Mantle cell lymphoma (MCL)
Mantle cell lymphoma (MCL) is a rare subtype of non-Hodgkin lymphoma that primarily impacts B lymphocytes within the lymphatic system. It is characterized by two forms: indolent, which progresses slowly, and aggressive, or high-grade, which advances more rapidly. MCL predominantly occurs in older adults, particularly white males, with most cases diagnosed in individuals in their sixties. The incidence of MCL has been rising since the 1970s, and unfortunately, many patients are diagnosed at an advanced stage, often Stage IV, where the lymphoma has spread beyond the lymph nodes.
Common symptoms of MCL include painless swelling of lymph nodes, fatigue, reduced appetite, fever, night sweats, weight loss, and breathlessness. Diagnosis typically involves surgical biopsy of the affected lymph nodes, and the presence of the cyclin D1 protein is a crucial indicator of MCL. Treatment strategies vary based on the disease stage and patient factors, but they often include aggressive therapies such as chemotherapy, radiation, immunotherapy, and sometimes allogeneic stem cell transplantation. The prognosis for MCL can vary widely, with average survival rates around three to four years, and curing the disease remains challenging.
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Subject Terms
Mantle cell lymphoma (MCL)
ALSO KNOWN AS: Non-Hodgkin lymphoma, B-cell lymphoma
RELATED CONDITIONS: Hodgkin lymphoma
DEFINITION: Mantle cell lymphoma (MCL), or B-cell lymphoma, is a rare form of non-Hodgkin lymphoma (NHL). MCL primarily affects the B lymphocytes of the lymphatic system. The lymphatic system is made up of lymph nodes linked by lymph vessels responsible for delivering fluid throughout the body, as well as collecting waste from tissues, purifying it, and returning it to the blood. MCL has two distinct groupsindolent or and aggressive or high-grade lymphoma.
Risk factors: There are no risk factors for MCL.
Etiology and the disease process: The causes of MCL have not been identified. However, it is known that White males are more susceptible to MCL, which is most prevalent in adult populations. It has been determined that patients with MCL have an overexpression of cyclin D1, a protein that encourages cellular growth. This cyclin D1 overexpression has been traced to a translocation between chromosomes 11 and 14.
Incidence: MCL is typically found in adults in their sixties, affecting males more often than females at a ratio of 3:1. Cases of MCL have been steadily increasing since the 1970s. As the initial symptoms of MCL often go undetected, upon diagnosis, the majority of patients already have Stage IV disease. Only around 10 percent of cases are diagnosed in the early stages. Around one in 200,000 people are diagnosed with MCL.
Symptoms: MCL is often detected because of painless swelling in the lymph nodes of the neck, groin, or armpit that is noticeable either visibly or to the touch. Accompanying symptoms often include fatigue, decreased appetite, fever, night sweating, weight loss, itchiness, and breathlessness.
Screening and diagnosis: MCL is diagnosed following surgical removal of a portion of the swollen lymph node. The tissue collected is sampled to review its cells under a microscope for diagnosis. The overproduction of the protein cyclin D1 is present in the lymphoma cells of 90 percent of MCL cases, making it a key diagnostic tool. Additional supportive tests may include blood tests, marrow biopsies, computed tomography scans, positron emission tomography scans, and X-rays to provide information on the extent of the spread of the lymphoma and its type. The information gained from these tests will assist the doctor in determining the appropriate treatment plan for the cancer.
Stage I is lymphoma limited to a primary lymph node, organ, or tissue site in one body area. Stage II includes two or more lymph nodes or regions of lymph nodes on the body's upper or lower half. Stage III includes two or more lymph nodes or regions of lymph nodes on both the upper and lower parts of the body, and Stage IV includes lymphoma that has spread from the lymph nodes to one or more organs in the body. Each stage also includes a subclassification of either A or B to identify the absence of symptoms (A) or the presence of symptoms (B). Healthcare providers use the Mantle Cell Prognostic Index (MCPI) to estimate the likelihood of MCL reoccurring. The MCPI is calculated using age, white blood cell count, speed of lymphoma cell division, lactate dehydrogenase levels, and the patient's ability to live an everyday life.
Treatment and therapy: Treatment for MCL depends on the patient’s stage of disease, its rate of growth, the patient's age, and TP53 gene mutations. As most patients with MCL are diagnosed at stage IV, aggressive therapy is often prescribed. Aggressive therapy can consist of chemotherapy alone. However, this is often combined with radiation, immunotherapy, or monoclonal antibody therapy. Allogenic stem cell transplantation has also been used to introduce stem cells that are not cancerous into the body to assist the body in its fight against cancer, but it is rarely used because of its severe side effects.
The patient may also be given medications to counteract the side effects caused by the first-line therapies, such as steroids to combat nausea or interferons to allow the body to increase its immune response.
Prognosis, prevention, and outcomes: Prognosis for MCL can be determined by its classification as either indolent or aggressive, and indolent MCL typically has a better prognosis than aggressive MCL. As the majority of cases of MCL are diagnosed as Stage IV, MCL has only a moderate prognosis. Patients diagnosed with MCL most often have an average survival of three to four years. Even in patients with a good or fair prognosis, curing MCL is uncommon.
Bibliography
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