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What is Mantle Cell Lymphoma?

Mantle cell lymphoma (MCL) is a rare type of cancer that belongs to a family of cancers known as non-Hodgkin lymphoma (NHL). MCL gets its name from the location where the tumor cells develop as they form in the mantle zone of the lymph node.1

Facts about MCL:

  • MCL usually affects men over the age of 601
  • Nearly 3,000 people are diagnosed with MCL each year, which accounts for 6% of all NHL cases in the United States2

What is the Role of B Cells in MCL?

MCL is a B-cell cancer.3 A B cell is a type of white blood cell (lymphocyte) that forms from a stem cell in bone marrow. B cells play an important role in fighting infection.3

Cells have a specific life cycle in which they naturally mature and die. Healthy B cells are produced in the bone marrow, mature in the lymph nodes, die, and are removed from the body by the immune system.4

B-cell cancers, such as MCL, happen when there is an interruption in the normal life cycle of a B cell. Cancerous B cells do not die at the end of their life cycle and begin reproducing more faulty B cells. This creates too many B cells within the bone marrow and they begin traveling throughout the body (to the lymph nodes and other areas of the immune system) where they can cause tumors.4

Causes and Risk Factors

The exact cause of MCL is unknown.5 However, in approximately 85% of all cases of MCL, doctors find a genetic change in which pieces of one chromosome (chromosome 11) switch places with another (chromosome 14). This change, known as translocation, causes too much protein (cyclin D1) to be made, which makes the cells divide and grow too quickly.6 MCL is more often diagnosed in men than in women and is somewhat more common in European populations.4

Signs and Symptoms

Unfortunately, MCL is usually not found until it has spread to other parts of the body such as the lymph nodes and the spleen.1,7

People with MCL may lose their appetite and/or suffer from7:

  • Weight loss
  • Fever
  • Night sweats
  • Nausea
  • Vomiting
  • Indigestion
  • Abdominal pain
  • Bloating and fatigue

Diagnosis and Treatment

If you have symptoms of MCL, typically your doctor will first perform a physical exam and test your blood for the disease. Then your doctor will ask for a biopsy to be performed, whereby a small sample of a lymph node is removed for detailed analysis to confirm the diagnosis.8 A computed tomography (CT) scan may also be conducted to determine if the cancer has spread.1

Treatment options include:

  • Chemotherapy—Medications that are given either orally, by injection, or by infusion to destroy or control cancer cells9
  • Immunotherapy (including monoclonal antibodies)—Medications that help the body’s immune system fight cancer10
  • Kinase inhibitors—Medications that block the activity of a protein called a kinase. A kinase can tell leukemia cells to divide and help them survive. By blocking this protein, kinase inhibitors can address changes inside of cells that may cause them to become cancerous11
  • Stem cell transplant—A process by which stem cells (cells that create new, undamaged cells) are removed and stored at the hospital while treatment is ongoing. After treatment, these stem cells are reintroduced (infused) into the bone marrow to help create new blood cells12

New Hope for People With MCL

Today, new treatment options—ones that have been FDA approved or that are in clinical trials—offer hope that MCL can be controlled.

Clinical trials are underway across the United States to test potential new treatments for MCL. Oral formulations (pills), intravenous medications (given directly into the bloodstream), and new combinations of treatments are being studied in these trials.

Some treatments are available now, even while they continue to be tested. Early reports from these clinical trials to test new treatments are encouraging. In some of these studies, people treated with newer therapies experienced significant remission of their disease.13

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Scientists have learned how to disrupt certain pathways in cancer cells. Disrupting these pathways can encourage defective white blood cells to die or stop growing, interfere less with normal blood cells, and not collect in the lymph nodes and other organs.

In cases in which the patient has relapsed or MCL does not respond to therapy, other forms of chemotherapy or immunotherapy may be explored.14

As with any medical decision, it is important to discuss all treatment options with your doctor or healthcare provider.

You Are Not Alone

Now is an important time to look to your healthcare providers and caregivers for support. There are also plenty of resources available if you would like to learn more about MCL. Start by visiting the following Web sites*:

* These resources are an incomplete listing of cancer support organizations and are not controlled by, endorsed by, or affiliated with Pharmacyclics, LLC, or Janssen Biotech, Inc. The list is meant for informational purposes only and is not intended to replace your healthcare professional’s medical advice. Ask your doctor or oncology nurse educator any questions you may have about your cancer or treatment plan.

Glossary of Terms

Here are the definitions of terms you may come across as you learn more about MCL.

Chromosomes: Threadlike parts inside cells that carry hereditary information. Chromosomes are made up of protein and DNA.

Controlled: When treatment by a doctor relieves symptoms and keeps MCL from getting worse.

Kinase inhibitor: Blocks the activity of a protein called a kinase, which can tell leukemia cells to divide and help them survive. By blocking this protein, a kinase inhibitor can address changes inside of cells that cause them to become cancerous.11

Lymph nodes: Small immune system organs that store white blood cells until they are needed to fight disease. Lymph nodes are scattered around your body in places such as the base of the neck, groin, and armpits.

Lymphocytes: White blood cells that help the body fight disease. The two main types are B cells and T cells. B cells make antibodies that attack bacteria and toxins. T cells attack cells that have been taken over by viruses (such as HIV) or have become cancerous.

Lymphoma: The most common blood cancer. It occurs when white blood cells called lymphocytes grow abnormally. Cancerous lymphocytes can travel to the lymph nodes, blood, spleen, bone marrow, or other organs. They can also clump together to form tumors.

Mantle zone (Also called a corona): A circle of lymphocytes (white blood cells) surrounding lymph node growth areas. MCL develops in the mantle of lymph nodes.

Progressed: When MCL becomes worse. Symptoms become more serious and signs of the disease increase.

Remission: When signs of MCL go away, it is called "total remission." When signs of MCL are reduced by at least 50%, it is called "partial remission."14

White blood cells: One of the main parts of blood. White blood cells fight disease. White blood cells are made in the bone marrow and wait in the lymph nodes until they are needed.

References:
  1. Mantle cell lymphoma: overview. Lymphoma Research Foundation Web site. http://www.lymphoma.org/site/pp.asp?c=bkLTKaOQLmK8E&b=6300157. Accessed September 23, 2014.
  2. Getting the facts: mantle cell lymphoma. Lymphoma Research Foundation Web site. http://www.lymphoma.org/atf/cf/%7Baaf3b4e5-2c43-404c-afe5-fd903c87b254%7D/MCL_11.11.PDF. Accessed November 6, 2014.
  3. Dictionary of terms: B cell [definition]. National Cancer Institute Web site. http://www.cancer.gov/dictionary?cdrid=45611. Accessed September 23, 2014.
  4. Mantle cell lymphoma facts: about mantle cell lymphoma. Leukemia and Lymphoma Society Web site. http://www.lls.org/content/nationalcontent/resourcecenter/freeeducationmaterials/lymphoma/pdf/mantlecelllymphoma.pdf. Accessed September 23, 2014.
  5. Rare disease report: mantle cell lymphoma: causes. National Organization for Rare Disorders Web site. https://www.rarediseases.org/rare-disease-information/rare-diseases/byID/1114/viewFullReport. Accessed September 23, 2014.
  6. Mantle cell lymphoma facts: causes. Leukemia and Lymphoma Society Web site. http://www.lls.org/content/nationalcontent/resourcecenter/freeeducationmaterials/lymphoma/pdf/mantlecelllymphoma.pdf. Accessed September 23, 2014.
  7. Mantle cell lymphoma facts: signs, symptoms and complications. Leukemia and Lymphoma Society Web site. http://www.lls.org/content/nationalcontent/resourcecenter/freeeducationmaterials/lymphoma/pdf/mantlecelllymphoma.pdf. Accessed September 23, 2014.
  8. Mantle cell lymphoma facts: diagnosis. Leukemia and Lymphoma Society Web site. http://www.lls.org/content/nationalcontent/resourcecenter/freeeducationmaterials/lymphoma/pdf/mantlecelllymphoma.pdf. Accessed September 23, 2014.
  9. Rare disease report: mantle cell lymphoma: treatment. National Organization for Rare Disorders Web site. https://www.rarediseases.org/rare-disease-information/rare-diseases/byID/1114/viewFullReport. Accessed September 23, 2014.
  10. Mantle cell lymphoma facts: treatment for patients with relapsed and/or refractory MCL. Leukemia and Lymphoma Society Web site. http://www.lls.org/content/nationalcontent/resourcecenter/freeeducationmaterials/lymphoma/pdf/mantlecelllymphoma.pdf. Accessed September 23, 2014.
  11. Getting the facts: biologic and other novel agents in lymphoma. Lymphoma Research Foundation Web site. http://www.lymphoma.org/atf/cf/%7Baaf3b4e5-2c43-404c-afe5-fd903c87b254%7D/LRF_FACTSHEET_BIOLOGIC_AND_OTHER_NOVEL_AGENTS_IN_LYMPHOMA.PDF. Accessed September 23, 2014.
  12. Mantle cell lymphoma facts: types of treatment. Leukemia and Lymphoma Society Web site. http://www.lls.org/content/nationalcontent/resourcecenter/freeeducationmaterials/lymphoma/pdf/mantlecelllymphoma.pdf. Accessed September 23, 2014.
  13. Current and emerging treatment approaches in relapsed/refractory mantle cell lymphoma. Medscape Multispecialty Web site. http://www.medscape.org/viewarticle/82093. Accessed October 22, 2014.
  14. Cheson BD, Pfistner B, Juweid ME, et al. Revised response criteria for malignant lymphoma. J Clin Oncol. 2007;25(5):579-586. http://jco.ascopubs.org/content/25/5/579.full.pdf. Accessed October 22, 2014.