Mantle cell lymphoma a very rare disease is cancer that is a subtype of B-cell non-Hodgkin lymphomas (NHLs) that affects certain cells of the immune system. Treatment depends on the course of the lymphoma and the person’s condition.
Mantle Cell Lymphoma. Image Courtesy of Gabriel Caponetti
What is mantle cell lymphoma?
Table of Contents
Mantle cell lymphoma is a form of non-Hodgkin’s lymphoma. In other words, it is a form of cancer of the lymphatic system that affects cells of the immune system, i.e. certain cells involved in the body’s defense.
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In the case of mantle cell lymphoma, it is the B lymphocytes (B Cells) that are responsible for producing antibodies. The term ‘mantle’ refers to the place where lymphoma develops, namely a specific area of the lymph nodes called the ‘mantle zone’.
Mantle cell lymphoma progresses slowly and can take years before it is diagnosed. It usually goes undetected for several years before it becomes apparent.
Cause of mantle cell lymphoma
Mantle cell lymphoma has a genetic origin. It is caused by a type of genetic change known as translocation: a segment is exchanged between two chromosomes that do not belong to the same pair of chromosomes.
The translocation affects chromosomes 11 and 14 and causes excessive production of a specific protein, cyclin D1. This abnormality leads to abnormal proliferation of B cells in the mantle.
Diagnosis of mantle cell lymphoma
Your doctor may suspect non-Hodgkin’s lymphoma on the basis of a clinical examination. However, the diagnosis of mantle cell lymphoma usually needs to be confirmed by blood tests, imaging studies, and biopsy (taking a tissue sample).
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Mantle cell lymphoma affects different parts of the body, such as lymph nodes, bone marrow, spleen, and blood. In some cases, the gastrointestinal tract is also affected.
Who is at risk for mantle cell lymphoma?
Mantle cell lymphoma is a rare disease. It accounts for 6% of non-Hodgkin’s lymphomas in Western countries. In the US it is estimated that one person per 200 000 people has the disease.
Mantle cell lymphoma is most common after the age of 60. The disease is more common in men, with a ratio of 3 men to 1 woman affected.
Symptoms of mantle cell lymphoma
Swollen lymph nodes
The lymphoma can remain asymptomatic for many years. If it does occur, swelling of one or more lymph nodes is a common symptom.
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In addition to swollen lymph nodes the condition may be associated with the following symptoms:
- Persistent fever
- Night sweats
- Unexplained weight loss
- Damage to the digestive tract with abdominal pain, diarrhea, nausea or vomiting, and sometimes gastrointestinal bleeding.
How is mantle cell lymphoma treated?
Treatment depends on the course of the lymphoma and the person’s condition.
Medical supervision (watch and Wait)
In rare cases of limited mantle cell lymphoma, treatment consists only of regular medical supervision.
Radiotherapy
If the mantle cell lymphoma is localized, radiotherapy may be offered. This technique involves exposing the tumor area to radiation that destroys the diseased cells.
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Immunotherapy
In most cases, immunotherapy is used. Its aim is to stimulate the body’s immune system to prevent cancer cells from developing.
Chemotherapy
Immunotherapy is usually combined with chemotherapy, which relies on the use of chemicals to kill cancer cells.
Hematopoietic stem cell transplantation
In young people, hematopoietic stem cell transplantation may be considered. These immature cells help to produce healthy blood and lymphoid cells.
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Prevention of mantle cell lymphoma
To date, no preventive measures have been identified. Research is being carried out to better understand the development of this type of lymphoma.
Final thoughts
Because Mantle cell lymphoma goes undetected for many years its treatment is usually delayed because of the late diagnosis. Ideally, it would be best to catch it at the first stage as treating it at that time is much easier and the long-term survival rate could be more than 20 years.
References
https://www.ncbi.nlm.nih.gov/books/NBK536985/
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