational resources, programs, helplines, and more. Explore our free programs and services at the Cancer Support Community. Or ask your care team if they can connect you with local or virtual support services.