Determining the presence of mantle cell lymphoma involves a physical examination, microscopic analysis, genetic screening, and specialized antigen tests.
Your body’s lymphatic system is made up of lymph nodes, lymph vessels, and lymphatic organs, such as your tonsils and adenoids. Lymph fluid circulates among the structures of your lymph system, transporting important immune cells and helping your body maintain fluid balance.
Cancers that begin in your lymph system are called lymphomas. They affect the function and integrity of white blood cells called lymphocytes, which have a variety of jobs related to your immune response.
Mantle cell lymphoma (MCL) is a rare and aggressive type of lymphoma with an overall survival time of 4–5 years. Early diagnosis can help you get treatment as soon as possible, which may extend your life span and improve your quality of life.
MCL is a rare cancer of your lymphatic system.
It’s a type of B-cell non-Hodgkin’s lymphoma that affects specific lymphocytes called B-lymphocytes. These are white blood cells responsible for making antibodies to help fight infection.
MCL develops in the mantle zone of your lymphoid follicles. These are tissue nodules found throughout lymphatic structures that help start immune responses. The mantle zone, which consists of B cells called mantle cells, makes up the outer region of the follicle.
As a type of non-Hodgkin’s lymphoma, MCL is differentiated from Hodgkin’s lymphomas by the absence of Hodgkin cells and Reed-Sternberg cells.
Hodgkin cells and Reed-Sternberg cells are lymphocytes that have undergone significant changes. They are larger than they should be and have unusual, distinct features.
Non-Hodgkin’s lymphomas such as MCL don’t have Hodgkin or Reed-Sternberg cells. MCL accounts for
Most people experiencing MCL make a doctor’s visit for persistent, painless lymph node swelling in the neck and throat area. Fever, unexplained weight loss, and unusual sweating are also common.
During your first visit, your doctor will take your medical history and ask questions about your lifestyle habits, any chronic conditions in your family, and whether you’ve had any exposures to possible infectious agents.
Your doctor will then perform a thorough physical examination to check your baseline functions, such as heart rate, respiratory rate, and blood pressure. They will also use their fingers to feel lymph nodes in different areas of your body and to check for organ enlargement. This can involve putting pressure on your abdomen, which may be uncomfortable.
Initial tests for lymphoma involve blood work to check your organ function and look for unusual variations in specific cell counts.
Your doctor may also request imaging scans such as MRI, PET, or CT scans. These tests can allow your doctor to see your lymphatic organs and their structures.
Specific testing for MCL involves:
- antigen testing
- genetic screening
During a biopsy, a healthcare professional removes a small sample of tissue suspected to be cancerous for microscopic review. You may need multiple biopsies from different areas of your body to assess the extent of MCL.
Antigen testing, which is part of an immunohistochemistry workup, uses cell samples from your biopsies to screen for specific antigens associated with MCL, such as CD5, CD19, CD20, and CD22.
Antigens are markers on the surface of cells that uniquely identify a cell for your immune system.
Lastly, genetic testing plays an important role in diagnosing MCL. Using biopsy tissue, your doctor can look for a specific genetic translocation featured in this condition.
A genetic translocation occurs when a section of a chromosome’s genetic material breaks off and attaches to a different chromosome.
The genetic translocation associated with MCL is specific to the CCND1 gene, though other genetic markers can support the diagnosis.
Doctors will assign MCL a stage that indicates the disease progression and course.
Staging for MCL has four categories:
- Stage I: early, localized cancer in a single area
- Stage II: locally advanced cancer that involves more than one lymph node or organ on one side of your diaphragm
- Stage III: advanced cancer involving multiple lymphatic regions and possibly your spleen or other organs
- Stage IV: widespread lymphoma
In addition to the stages above, MCL is subdivided into categories by symptom presence and organ involvement, such as:
- A: no systemic symptoms
- B: drenching night sweats, unexplained fever, or unexplained weight loss
- E: one non-lymphatic organ involved
- S: lymphoma affecting your spleen
- X: large masses of lymphoma detected
After you receive a diagnosis from your doctor, the next steps will be set in motion. Your doctor will recommend a course of action and may take the initiative to schedule procedures or testing for you.
If you’re feeling overwhelmed, ask a family member or friend to help advocate for you throughout the process. They can make phone calls for you, come to clinic visits and treatment appointments, and take notes if you’re finding it challenging to process all the information at once.
While you wait for the next steps, learning more about MCL can help you feel more in control of what’s to come. You’ll understand why certain tests are necessary and what the terminology your doctor uses means.
You can also take general steps toward improving your overall health and wellness. The stronger your body is when you begin treatment, the better you’ll be able to manage symptoms and side effects.
Because the treatment of MCL can be quite involved, a second opinion to confirm the diagnosis may be helpful.
Your treatment plan for MCL will depend on many individual factors, such as your age, your overall health, and how far your lymphoma has advanced.
For some people with early stage MCL and no symptoms, doctors may recommend waiting on active treatment to help preserve your quality of life as long as possible. Medications used to treat MCL can have many negative side effects.
In more advanced cases, MCL is treated using:
- stem cell therapy
Immunotherapy involves medications, such as rituximab, that target and destroy cells with CD20 antigens, including MCL cells.
It’s typically used in conjunction with chemotherapy agents that are administered intravenously (directly into a vein) or taken by mouth. If MCL is widespread, medications may be injected directly into the fluid around your spinal cord.
Stem cell therapy in MCL involves taking your own previously collected stem cells or donor stem cells and transplanting them into your body to help restore your immune system after chemotherapy treatments.
According to a 2017 review, once you enter remission, maintenance immunotherapy with rituximab typically continues for several years.
Mantle cell lymphoma is a rare form of B-cell non-Hodgkin’s lymphoma. Doctors can definitively diagnose it through genetic screening, antigen testing, and review of biopsy samples.
MCL is often an aggressive form of cancer, but early diagnosis can help improve your longevity and overall quality of life.
To learn more about MCL, find clinical trials, or review treatments, you can visit the following sites: