What Is Mantle Cell Lymphoma?
Lymphoma is a type of blood cancer developing when the white blood cells called lymphocytes grow out of control. There are two types of lymphocytes: B lymphocytes (B cells) and T lymphocytes (T cells). Depending on the type of cells lymphoma contains it is grouped as Hodgkin lymphomas or non-Hodgkin lymphomas. Mantle cell lymphoma is an uncommon type of blood cancer and a subtype of B-cell non-Hodgkin lymphomas (NHLs). It is referred to as mantle cell lymphoma because the abnormal B cells develop in a lymph node part called the mantle zone. The abnormal B lymphocyte grows uncontrolled, accumulating lymphoma cells and leading to the enlargement of lymph nodes. These lymph nodes enlarge or grow in other body parts forming tumors. These cancer cells can enter the lymphatic channels and spread to other lymph nodes or tissues, like the bone marrow, gastrointestinal tract, and liver. Mantle cell lymphoma is unusual cancer because it has features of both high-grade lymphomas (usually grows quickly) and low-grade lymphoma (comes back after treatment and needs more treatment).
What Are the Types of Mantle Cell Lymphoma?
There are two types of mantle cell lymphoma:
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Nodal Mantle Cell Lymphoma - The most common type of mantle cell lymphoma. It affects the lymph nodes and spreads to other body parts, such as the bloodstream, bone marrow, liver, and gastrointestinal tract. It is usually high-grade lymphoma. There are two subtypes of high-grade variants of nodal mantle cell lymphoma: pleomorphic mantle cell lymphoma and blastoid mantle cell lymphoma.
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Leukemic Non-Nodal Mantle Cell Lymphoma - This is not a commonly seen low-grade mantle cell lymphoma. The affected individuals have swollen spleen and lymphoma cells in their bone marrow and blood vessels but often do not develop swollen lymph nodes.
What Is the Cause of Mantle Cell Lymphoma?
In most cases, it is believed that individuals with this disease and other lymphomas have a gene mutation or change in some of their genes. The genetic lesion involves chromosome 11 and chromosome 14. A reciprocal translocation of the two genes happens, resulting in short segments of chromosomes 11 and 14 exchanging places. This exchange takes place at the cyclin D1 gene site, present on chromosome 11 and on the site where the formation of antibody molecules happens on chromosome 14. This triggers an overproduction of cyclin D1 (a protein responsible for tumor cell division and growth), leading to the accumulation of uncontrolled growth of a type of B cell, causing mantle cell lymphoma. In some rare cases, MCL may not occur due to chromosomal 11 and 14 reciprocal translocation. It can be due to other genetic changes causing excess cyclin D1 production. Rarely, MCL can occur due to overexpression of other cyclin genes like cyclin D2 and cyclin D3.
What Signs and Symptoms Does a Mantle Cell Lymphoma Patient Have?
People with MCL have a disease involving many lymph nodes and other body sites like the spleen, bone marrow, bloodstream, liver, or gastrointestinal tract. Rarely, it also invades the brain, lungs, and spinal cord. The symptoms experienced by mantle lymphoma patients are:
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Loss of appetite.
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Weight loss.
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Fever.
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Nausea and vomiting.
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Night sweats.
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Indigestion.
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Abdominal pain.
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Bloating or feeling of fullness.
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Pain and irritation due to enlarged tonsils.
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Pressure or pain in the lower back sometimes extends to one or both legs.
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Fatigue from developing anemia.
Complications that occur due to disease progression may include:
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A decrease in the production of blood cells as the growing lymphoma cells crowd out normal blood cells, causing low blood cell counts, anemia, and thrombocytopenia (low numbers of platelets).
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High white blood cell counts in the peripheral blood may result in the leukemia phase of the disease.
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Gastrointestinal complications like the development of multiple small-intestine polyps. Also, complications in the pulmonary and central nervous system (CNS).
What Is the Management for Mantle Cell Lymphoma?
The mantle cell lymphoma treatment happens soon after cancer diagnosis and staging. But with no symptoms and a slow-growing form of cancer, the physician may suggest watchful waiting, like visiting the doctor every two to three months and having tests every three to six months. If the patient develops any symptoms or their lymph nodes get bigger, then the doctor may start treatment which includes:
Chemotherapy and Immunotherapy Combination - Chemotherapy is a drug treatment using powerful chemicals to kill cancer cells. Immunotherapy drugs help the immune system recognize and destroy cancer cells. They can be administered through a pill or IV (intravenously). The combination drugs used are:
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R-CHOP (Rituximab, Cyclophosphamide, Doxorubicin hydrochloride, Vincristine sulfate, and Dexamethasone).
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R-DHAP (Rituximab, Dexamethasone, Cytarabine, and Cisplatin).
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Nordic protocol (Rituximab, Cyclophosphamide, Vincristine, Cytarabine, Doxorubicin, Prednisolone).
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Bendamustine and Rituximab.
Targeted Therapy - These drugs block proteins in cancer cells that help them to survive and spread.
Stem Cell Transplant - This treatment is suggested if the patient responds to chemotherapy and they are fit for high-dose treatment, also in the case of mantle cell lymphoma recurrence. Stem cells are present in the bone marrow and help produce new blood cells. There are two types of stem cell transplants that are autologous (stem cells are obtained from an individual's own body) and allogeneic (obtained from another individual). The doctors collect stem cells from a patient and are treated with high-dose chemotherapy or radiation treatment. This can be a hard period for the patient as it can cause many side effects like mouth ulcers, nausea, vomiting, etc.
What Are the Disparities Associated With Mantle Cell Lymphoma?
The treatment of mantle cell lymphoma with autologous stem cell transplantation and maintenance with Rituximab has provided good patient outcomes in recent years. However, not all patients achieve long-term remission leading to poor survival outcomes. This is because of the racial and socioeconomic disparities experienced by the patients. Racial disparities have been described in mantle cell lymphoma. In a National Cancer Database (NCDB) analysis, individuals of black race, uninsured, and lower socioeconomic status were less likely to receive chemotherapy. Also, black patients received less chemoimmunotherapy than white patients. Other analyses have shown that black patients with lymphoma present at younger ages with advanced disease conditions have lower treatment rates and delayed treatment from the time of diagnosis. So, the survival rate was less.
Intensive therapy is often provided for newly diagnosed patients, but patients with MCL who have poor access to care, lower socioeconomic status, or other disadvantages might be affected. Also, oral therapies usage such as Ibrutinib in the relapse stage often requires considerable cost and adherence to therapy, which might affect some patients and lead to poor outcomes. Therefore, the problems should be addressed, and current treatment should be open for all people without bias.
Conclusion:
Mantle cell lymphoma is an uncommon blood cancer type affecting the lymph nodes and other body parts. The treatment depends on the stage of MCL, age, and many other factors. However, socioeconomic and racial also play a key role in the treatment, so the outcome is affected. So it is necessary to check that all the patients receive proper and effective treatment without any bias to get a good outcome.
