Mantle Cell Lymphomas - Symptoms, Risk Factors, Diagnosis, and Treatment

Introduction:

These are seen as diseases occurring in the lymph nodes, spleen, bone marrow cells, and in gastrointestinal tract region. Mantle cell lymphomas are an aggressive form of lymphoma and are difficult to cure. Non-Hodgkin’s lymphomas are the most common mantle cell lymphoma. These are B-cell and aggressive forms of lymphoma. Mantle cell lymphomas constitute six percent of Non-Hodgkin’s lymphomas. Most patients affected by this condition are young individuals sensitive to chemotherapy. The average survival rate of this disease is one to two years.

How Does Mantle Cell Lymphoma Occur?

Mantle cell lymphoma is cancer occurring in the blood cells of lymph nodes. They grow rapidly, making the treatment incurable. This is circulated throughout the body through the lymphatic system, making the metastasis rate high. The Cancer spreads to organs like the digestive system and lymph nodes.

How Does Mantle Cell Lymphoma Appear Under Microscopy?

• Mantle cell lymphoma involves the lymphatic system showing a nodular pattern.

• They show diffused structures.

• The tumor cells appear larger.

• The nuclei appear irregular, penetrating, and dull in appearance.

• These large tumor cells resemble lymphoblasts.

• It involves bone marrow cells in most cases and rarely presents as a leukemic condition.

• They involve the gastrointestinal region, leading to polyps known as lymphomatoid polyposis.

Where Does Mantle Cell Lymphoma Occur in the Body?

Mantle cell lymphomas coexist within the surfaces of IgM and IgD, the pan B-cell antigens CD19, CD20, CD22, and CD5 antigens. They are present in areas with a high level of cyclin D1 protein. It shows immunophenotype (CD20 bright with absent CD23) and fluorescence in situ hybridization (FISH) cytogenetics demonstrated at t(11;14) are crucial in the diagnosis of mantle cell lymphoma. Certain deletions of chromosomes, like the 13q and 17p, can also be seen in some cases.

What Are the Symptoms Associated With Mantle Cell Lymphoma?

Patients experience symptoms such as:

• Generalized weakness.

• Lymphadenopathy (where small immune glands known as lymph nodes get swollen in one localized area of infection or multiple areas).

• Enlarged spleen and liver.

• Diseases of the gastrointestinal region.

• Bone marrow conditions like infiltration of tumor cells.

• Small polyps in the bowel.

• Fever.

• Night sweats.

• Weight loss.

• Swollen lymph nodes in the arm, neck, and groin areas.

• Bloating and heartburn.

What Are the Risk Factors for Developing Mantle Cell Lymphoma?

Certain factors are crucial in the development of mantle cell lymphoma, such as:

• It affects the older generation.

• It affects men more than women.

• They are the subtypes of non-Hodgkin's lymphoma.

• Positive for family history of lymphoma.

How Does Mantle Cell Lymphoma Arise in the Body?

Mantle cell lymphoma (MCL) arises from the pre-germinal center of B-cells. The abnormal multiplication of B-cells gives way to cyclin D1 protein receptors which help B-cells to grow. Such an overload of cells gives it the tendency to duplicate abnormally. They cause the rearrangement of light and heavy chain genes. Such translocations can be observed at t(11;14) q (13;32) genes. Half of the patients suffering from MCL show translocations at the t (11;14) gene. Translocation on chromosome 14 and overexpression of cyclin D1 are also seen. Abnormalities in cyclic reactions are also observed in the p16, p17, and p27 cycles. Many cytogenetic abnormalities with frequent mutations involving p53 are also seen.

What Are the Tests Done to Detect Mantle Cell Lymphoma?

Depending on the symptoms, various tests can be conducted to confirm the diagnosis, such as:

• Complete Blood Count: All the sub-particles in the blood are calculated. Any variation in a number other than the usual count is a red flag.

• Lactate Dehydrogenase Test (LDT): This is done to check the tissue damage caused due to any disease.

• Comprehensive Metabolic Panel (CMP): This is done to check kidney and liver function. The normal electrolyte concentration in the blood is checked as electrolyte balance is important for neuro and muscular function.

• Uric Acid Level Test: The uric acid in the blood is checked for any dead immune cells.

• Bone Marrow Aspiration Biopsy: A tissue sample is collected to study for any malignancies.

• Lymph Node Biopsy: This is done to confirm the presence of cancer.

• Computed Tomography (CT): This is a diagnostic scan to visualize the internal structures.

• Positron Emission Tomography (PET): This produces images of internal organs and tissues at work.

• Esophagogastroduodenoscopy (EGD): This is a rapidly spreading condition. Hence, its spread into the stomach areas is evaluated.

• Colonoscopy: The colon and rectum areas are checked for the presence of cancer cells.

How Can We Undertake Treatment for Mantle Cell Lymphoma?

For the treatment to start, the disease should manifest some symptoms. The effect of treatment depends upon its staging. Here are some treatment options:

• Chemotherapy: Chemicals used in the form of drugs to kill cancer cells. Ibrutinib, Bortezomib, Lenalidomide, and Bendamustine are administered. These drugs are mainly used against relapse conditions.

• Monoclonal Antibody Therapy: Antibodies are created in the laboratory to kill these cancer-causing cells.

• Immunotherapy: This is done by boosting the immune system, such as CAR T-cell therapy.

• Autologous Stem Cell Transplantation: Stem cells from a donor are injected into the patient. There is a high chance of recovery in this procedure. For younger patients who do not have other medical history, allergens of reduced-intensity stem cell transplantation are done.

• Disorder Affecting Central Nervous System: In such patients, drugs with high-dose Methotrexate or Cytarabine-based regimens are given to penetrate the central nervous system with or without intrathecal chemotherapy for leptomeningeal involvement. If the patient responds positively to this treatment, stem cell transplantation can proceed.

Prognosis of Mantle Cell Lymphoma:

The prognosis of lymphoma depends on factors like age, stage, overall health, and response to the treatment. Generally, the prognosis for mantle cell lymphoma is very poor. Its fast-growing, aggressive nature and lack of cure make it untreatable. Treatment might seem to be progressive. But after the treatment also, it has a high tendency to relapse and can occur at later stages of life. The survival rate of mantle cell lymphoma is two to nine years after the diagnosis.

Conclusion:

Lymphomas are solid tumors of the immune system. Understanding gene mutations are very important in formulating the treatment plan and its outcome. The reason for such genetic mutations is still unknown. Hence, nothing can be done as a preventive mechanism to control its onset.