Extranodal natural killer (NK)/T-cell lymphoma is a rare and aggressive form of non-Hodgkin lymphoma. It is characterized by the proliferation of NK cells or, less commonly, T-cells, usually in extranodal sites, including the nasal cavity, skin, gastrointestinal tract, and testes. The treatment of this disease is challenging due to its aggressive nature and resistance to conventional chemotherapy. However, several treatment options have been explored, including chemotherapy, radiotherapy, stem cell transplantation, and targeted therapies. The choice of treatment depends on the stage of the disease, the patient's overall health, and the specific characteristics of the lymphoma.

1. Chemotherapy

Chemotherapy is often the first line of treatment for extranodal NK/T-cell lymphoma. The most commonly used regimen is called SMILE, which stands for Steroid (dexamethasone), Methotrexate, Ifosfamide, L-asparaginase, and Etoposide. This regimen has shown promising results in terms of response rate and overall survival. However, it is associated with significant toxicity, including myelosuppression and infection.

2. Radiotherapy

Radiotherapy is often used in combination with chemotherapy, especially for localized disease. It can be very effective in controlling the disease in the irradiated area. However, it can also cause significant side effects, including skin changes, fatigue, and long-term risks of secondary cancers.

3. Stem Cell Transplantation

Stem cell transplantation, either autologous (using the patient's own stem cells) or allogeneic (using stem cells from a donor), is considered for patients with refractory or relapsed disease. This treatment can potentially cure the disease, but it is associated with significant risks, including graft-versus-host disease, infection, and organ damage.

4. Targeted Therapies

Targeted therapies are a newer type of treatment that targets specific characteristics of the lymphoma cells. Examples include monoclonal antibodies (such as rituximab), proteasome inhibitors (such as bortezomib), and immune checkpoint inhibitors (such as pembrolizumab). These treatments can be effective, especially in patients who have not responded to other treatments, but they can also cause side effects, including immune-related adverse events.

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