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Primary mediastinal B-cell lymphoma (PMBCL) is a rare type of non-Hodgkins lymphoma that originates in the mediastinum, the area in the chest between the lungs. Because PMBCL is uncommon, there is no standardized treatment approach. The most commonly used treatment for PMBCL is chemotherapy, often followed by radiation therapy. However, targeted therapies and immunotherapies are also being explored as potential treatments. The choice of treatment depends on various factors, including the patient's overall health, the stage of the disease, and the patient's personal preferences.

1. Chemotherapy

Chemotherapy is the most common form of treatment for PMBCL. It involves the use of drugs to kill cancer cells or stop them from growing. The most commonly used regimen is R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone). This regimen is typically given every 21 days for 6 cycles. Another regimen that may be used is DA-EPOCH-R (dose-adjusted etoposide, prednisone, vincristine, cyclophosphamide, doxorubicin, and rituximab), which is given every 21 days for 6-8 cycles.

2. Radiation Therapy

After chemotherapy, some patients may receive radiation therapy to the chest. This treatment uses high-energy X-rays or other types of radiation to kill cancer cells or keep them from growing. The decision to use radiation therapy depends on the patient's response to chemotherapy and the presence of residual disease.

3. Immunotherapy

Immunotherapy is a treatment that uses the patient's immune system to fight cancer. For PMBCL, the immunotherapy drugs pembrolizumab (Keytruda) and nivolumab (Opdivo) may be used. These drugs are known as PD-1 inhibitors, and they work by blocking a pathway that prevents the immune system from attacking cancer cells. Rituximab (Rituxan) is often used in combination with chemotherapy. This drug is a monoclonal antibody that targets a protein called CD20, which is found on the surface of B cells, including the cancerous cells in PMBCL.

4. Stem Cell Transplant

In some cases, an autologous stem cell transplant may be considered for patients with PMBCL, especially those who have relapsed or refractory disease. This treatment involves the use of high-dose chemotherapy, sometimes combined with radiation therapy, to destroy the patient's bone marrow. Then, healthy stem cells are infused into the patient's body to replace the bone marrow and restore the production of blood cells.

5. Clinical Trials

Clinical trials are not just a last resort for treating PMBCL. They can actually provide access to cutting-edge treatments that are not yet approved but show promise over years of testing. The choice of a clinical trial depends on a variety of factors including the patient's age, overall health, the subtype of PMBCL, and genetic changes in the lymphoma cells. It's important to discuss all treatment options, including goals and possible side effects, with your healthcare team to help make the best decision. 

To learn more about your clinical trial options, visit HealthTree's PMBCL Clinical Trial Finder. Utilize the advanced filter option to locate trials that you are eligible to participate in. You can create a HealthTree account to favorite your top trial options so you can discuss them with your specialist.

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