Mantle cell lymphoma

Mantle cell lymphoma is a type of lymphoma. Lymphoma is cancer that affects the lymphatic system. The lymphatic system is made up of organs, glands, tubelike vessels and clusters of cells called lymph nodes. It's part of the body's germ-fighting immune system. Mantle cell lymphoma typically affects the lymph nodes, but it also can affect the blood, bone marrow and other tissues throughout the body.

There are many types of lymphoma. Lymphoma types are often divided into two categories: Hodgkin lymphoma and non-Hodgkin lymphoma. Mantle cell lymphoma is a type of non-Hodgkin lymphoma.

Mantle cell lymphoma gets its name from the fact that the cancer starts inside an area called the mantle zone of a lymph node. The mantle zone is a ring of cells surrounding the inner part of the lymph node. These cells are germ-fighting white blood cells called B lymphocytes. In mantle cell lymphoma, changes happen that turn the B lymphocytes into cancer cells. The cancer cells build up in the lymph nodes and other parts of the body.

Mantle cell lymphoma is a rare and often aggressive type of cancer. Treatment for mantle cell lymphoma may include radiation therapy, immunotherapy, chemotherapy, targeted therapy, CAR-T cell therapy and bone marrow transplant, also called a bone marrow stem cell transplant. While mantle cell lymphoma often can't be cured, treatments can shrink the cancer size and help contain it. Treatment may increase the time that the cancer stays away, known as remission, and help prolong life.

Types

The types of mantle cell lymphoma include:

• Classic mantle cell lymphoma. Also known as nodal mantle cell lymphoma, this is the most common type of mantle cell lymphoma. It is typically found in the lymph nodes but also can be found in the bone marrow, spleen and digestive system.
• Leukemic nonnodal mantle cell lymphoma. This type of mantle cell lymphoma grows slowly and is mainly found in the blood and spleen. This type may not cause symptoms and may not need treatment right away. Your healthcare team may watch it over time.
• In situ mantle cell neoplasia. This type is a very early form of mantle cell lymphoma. It is often found accidentally during testing for other reasons. In situ mantle cell lymphoma is found in small areas of lymph tissue and doesn't cause symptoms. This type usually never turns into cancer and may only need monitoring.

Classic and leukemic nonnodal mantle cell lymphomas can have variants called blastoid and pleomorphic variants. These are rare but aggressive types of mantle cell lymphoma. They are found in the lymph nodes and bone marrow and can spread to other organs. These variants are often harder to treat and need stronger therapy.

Mantle cell lymphoma may not cause symptoms at first. When they happen, signs and symptoms may include:

• Painless swelling in the neck, armpit or groin.
• Fever.
• Fatigue.
• Decreased appetite.
• Weight loss without trying.
• Nausea.
• Diarrhea.
• Bloating.

If the cancer involves the digestive system, symptoms may include changes in bowel habits and blood in the stool.

When to see a doctor

Make an appointment with a healthcare professional if you have any symptoms that worry you.

It's not clear what causes mantle cell lymphoma. This cancer starts inside the mantle zone of a lymph node. The mantle zone is a ring of cells surrounding the inner part of the lymph node, called the germinal center. These cells are germ-fighting white blood cells called B cells. In mantle cell lymphoma, gene changes cause the cell's DNA to rearrange. This is known as translocation. Certain translocations cause the B cells to turn into cancer cells that multiply rapidly and invade and destroy healthy body tissue.

Mantle cell lymphoma can affect the:

• Lymph nodes.
• Bone marrow.
• Blood.
• Spleen.
• Liver.
• Digestive tract.
• Rarely, the central nervous system.

Factors that can increase the risk of mantle cell lymphoma include:

• Older age. Mantle cell lymphoma can happen at any age. But it's most common in people 60 or older.
• Race. White people are more likely to develop mantle cell lymphoma than are people of other races.

There is no known way to prevent mantle cell lymphoma.

Complications of mantle cell lymphoma can include risk of relapse, complications of advanced cancer and side effects of treatment.

Risk of relapse

Even after successful treatment, mantle cell lymphoma usually comes back. This is called a relapse. Sometimes the relapse happens years later. The risk of relapse continues throughout life, so most people have a lifetime of monitoring after treatment.

Complications of advanced cancer

Complications of advanced mantle cell lymphoma may include swollen lymph nodes that can press on nearby parts of the body and cause issues. This may include pressing on the throat and causing difficulty swallowing or breathing. When swollen, the lymph nodes also may not be able to drain properly. This can cause swelling in the legs. Advanced mantle cell lymphoma also can cause fluid to build up in the chest and belly. This can cause breathing troubles, heart troubles, discomfort and swelling in the belly.

Side effects of treatment

Treatments for mantle cell lymphoma may have side effects. These may include nausea, vomiting, fatigue, fever, rash, diarrhea and more. Harmful side effects from treatments may include:

• Bone marrow suppression. The bone marrow can't make enough blood cells, including white blood cells and platelets. White blood cells help fight infections. When you have fewer white blood cells, you are at a higher risk of infections. Platelets prevent bleeding, so a low amount can increase the risk of bleeding.
• Medicine toxicity. Some medicines used to treat cancer can be harmful to the body. They can cause organ damage and other issues. Whether a medicine is toxic depends on the type and how much you take. There are many different medicine options. Your healthcare team can work with you to find the best ones for you.
• Reactivating viruses. If you have had a viral infection in the past, such as hepatitis B or hepatitis C, some treatments can cause the virus to become active again. This can lead to liver inflammation, liver damage and other complications.
• Tumor lysis syndrome. Tumor lysis syndrome is a serious condition that can happen when cancer cells break down quickly after treatment. When these cells die, they release substances into the bloodstream, such as potassium and uric acid, that can overwhelm the organs. This can lead to problems with the kidneys, heart and other organs. Medicines may help prevent or treat harmful side effects.

Mantle cell lymphoma diagnosis often begins with a physical exam that checks for swollen lymph nodes in the neck, underarms and groin. The exam also checks for a swollen spleen or liver. Other tests and procedures might include blood tests, imaging tests and taking a sample of tissue for lab testing.

Blood tests

Blood tests can sometimes show if lymphoma cells are present. Blood tests also measure levels of lactate dehydrogenase (LDH), which is often higher in people with lymphoma.

Imaging tests

Imaging tests make pictures of the body. They can show the location and extent of a mantle cell lymphoma. Tests might include CT and positron emission tomography (PET) scans.

Lymph node biopsy

Your healthcare professional may suggest a lymph node biopsy to look for cancer cells. A biopsy is a procedure to remove a sample of tissue for testing in a lab. A lymph node biopsy involves removing all or part of a lymph node. In the lab, tests may show whether you have mantle cell lymphoma.

Bone marrow aspiration and biopsy

Bone marrow aspiration and biopsy are procedures to collect cells from the bone marrow for testing. Bone marrow is the soft matter inside bones where blood cells are made. Bone marrow has a solid part and a liquid part.

In a bone marrow aspiration, a needle is used to collect a sample of the fluid. In a bone marrow biopsy, a needle is used to collect a small amount of the solid tissue. Most often, the samples are taken from the hip bone. The samples go to a lab for testing. In mantle cell lymphoma, this procedure is done to help determine the cancer's extent.

Testing lymphoma cells in the lab

Lymphoma cells collected from a lymph node biopsy or bone marrow aspiration and biopsy go to a lab for testing. In the lab, specialized tests look for specific things about the cells. The healthcare team uses the results to decide on the type of lymphoma that you have.

To decide whether the cells are mantle cell lymphoma cells, the healthcare professionals in the lab look for:

• Proteins on the surface of the cancer cells. Mantle cell lymphoma cells have certain proteins on their surface that help identify them. These are known as markers. Common markers found in mantle cell lymphoma are CD19, CD20, CD5 and SOX11.
• Changes in the DNA of the cancer cells. Cancer happens when the DNA inside a cell changes. A cell's DNA holds the instructions that tell a cell what to do. In mantle cell lymphoma, the cells have a gene change that causes their DNA to rearrange. This is known as translocation. This gene change causes the cells to stop dying and multiply rapidly. The genes linked to mantle cell lymphoma include cyclin D1 and TP53 genes.

Mantle cell lymphoma staging

If you're diagnosed with mantle cell lymphoma, the next step is to determine the cancer's extent, called the stage. Imaging tests, blood tests and biopsies may be used to determine the stage of your mantle cell lymphoma. Your healthcare team uses the cancer stage to help create your treatment plan.

The stages of mantle cell lymphoma range from 1 to 4:

• Stage 1 mantle cell lymphoma. A stage 1 mantle cell lymphoma means only one lymph node region is affected.
• Stage 2 mantle cell lymphoma. A stage 2 mantle cell lymphoma involves two or more lymph node regions on the same side of the diaphragm.
• Stage 3 mantle cell lymphoma. A stage 3 mantle cell lymphoma involves lymph node regions above and below the diaphragm.
• Stage 4 mantle cell lymphoma. A stage 4 mantle cell lymphoma involves regions outside of the lymph nodes.

Mantle cell lymphoma prognosis

The cancer prognosis tells you how likely it is that the cancer can be treated. Your healthcare team can get a general sense of your outlook using your mantle cell lymphoma stage. But the stage can't tell your future. Your prognosis may depend on:

• Your age.
• Your overall health.
• The cancer's stage.
• Lymph node and bone marrow biopsy results.
• Certain gene changes.

If you want to know more about your prognosis, talk with your care team.

Mantle cell lymphoma survival rates

Mantle cell lymphoma is often a fast-growing cancer. The survival rate for this cancer is often lower than other types of lymphoma that grow more slowly.

Mantle cell lymphoma treatments may include the "watch and wait" method, radiation therapy, immunotherapy, chemotherapy, targeted therapy, CAR-T cell therapy and bone marrow transplant, also called bone marrow stem cell transplant. Which treatment is best for you depends on the extent of your cancer, called the stage. Your healthcare team also considers how quickly the cancer is growing, your overall health and what you prefer.

Watch and wait

If your mantle cell lymphoma doesn't cause symptoms, you might not need treatment right away. Instead, you may have checkups every few months. The checkups help your healthcare team watch your condition to see if your cancer is growing.

Radiation therapy

Radiation therapy for cancer is a treatment that uses powerful energy beams to kill cancer cells. The energy can come from X-rays, protons or other sources. During radiation therapy, you lie on a table while a machine moves around you. The machine directs radiation to precise points on your body.

Radiation therapy is not a common treatment option because mantle cell lymphoma often affects multiple parts of the body by the time it is diagnosed. However, radiation therapy may be used for people with early-stage mantle cell lymphoma or for people who may not handle other treatments well. Radiation therapy also may be used to relieve symptoms and improve quality of life.

Immunotherapy

Immunotherapy for cancer is a treatment with medicine that helps the body's immune system kill cancer cells. The immune system fights off diseases by attacking germs and other cells that shouldn't be in the body. Cancer cells survive by hiding from the immune system. Immunotherapy helps the immune system cells find and kill the cancer cells.

Immunotherapy may be used as a first line treatment for mantle cell lymphoma. It is often combined with chemotherapy. Immunotherapy also may be combined with other medicines to treat advanced cancer or cancer that has come back after treatment, known as relapsed mantle cell lymphoma.

Immunotherapy also may be used after successful treatment to help keep the cancer away longer. This is called maintenance therapy.

Chemotherapy

Chemotherapy treats cancer with strong medicines. There are many chemotherapy medicines. Most chemotherapy medicines are given through a vein. Some come in pill form.

Chemotherapy may be used as a first line treatment for mantle cell lymphoma. It is often combined with immunotherapy. Chemotherapy may be combined with other medicines in people with advanced cancer or cancer that has come back after treatment, known as relapsed mantle cell lymphoma.

Targeted therapy

Targeted therapy for cancer is a treatment that uses medicines that attack specific chemicals in the cancer cells. By blocking these chemicals, targeted treatments can cause cancer cells to die. For mantle cell lymphoma, targeted therapy medicines often target a protein that the cancer cells make. This protein is called Bruton tyrosine kinase (BTK). Medicines that target this protein are called BTK inhibitors.

For mantle cell lymphoma, targeted therapy may be used as a first line treatment. Targeted therapy also may be used when cancer comes back after treatment, known as relapsed mantle cell lymphoma. Targeted therapy also may be an option when other treatments haven't worked. This is known as refractory mantle cell lymphoma.

CAR-T cell therapy

Chimeric antigen receptor (CAR)-T cell therapy trains the immune system cells to fight mantle cell lymphoma. This treatment begins with removing some white blood cells, including T cells, from the blood.

The cells are sent to a lab. In the lab, the T cells are treated so that they make special receptors. The receptors help the cells recognize a marker on the surface of the lymphoma cells. Then the cells are put back into the body. There the CAR-T cells find and destroy the mantle cell lymphoma cells.

CAR-T cell therapy might be a treatment option for mantle cell lymphoma that has not responded to other treatments, called refractory mantle cell lymphoma. It also may be an option for mantle cell lymphoma that has come back after treatment, called relapsed mantle cell lymphoma.

Bone marrow transplant

A bone marrow transplant, also called a bone marrow stem cell transplant, involves putting healthy bone marrow stem cells into the body. These cells replace cells hurt by chemotherapy and other treatments.

Stem cells can come from your own body, called an autologous transplant. Or they can come from a donor, called an allogeneic transplant. For some mantle cell lymphomas, a bone marrow transplant can slow the progression of the cancer and extend the amount of time the cancer stays away, known as remission.

A bone marrow transplant may be an option for some people who are younger and in good health. Chemotherapy typically is done before the transplant. Maintenance therapy using immunotherapy typically is done after the transplant.

With time, you'll find what helps you cope with the uncertainty and worry of a cancer diagnosis. Until then, you may find that it helps to:

Learn enough about mantle cell lymphoma to make decisions about your care

Ask your healthcare team about your cancer. Ask about your test results, your treatment options and, if you want, your prognosis. As you learn more about mantle cell lymphoma, you may become more confident in making treatment decisions.

Keep friends and family close

Keeping your close relationships strong can help you deal with mantle cell lymphoma. Friends and family can provide the practical support you may need, such as helping take care of your home if you're in the hospital. And they can serve as emotional support when you feel overwhelmed by having cancer.

Find someone to talk with

Find someone who is willing to listen to you talk about your hopes and worries. This may be a friend or family member. The concern and understanding of a counselor, medical social worker, clergy member or cancer support group also may be helpful.

Ask your healthcare team about support groups in your area. Other sources of information include the National Cancer Institute and the American Cancer Society.

Make an appointment with your healthcare professional if you have any symptoms that worry you.

You may be referred to a doctor who specializes in diseases that affect blood cells, called a hematologist. Or you may be referred to a doctor who specializes in treating cancer, called an oncologist.

Here's some information to help you get ready for your appointment.

What you can do

• Be aware of anything you need to do ahead of time. At the time you make the appointment, be sure to ask if there's anything you need to do in advance, such as restrict your diet.
• Write down symptoms you have, including any that may not seem related to the reason for which you scheduled the appointment.
• Write down important personal information, including major stresses or recent life changes.
• Make a list of all medicines, vitamins and supplements you're taking and the doses.
• Take a family member or friend along. It can be hard to remember all the information provided during an appointment. Someone who goes with you may remember something that you missed or forgot.
• Write down questions to ask your healthcare team.

Your time with your healthcare team is limited, so preparing a list of questions can help you make the most of your time together. List your questions from most important to least important in case time runs out. For mantle cell lymphoma, some basic questions to ask include:

• Do I have mantle cell lymphoma?
• What is the stage of my mantle cell lymphoma?
• Will I need more tests?
• What are the treatment options?
• What are the potential side effects of each treatment?
• How will each treatment affect my daily life?
• Is there one treatment option you believe is the best?
• What would you recommend to a friend or family member in my situation?
• Should I see a specialist?
• Are there any brochures or other printed material that I can take with me? What websites do you recommend?
• What will determine whether I should plan for a follow-up visit?

Don't hesitate to ask other questions.

What to expect from your doctor

Be prepared to answer questions, such as:

• When did your symptoms begin?
• Have your symptoms been continuous or occasional?
• How severe are your symptoms?
• What, if anything, seems to improve your symptoms?
• What, if anything, appears to worsen your symptoms?
• Have you had any fevers, night sweats or weight loss?
• Have you noticed any lumps, swelling or pain anywhere in your body?
• Have you had any other cancers in the past?
• Do you have any other health conditions, such as diabetes, heart disease or kidney problems?
• What are your biggest concerns or fears about your diagnosis or treatment?