EN
1. What is mantle cell lymphoma |
2. Symptoms of the disease |
3. Methods of treatment |
4. Treatment of metastases |
5. Prognosis of survivalи |
6. Leading clinics |
Mantle cell lymphoma (MCL) is a rare type of B-cell non-Hodgkin’s lymphoma. It arises from cells originating from the ‘mantle zone’ – the outer ring of small lymphocytes that surrounds the centre of the lymph nodes. The pathology is more often found in men than women. The average age of diagnosis is 60-70 years. It is currently impossible to completely get rid of the disease, but doctors can control its progression. Learn more about mantle cell lymphoma, its symptoms and treatment from our article.
What is mantle cell lymphoma?
Mantle cell lymphoma occurs in one person in 200,000. It accounts for about 5% of all non-Hodgkin’s lymphomas. The disease usually progresses rapidly, however, in some people it spreads more slowly.
There are 2 main types of mantle cell lymphoma: nodular and leukaemic non-nodular. In the first type, cancer affects the lymph nodes and can quickly spread to other organs, in particular the bone marrow, intestines and liver. The pathology is in turn divided into blastoid and polymorphic. Usually, it proceeds rapidly.
Leukaemic non-nodular mantle cell lymphoma is less common. It usually affects the spleen and bone marrow, but not the lymph nodes. There are also malignant cells in the bloodstream. This type of lymphoma tends to develop slowly.
The exact causes of the disease are unknown. Most patients have certain genetic mutations in their cells. They lead to increased levels of a protein called cyclin D1 and the development of B-cell lymphoma.
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Can mantle cell lymphoma be asymptomatic?
Mantle cell lymphoma is often diagnosed at a later stage. This is because the disease may be asymptomatic or symptom-free in its early stages.
As the disease progresses, patients develop enlarged lymph nodes in various parts of the body, particularly in the groin, neck and armpits. Many patients have an enlarged spleen. They also complain of excessive sweating at night, unexplained weight loss and fever.
Other signs of lymphoma may include:
constant fatigue,
increased vulnerability to infections,
itchy skin,
increased likelihood of bruising and bleeding.
The appearance of symptoms depends on which areas of the body are affected by mantle cell lymphoma. The disease often involves the internal organs, so patients experience abdominal or back pain, diarrhoea, bloating, nausea and vomiting.
Clinical guidelines for the treatment of mantle cell lymphoma
The best method of treatment for mantle cell lymphoma depends on the aggressiveness of the disease, the patient’s condition and their age. For people diagnosed with a slowly progressing cancer without any symptoms, treatment may not be carried out immediately.
Instead, active monitoring is recommended. This means that the patient will have regular check-ups with the doctor and monitor the course of the disease.
If the lymphoma begins to cause health problems, then treatment is necessary. The treatment options are the same as for the rapidly progressing MCL.
Usually, the patient is given combined chemotherapy. It is also possible to use immunotherapy (CAR-T cells) and steroids, or radiotherapy of the affected area.
For mantle cell lymphoma, chemotherapy regimens such as R-CHOP, R-DHAP, NORDIC, Maxi R-CHOP, Hyper-CVAD, CHOP and BR are used. The duration and intensity of this treatment are determined for each patient individually.
Patients who are in the good physical condition and do not have underlying severe pathologies are usually offered more intensive chemotherapy. It involves the use of the drug Cytarabine. This medicine can help prevent the spread of lymphoma cells to the central nervous system.
Patients who cannot be treated intensively with chemotherapy drugs are given a standard chemotherapy regimen in combination with Rituximab (most often CHOP or R-CHOP). If the patient responds well, then maintenance therapy may be recommended. This will control the lymphoma and keep it from developing for as long as possible. This treatment involves injections of a monoclonal antibody every 2 months for 2-3 years.
Some patients may be indicated for a stem cell transplant. It is performed after a course of intensive chemotherapy. It may also be recommended for relapsed mantle cell lymphoma. Usually, the patient’s cells are used. After the procedure, maintenance treatment with rituximab (a monoclonal antibody) is usually prescribed. This lasts for several years.
Can mantle cell lymphoma metastasise?
Mantle cell lymphoma can metastasise, spreading to other organs and tissues, particularly the gastrointestinal tract and skin. In some cases, it progresses quite rapidly. This form of pathology, therefore, requires timely and comprehensive treatment. This is the key to success in the fight against aggressive lymphoma. The right therapy programme can lead to remission that lasts for several years.
Read: How is refractory mantle cell lymphoma treated?
Refractory mantle cell lymphoma is a lymphoma that does not respond well to treatment. It is treated in the same way as recurrent lymphoma:
chemotherapy regimens that have not previously been used by the patient;
targeted drugs, particularly ibrutinib;
CAR-T cell therapy (usually recommended in patients who have relapsed more than once, or in patients who have been treated with intrubim);
allogeneic stem cell transplantation (donor cell transplantation is a very intensive treatment option and not suitable for everyone; it is usually indicated if the patient has already had an autologous transplant);
clinical trials of new treatments;
palliative care for symptom control.
The prognosis for mantle cell lymphoma
The prognosis for survival in this pathology depends on the type and degree of progression of the disease and the general health of the patient. It also depends on the methods used to fight the disease. For example, after traditional chemotherapy, the median survival rate is 3 years.
The International Prognostic Index for Mantle Cell Lymphoma divides patients into low, intermediate and high-risk groups. Based on this grading, the low-risk group has a 5-year overall survival rate of 60%, the intermediate-risk group has a survival rate of 51 months, and high-risk patients have a survival rate of 29 months or less.
Where is mantle cell lymphoma treated abroad?
Summary
Mantle cell lymphoma is a rare type of B-cell lymphoma that originates from the ‘mantle’ cells of lymph nodes. The disease most often affects mature and elderly men. It usually has an aggressive pattern of progression, but there are exceptions.
Several methods of treating MCL are available. The choice of one or the other is determined by the characteristics of the pathology, the general state of health and the age of the patient. First-line treatment usually includes chemotherapy combined with immunotherapy or irradiation of the affected area. This approach achieves complete or partial remission. After this, the patient may be recommended a stem cell transplant and/or an extended course of immune or targeted therapy. This will prolong the remission.
The average life expectancy for mantle cell lymphoma ranges from 1,8 to 9,4 years. Approximately 5-10% of patients pass the 10-year survival threshold.
Treatment for mantle cell lymphoma can be carried out at medical institutions such as Medicana, Medipol, Medistate, SoonChunHyang, Quiron Madrid and Navarra University Hospital.
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Sources:
- National Library of Medicine
- National Organization for Rare Disorders (NORD)
- Lymphoma Research Foundation
- Cancer Research UK
- Springer Nature
- American Society of Hematology
- Lymphoma Action
