If you or a loved one has been diagnosed with mantle-cell lymphoma (MCL), you likely have many questions about the disease. Understanding MCL begins with a brief explanation of the lymphatic system, which is where this type of cancer begins.
We have a complex lymphatic system that includes fluid (lymph) and lymph nodes that help our bodies to work efficiently and to fight germs. There are hundreds of pea-sized lymph nodes throughout the body, including in the chest, neck, armpits, groin, pelvis, and abdomen.1
Lymph nodes and other lymphoid tissue contain high numbers of lymphocytes. Lymphocytes are a type of white blood cell, and their primary function is to fight infection. The 3 types of lymphocytes are natural killer cells, B-cells, and T-cells. Lymphocytes are made in bone marrow, and then are moved by blood to the lymphatic system.2
Cancers that develop in the lymphatic system are called lymphomas. Lymphomas can involve either B-cells or T-cells. The 2 main forms of lymphoma are Hodgkin lymphoma and non-Hodgkin lymphoma (NHL).1
MCL is 1 of about 70 different subtypes of NHL. Patients are usually diagnosed with MCL in their 60s, and the disease affects men twice as often as women.3
If you have MCL, some of your lymphocytes, called “B-cell” lymphocytes, change into cancer cells and begin to multiply quickly. These cancer cells start to form tumors in your lymph nodes. The disease is called “mantle-cell lymphoma” because the tumor cells originate from the outer edge of the lymph nodes, called the “mantle zone.”1
Cancer cells do not behave like normal cells. First, cancer cells tend to grow more quickly and live longer than normal cells. Normal cells grow and then divide to form new cells when needed. They also die when old or damaged. In contrast, cancer cells make new cells that aren’t needed and don’t die quickly when old or damaged.4 In MCL, the cancerous cells may build up in tissues and may travel in blood or lymph to other areas of the body, such as the bone marrow, digestive tract, spleen, liver, and other lymph nodes. Without treatment, the cancer may cause organs not to work.3,5
The exact cause of MCL is not known, but about 85% of patients with MCL have the same type of genetic mutation in their chromosomes. This genetic abnormality leads to an overproduction of an intracellular protein (called cyclin D1) that causes tumor cell division and growth. The overproduction of the cyclin D1 protein leads to an accumulation of large numbers of MCL cells.6
Although researchers are working to better understand the underlying biochemical processes that result in MCL, much work remains to be done. The following articles in this series will explain how MCL is diagnosed and how patients are treated.
References
- AstraZeneca. About mantle cell lymphoma (MCL). www.calquence.com/patient/about-mantle-cell-lymphoma.html. Last updated January 2019. Accessed February 23, 2019.
- National Comprehensive Cancer Network. NCCN Guidelines for Patients®: Mantle Cell Lymphoma. www.nccn.org/patients/guidelines/nhl-mantle/files/assets/common/downloads/files/mantlecell.pdf. 2017. Accessed February 23, 2019.
- Vose JM. Mantle cell lymphoma: 2017 update on diagnosis, risk-stratification, and clinical management. Am J Hematol. 2017;92:806-813.
- Cancer Research UK. Cancer cells. www.cancerresearchuk.org/about-cancer/what-is-cancer/how-cancer-starts/cancer-cells. Last reviewed October 28, 2014. Accessed February 24, 2019.
- National Organization for Rare Disorders. Mantle cell lymphoma. https://rarediseases.org/rare-diseases/mantle-cell-lymphoma. Accessed February 24, 2019.
- Leukemia & Lymphoma Society. Mantle cell lymphoma. www.lls.org/sites/default/files/file_assets/FS4_MCL_Facts_2018-final.pdf. Accessed March 1, 2019.