A uncommon and severe kind of non-Hodgkin lymphoma (NHL) that affects B cells—a subset of white blood cells that aids in the defense against infections—is called mantle cell lymphoma (MCL). When B lymphocytes proliferate uncontrollably and develop into tumors in the bone marrow, spleen, lymph nodes, blood, or other organs, MCL occurs.
The exact causes of MCL are not fully understood, but they are likely to involve a combination of genetic and environmental factors. Some of the possible causes include:
Genetic mutation: MCL is associated with a specific genetic mutation called the t(11;14) translocation, which occurs when a part of chromosome 11 breaks off and attaches to a part of chromosome 14. This results in the overexpression of a protein called cyclin D1, which promotes the growth and survival of the B cells.
Immune system dysfunction: MCL may be influenced by the abnormal function or regulation of the immune system, which normally helps protect the body from infections and cancers. This may be due to aging, chronic inflammation, infection, or autoimmune disease.
Environmental exposure: MCL may be linked to exposure to certain chemicals, radiation, or viruses, that can damage the DNA of the B cells and make them more prone to cancer.
The symptoms of MCL can vary from person to person, but they generally include:
Swollen lymph nodes: Enlarged or tender lymph nodes in the neck, armpit, groin, or abdomen, that do not go away or change with infection.
Fatigue: Feeling tired, weak, or exhausted, due to low levels of red blood cells, which carry oxygen to the tissues.
Fever: Having a high body temperature, usually above 38°C (100.4°F), that does not respond to antibiotics or antipyretics.
Night sweats: Having excessive sweating during the night, that soaks the sheets or clothing.
Weight loss: Losing weight unintentionally, without changes in diet or exercise.
Abdominal pain or discomfort: Having pain, bloating, or fullness in the abdomen, due to enlarged spleen, liver, or lymph nodes, or due to intestinal obstruction or perforation.
Infections: Having frequent or severe infections, such as pneumonia, skin infections, or urinary tract infections, due to low levels of white blood cells, which fight infections.
Bleeding or bruising: Having easy bleeding or bruising, such as nosebleeds, gum bleeding, or petechiae (tiny red spots on the skin), due to low levels of platelets, which help blood clotting.
If you have symptoms of MCL, you should see your doctor for a diagnosis. Your doctor may perform a physical exam, ask about your medical history and family history of lymphoma, and order some tests, such as:
Blood tests: To check your levels of white blood cells, red blood cells, platelets, and other markers of inflammation, infection, or organ function.
Lymph node biopsy: To remove a small sample of lymph node tissue and examine it under a microscope to look for cancer cells and determine their type and grade.
Bone marrow biopsy: To remove a small sample of bone marrow tissue from your hip or chest bone and analyze it for the presence of cancer cells and their genetic features.
Imaging tests: To visualize your lymph nodes and other organs and look for any signs of tumor, such as enlargement, compression, or invasion, using X-rays, ultrasound, CT scan, MRI, or PET scan.
The treatment of MCL depends on the stage, type, and grade of the cancer, and your overall health and preferences. The main treatments for MCL are:
Chemotherapy: The intravenous or oral administration of medications that either eradicate or inhibit the spread of cancer cells throughout the body. Combining chemotherapy with additional therapies like immunotherapy, targeted therapy, or stem cell transplantation is an option as well as using it alone. Chemotherapeutic agents, including CHOP, R-CHOP, and bendamustine, differ in their modes of action and adverse effects; yet, they all function by disrupting the cell cycle or metabolism of cancer cells.
Immunotherapy: The use of oral or injectable medications that strengthen or alter the immune system to combat cancer cells. Immunotherapy can be used either on its own or in conjunction with other medical procedures including stem cell transplantation, chemotherapy, or targeted therapy. All forms of immunotherapy, including rituximab, ibrutinib, and CAR T-cell treatment, function by either stimulating or suppressing the activity or signaling of immune cells or molecules. Their targets and adverse effects vary.
Using medications that can specifically target molecules or pathways, such as cyclin D1, BTK, or BCL-2, that are essential to the development or survival of cancer cells is known as targeted treatment. Targeted therapy can be applied either on its own or in conjunction with other medical interventions like immunotherapy, chemotherapy, or stem cell transplantation. Targeted therapies come in several forms, including bortezomib, venetoclax, or acalabrutinib. Each has a unique target and set of adverse effects, but they all act by obstructing or reducing the target's ability to function or communicate.
Stem cell transplant: To replace unhealthy or diseased bone marrow with healthy stem cells from an allogeneic (donor) or autologous (your own body) donor, normal blood cell production is restored, and the immune system is strengthened. After high-dose chemotherapy, immunotherapy, or targeted treatment, a stem cell transplant can be utilized to eradicate any cancer cells that may still be present and stop recurrence. A stem cell transplant is a difficult and dangerous process that needs close supervision and monitoring. Serious side effects include organ damage, infection, and graft-versus-host disease.
While there is no sure way to prevent MCL, there are some steps you can take to reduce your risk or recurrence of MCL, such as:
Seeking help early: If you notice any signs or symptoms of MCL, do not hesitate to consult your doctor and get the diagnosis and treatment you need. The sooner you get help, the better your chances of survival and recovery.
Observing your treatment plan: If your doctor or oncologist has diagnosed MCL and given you a treatment plan, make sure you adhere to their instructions. This might entail coming to your follow-up visits, taking your medicine as prescribed, and getting tests and scans on a regular basis. Your progress or result may be impacted by stopping or altering your therapy without first speaking with your physician or oncologist.
Self-care: MCL may have an impact on your physical and mental well-being, so it's critical to look after yourself. This includes maintaining a good, balanced diet, getting enough sleep and exercise, abstaining from stress and tobacco use, and asking for emotional support from friends, family, or a therapist. These routines can help you stay healthy overall, avoid or lessen the effects of other variables that could aggravate your MCL.
