Mantle cell lymphoma (MCL)
What is it?
Mantle cell lymphoma is a neoplastic disease characterised by the presence of abnormal lymphocytes originating from peripheral B-cells in the inner follicles of the lymph node mantle.
Causes and risk factors
Mantle cell lymphoma accounts for 2-8% of all non-Hodgkin’s lymphomas in the United States and 7-10% of all non-Hodgkin’s lymphomas in Western Europe. The incidence varies by geographic area. It is more common in Central Europe (Switzerland and Northern Italy) than in Canada, North America, South Africa, England, Germany, and Hong Kong. Mantle cell lymphoma is more common in men. The average age at diagnosis is between 55 and 65.
The disease risk factors are not known. In some cases, lymphoma is caused by changes in DNA (mutations in oncogenes). Possible etiological causes can be attributed to known environmental factors such as diet and exposure to solvents, pesticides, fertilizers, and smoking.
Accumulation of leukemic cells in the bone marrow leads to a decrease in hemoglobin and platelet levels, while accumulation in the lymph nodes, spleen, and liver leads to enlargement of these organs.
Which are the symptoms?
Very often the diagnosis is associated with the appearance of the following clinical manifestations:
- enlargement of superficial and deep lymph nodes;
- enlargement of the spleen (splenomegaly) and liver (hepatomegaly);
- lesions of the Waldeyer's ring
- Bone marrow involvement;
- localisation of abnormal lymphocytes in various organs (especially in the gastrointestinal tract);
- gastrointestinal symptoms such as abdominal pain, diarrhoea or bleeding;
- intestinal polyposis;
- accumulation of abnormal lymphocytes in the peripheral blood;
- non-specific malaise;
- decreased exercise tolerance;
At any time during the illness, patients may experience:
- unexplained weight loss (>10% in the last 6 months);
- night sweating;
- fever above 38°C (for 2 weeks in the absence of infection);
- recurrent infections.
How is it diagnosed?
How is it treated?
Standard therapy consists of pharmacological treatment with chemotherapy or immunotherapy (monoclonal antibodies) and radiation therapy. The drugs used (individually or in combination) depend mainly on the age of the patients. In individual cases, for young patients with very aggressive disease, stem cell transplantation (autologous or from a compatible donor) may be considered.
Where do we treat it?
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