![Most people with mantle cell lymphoma respond to treatment initially, but the cancer will later progress after chemotherapy. The average life expectancy of patients with mantle cell lymphoma is about 6 to 7 years, while the 10-year survival rate is 5 to 10 percent.](https://images.medicinenet.com/images/article/main_image/how-bad-is-lymphoma-cancer.jpg?output-quality=75)
Mantle cell lymphoma (MCL) is a rare, aggressive form of non-Hodgkin lymphoma that originates in cells from a part of the lymph nodes called the “mantle zone.” About 6% of six percent of non-Hodgkin lymphoma cases in the U.S. are mantle cell lymphomas.
Mantle Cell Lymphoma Prognosis
Mantle cell lymphoma is generally not able to be cured with standard treatments and the prognosis is usually poor. About 50 to 70% of patients respond to some regimens initially, but the cancer tends to progress after chemotherapy. The average life expectancy of patients with mantle cell lymphoma is about 6 to 7 years. The 10-year survival rate, that is, how many people will be alive 10 years after diagnosis, is only 5 to 10 percent.
What Are Symptoms of Mantle Cell Lymphoma?
Mantle cell lymphoma may not cause any symptoms during the early stages of the disease. When symptoms occur, they may include:
- Lymph node swelling
- Persistent, usually painless, swelling of certain lymph nodes, especially lymph nodes within the neck and throat region
- Enlargement of other lymph nodes, such as nodes at the elbows or shoulders, under the arms, in the chest, abdominal, and/or pelvic regions
- Digestive symptoms
- Loss of appetite
- Nausea
- Vomiting
- Indigestion
- A feeling of fullness
- Abdominal swelling (distension) or bloating
- Abdominal pain or discomfort
- Generalized symptoms (B symptoms)
- Persistent or repeated fever
- Unexplained weight loss
- Sweating, particularly at night (night sweats)
- If MCL has spread to involve the bone marrow it may cause anemia
- Fatigue
- Listlessness and lack of energy
- Pale skin (pallor)
- Headaches
- Susceptibility to certain infections
- Easy bruising and excessive bleeding
- Involvement of the digestive tract which may be associated with the development of multiple polyps within the intestines
- In rare cases, MCL may spread to involve the brain and spinal cord resulting in neurologic signs
What Causes Mantle Cell Lymphoma?
The exact cause of mantle cell lymphoma is unknown but factors that may contribute to its development include:
- Genetic abnormalities
- Immunologic abnormalities
- Environmental factors
Other possible causes that are being investigated include:
- Family history
- Inflammation
- Infection
How Is Mantle Cell Lymphoma Diagnosed?
Mantle cell lymphoma is diagnosed with a patient history and a physical examination in which a doctor may feel (palpate) the lymph nodes in a certain area to detect swelling, including in the neck, tonsil, and adenoidal regions, under the arms, and in the groin.
A biopsy of an affected lymph node or the bone marrow is also performed to help confirm a diagnosis of mantle cell lymphoma.
Other tests used to evaluate the extent and the effects of the illness may include:
- Blood tests
- Complete blood count (CBC)
- Liver enzyme studies
- Renal function tests
- Tests to measure levels of the enzyme lactate dehydrogenase (ldh)
- Calcium levels
- Uric acid levels
- Imaging tests
- X-rays
- Computed tomography (CT) scan
- Magnetic resonance imaging (MRI) scan
- Positron emission tomography (PET) scan
- Analysis of cerebrospinal fluid (CSF)
- Endoscopy to look for polyps
- Other tests to evaluate the functioning of the heart and lungs
What Is the Treatment for Mantle Cell Lymphoma?
Treatment for mantle cell lymphoma depends on several factors, including the stage of disease, patient’s age, and their overall health. MCL is often diagnosed once it has spread (metastasized) throughout the body, and it is considered a difficult cancer to treat.
Treatment for mantle cell lymphoma includes:
- Active surveillance, also called “watchful waiting”
- For patients who do not yet have symptoms and who have a relatively small amount of slow-growing disease
- The disease is monitored with regular checkups visits and testing
- Active treatment is started if the patient develops lymphoma-related symptoms or there are signs the disease is progressing
- Chemotherapy
- Initial treatment for aggressive MCL in younger patients usually includes chemotherapy, typically in combination with the monoclonal antibody rituximab (Rituxan), followed by autologous stem cell transplantation (in which patients receive their own stem cells)
- R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone)
- Bendamustine (Treanda) in combination with rituximab
- Hyper-CVAD (cyclophosphamide, doxorubicin, vincristine, dexamethasone alternating with high-dose methotrexate and cytarabine) plus rituximab
- Initial treatment for aggressive MCL in younger patients usually includes chemotherapy, typically in combination with the monoclonal antibody rituximab (Rituxan), followed by autologous stem cell transplantation (in which patients receive their own stem cells)
- Proteosome inhibitor
- Transplantation
- Allogeneic stem cell transplantation (in which patients receive stem cells from a related or unrelated donor)
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https://lymphoma.org/aboutlymphoma/nhl/mcl/
https://rarediseases.org/rare-diseases/mantle-cell-lymphoma/
https://www.cancertherapyadvisor.com/home/decision-support-in-medicine/hematology/mantle-cell-lymphoma/