Mantle cell lymphoma is a cancerous disease that affects white blood cells that are used to fight off infections in the body. Often referred to as a form of non-Hodgkin's lymphoma, this cancer affects the body's lymphocytes that are a specific type of white blood cell. They are found in lymph nodes, the small glands in the groin, neck, armpits and other locations that work with the body's immune system.
The condition causes abnormalities in B-cell lymphocytes to develop into cancerous cells meaning may multiply at a rapid pace and grow out of control. Usually, cancer growth begins the formation of a tumor in a lymph node and enters the bloodstream to quickly spread to other left notes at distant sites. Mantle cell lymphoma can also affect bone marrow, the liver, spleen, and digestive tract.
The condition is difficult to diagnose and can often spread to other areas of the body before the doctor understands what is happening. Many forms of mantle cell lymphoma cannot be cured, however, with treatment and support, many patients live a long and healthy life.
- Who Is at Risk for Mantle Cell Lymphoma?
- What Causes Mantle Cell Lymphoma?
- Common Symptoms
- Diagnosing Mantle Cell Lymphoma
- Treating the Condition
Who Is at Risk for Mantle Cell Lymphoma?
Mantle cell lymphoma is a rare condition that affects approximately five percent of people who are diagnosed with some form of non-Hodgkin's lymphoma. While anyone can develop mantle cell lymphoma, there are certain factors that tend to affect some individuals more than others. The most common known risk factors associated with mantle cell lymphoma include:
- Gender – Men parked twice is likely to develop mantle cell lymphoma compared to women.
- Age – The elderly, especially those 65 years and older, are more likely to develop cancer compared to younger men and women.
What Causes Mantle Cell Lymphoma?
Approximately 85% of all cases of mantle cell lymphoma prove to have a characteristic genetic lesion (reciprocal translocation) that involves chromosomal 11 and chromosome 14. The mutation triggers the body to release cyclin D1 that manages cell growth. The defect causes and uncontrollable growth of certain forms of B-cells that eventually produced mantle cell lymphoma. Doctors do not yet understand why this happens however, there are probable causes that include:
- An issue would be immune system
- Suffering from specific infections
- Receiving previous cancer treatments
- Genetic predisposition where a relative has developed lymphoma in the past
Lymphoma diseases are not infectious conditions meaning they cannot be transmitted or passed on to another individual. The condition is also not inherited or passed down between generations. That said, having a parent, sister or brother who suffers from blood cancer gives the patient a slightly increased risk of developing lymphoma (approximately twice the potential).
The symptoms involved with mantle cell lymphoma often present themselves in a similar fashion to other forms of non-Hodgkin's lymphoma. These include:
- Unexpected weight loss
- Loss of appetite
- Vomiting or nausea
- Night sweats
- Belly bloating
- A sense of feeling full
- Discomfort caused by enlarged tonsils, enlarged spleen, or enlarged liver
- Abdominal pain
- Swollen lymph nodes in the groin or armpits
- Pain or pressure occurring in the lower back that travels to one or both legs
If the disease progresses, it can produce serious complications that involve:
- Low Blood Cell Count that could involve anemia (low red blood cell count), neutropenia (low white blood cell count), thrombocytopenia (low platelet count) because the growing abnormal lymphoma cells in the body's bone marrow are multiplying faster than healthy blood cells.
- Central nervous system, pulmonary, or gastrointestinal complications caused by extranodal issues that occur in the organs of the body.
- High white blood cell count (leukocytosis) that occurs is the disease progresses into the peripheral bloodstream (veins and arteries) that eventually produce leukemia.
Diagnosing Mantle Cell Lymphoma
Diagnosing mantle cell lymphoma requires a biopsy involving the removal of an enlarged lymph node that can be examined under the microscope. The biopsy is usually obtained the patient is under a general or local anesthetic. However, the doctor will also perform a comprehensive physical examination and gather a medical history to determine if there are other factors that could help diagnose the condition. These include:
- Comprehensive blood tests that can accurately account the number of blood cells in the body and how well the liver or kidneys are functioning or determine if certain proteins in the bloodstream associated with mantle cell lymphoma are present.
- X-rays and MRI/CT scans to capture detailed imaging of the inside of the body.
- PET scan that requires the use of radioactive material to detect any sign of cancer.
- Lumbar puncture (spinal tap) to examine the body's cerebrospinal fluid that protects a spinal cord and the brain.
- Bone marrow samples that can be analyzed and evaluated under a microscope to identify cancer cells or noticeable cell changes associated with mantle cell lymphoma.
The test results can assist the doctor in validating a diagnosis for lymphoma and determine if it has metastasized (spread) throughout the body.
Only a trained a hematopathologist can determine if the mantle cell lymphoma is a usual form or rare blastoid variant that are identified with vigor cell formations that grow and divide at a rapid aggressive pace. The variant form is extremely difficult to treat. It is not often identified in a diagnosis and can emerge over time.Staging the Disease
After making an accurate diagnosis, the doctor must stage the disease before formulating an effective treatment to cure or manage the condition. The staging will determine how far the disease has progressed and whether it is metastasized (spread) from its original location to other sites in the body.
Staging can assist the doctor in making a prognosis and determine the future course of a progressive disease and the patient's chance of survival. Staging also allows the doctor to tailor their treatment to meet the individual patient's needs and minimize any potential effects associated with treatment options.
To stage the disease, the doctor will recommend a complete blood cell count, obtain a biopsy of bone marrow through aspiration, collect imaging studies to determine the presence of the disease in deep lymph nodes, spleen, liver and other areas of the body, and review studies that describe the patient's protein levels in the blood system to measure markers associated with the disease's progression and extent throughout the body.
Treating the Condition
Mantle cell lymphoma is recognized as a fast-growing (aggressive) form of B-cell non-Hodgkin lymphoma. Most patients with the disease will receive treatment after they have been diagnosed with the condition. However, there are individuals that have indolent (slow-growing) mantle cell lymphoma which requires only "watchful waiting" to see if the disease progresses over time.
The doctor may recommend one or more treatments that include:
- Chemotherapy that uses chemical medicines given orally or intravenously that destroy cancer cells.
- Immunotherapy that uses medications to stimulate the body's immune system so it will recognize and kill cancer cells.
- Targeted Therapy that uses drugs to block proteins that allow cancer cells to survive and metastasized throughout the body.
In recent years, doctors have initiated stem cell transplant treatments used in conjunction with high-dose chemotherapy to cure or manage the disease.