Mantle cell lymphoma
What is mantle cell lymphoma (MCL)?
Mantle cell lymphoma is a relatively uncommon type of lymphoma, accounting for approximately 5% to 10% of all non-Hodgkin lymphomas.
It affects the outer edge (mantle zone) of B-cells in the lymph node follicle. In 85% of cases, an abnormality occurs when two chromosomes (11 and 14) break and join together with each other. This is called a ‘translocation’ and causes B-cells to over-produce a protein called cyclin D1. In usual quantities, Cyclin D1 helps to promote normal cell growth, with excess amounts resulting in uncontrolled growth of mantle zone cells which can lead to MCL.
How does mantle cell lymphoma affect the body?
Usually the first sign of MCL is a painless swelling in the neck, armpit and/or groin. Multiple lymph nodes may be affected as well as other sites of the body including the spleen, bone marrow, blood, tonsils and adenoids, liver, brain, spinal cord and gastrointestinal tract. MCL commonly affects the bowel, with worsening diarrhoea often a sign of this. Other symptoms may include abdominal bloating, nausea, tiredness, loss of appetite, fevers, unexplained weight loss and night sweats.
Who does mantle cell lymphoma commonly affect?
The average age at diagnosis of MCL is 60-65 years. It is two to three times more common in men than in women.
What causes mantle cell lymphoma?
The exact cause of MCL is unknown. It is not due to infection and cannot be passed on to other people.
How is mantle cell lymphoma treated?
Whilst MCL appears as a low-grade lymphoma under the microscope, most behave like a high-grade (aggressive) lymphoma. In the majority of cases they are treated as a high-grade lymphoma. MCL is usually treated with combination chemotherapy with or without rituximab (a monoclonal antibody). The most common regimens are: R-HyperCVAD, R-CHOP, and F+CR. Elderly people or those not well enough to endure the side-effects of combination chemotherapy may be given single drug treatments such as chlorambucil, cyclophosphamide and fludarabine*, most commonly given in tablet form.
Other therapies include radioimmunotherapy which combines radiotherapy with a monoclonal antibody drug (e.g. rituximab); radiotherapy; and stem cell transplants (autologous or allogeneic). Treatment is often successful initially, however relapses are common. New treatments for MCL are being researched all the time with such drugs as temsirolimus, bortezomib and lenalidomide being investigated in clinical trials in some Australian centres.
*Not approved by the Therapeutic Goods Administration for this indication in Australia. May be available in some health facilities through local protocols.Posted on February 1st, 2018