Mantle Cell Lymphoma

Definition, Characteristics of Mantle Cell Lymphoma

What is mantle cell lymphoma, what are the risk factors, and what are the symptoms and treatments?. Credit: Istockphoto.com/Stock Photo©jessicaphoto


What is mantle cell lymphoma, and what should I know about this kind of cancer?

Mantle Cell Lymphoma - Definition

Mantle Cell Lymphoma is an uncommon type of non-Hodgkin's lymphoma (NHL). Only 5-7% of all NHL belong to this group. It is a lymphoma of B lymphocytes, or "B-cells." Though it looks like a slow growing, low-grade tumor under the microscope, it usually grows fast and behaves like a high-grade lymphoma.

  It is called a ‘mantle’ cell lymphoma because it first arises within a part of the lymph node called the ‘mantle’ zone.

Risk Factors - Who Gets Mantle Cell Lymphoma?

Mantle Cell Lymphoma is more common in men than in women, and in older individuals. The mean age at diagnosis is more than 60 years.  There are no definite risk-factors for getting this type of lymphoma,though many factors from infections to chemical exposures have been looked at as possible risk factors for non-Hodgkin's lymphoma in general.

Symptoms and Locations

Mantle cell lymphoma starts in the lymph nodes. The first thing that most people notice is enlarged nodes in the neck, groin or armpits. The disease generally grows fast and by the time it is seen by a doctor, the disease has spread to other organs in the body. For some people the disease is slow growing, something oncologists refer to as "indolent" but this is the exception.

The bone marrow, spleen and throat are affected in many people, and there are usually lymphoma cells in the blood. The intestines can also be affected in some people, and give rise to symptoms like abdominal pain or diarrhea.

Tests and Staging

Tests for Mantle cell lymphoma are the same as those for other kinds of lymphoma.

They include blood tests, scans and a bone marrow test. Once the tests reveal which parts of the body are affected, the disease stage can be determined. Most people have stage III or IV disease that has spread to different organs of the body.

Treatment and Outcomes

Mantle cell lymphoma is treated mainly with chemotherapy, but how aggressively it is treated depends significantly on age.  For young, healthy adults, chemotherapy followed by a stem cell transplant may be a good option, but entails significant toxicity.  Cure is uncommon unless the disease is diagnosed early, and the aim of treatment is to keep the disease in control for as long as possible.  Most treatments are combined with Rituxan (rituximab) and this medication may be used for maintenance following treatment as well.

Newer drugs such as Velcade (bortezomib) and Treanda (bendamustine) are an alternative to these chemotherapy regimens.  Many combinations of drugs and new drugs are being studied in clinical trials, and it's likely that new treatments and treatment combinations for mantle cell lymphoma will be available in the near future.

Since the disease is most often advanced, treatments such as surgery and radiation therapy often have only a limited role.

Coping

Coping with a new diagnosis of lymphoma can be devastating.  Where should you start when you are first diagnosed?  For some people, aggressive treatment may be the best option.   For others,  aggressive treatment may not be the best choice.  Even so, there is still hope for a good quality of life with the time you have.  Check out these tips on coping with terminal cancer Reach out to family and friends.  There is a large support community of people with lymphomas online for those who wish the support of others who are experiencing something similar.  And remember that research is ongoing, and that there are very promising advances taking place in cancer treatment today.

Sources:

Avivi, I., and A. Goy. Refining the Mantle Cell Lymphoma paradigm: Impact of Novel Therapies on Current Practice. Clinical Cancer Research. 2015. 21(17):3853-61.

Cheah, C., Seymour, J., and M. Wang. Mantle Cell Lymphoma. Journal of Clinical Oncology. Published online before print January 11, 2016.

Dreyling, M., Ferrero, S., and the European Mantle Cell Lymphoma Network. The role of targeted treatment in mantle cell lymphoma: is transplant dead or alive? Haematologica. 2016. 101(2):104-14.

Lunning, M., and J. Armitage. The place of transplantation in mantle cell lymphoma. Oncology. 2013. 27 Suppl 2:2-6.

Maddocks, K., and K. Blum. Treatment strategies in mantle cell lymphoma. Cancer Treatment and Research. 2015. 165:251-70.

Martin, P. Ibrutinib – a new standard treatment for relapsed mantle cell lymphoma? Lancet. 2015 Dec 4. (Epub ahead of print).

Tucker, D., and S. Rule. Ibrutinib for mantle cell lymphoma. Future Oncology. 2016. 12(4):477-91.

Vose, J. Mantle cell lymphoma: 2015 update on diagnosis, risk-stratification, and clinical management. American Journal of Hematology. 2015. 90(8):739-45.

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