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Extranodal NK/T-cell lymphoma, nasal type, is a rare type of non-Hodgkin lymphoma. It is more common in Asian and Central- and South–American countries than it is in North America. Extranodal NK/T-cell lymphoma, nasal type, can develop in either T cells or natural killer (NK) cells, but most often in the NK cells. Natural killer cells are a type of lymphocyte that are closely related to T cells and attack foreign cells. Sometimes it is difficult to tell which cells, T cells or NK cells, are present. Extranodal NK/T-cell lymphoma, nasal type, used to be called angiocentric T-cell lymphoma.
Extranodal NK/T-cell lymphoma, nasal type, occurs in all age groups. However, it seems to occur more often in people in their 50s and affects more men than women. It is strongly linked to the Epstein-Barr virus, especially in people of Asian ancestry.
Because this type of lymphoma occurs in organs or tissues other than the lymph nodes, it is called extranodal. Extranodal NK/T-cell lymphoma, nasal type, most commonly affects the nose or nasal passages and paranasal sinuses. It can cause swelling of the face, discharge from the nose, nose bleeds (epistaxis) and blockage of the nasal passages (obstruction).
Extranodal NK/T-cell lymphoma can also affect other organs or tissues besides the nose and may be referred to as extranasal type. It can affect the:
Late in the disease, people with extranodal NK/T-cell lymphoma, nasal type, often develop a very serious condition where there is uncontrolled activation of certain parts of the immune system (hemophagocytic syndrome). This condition results in fever, an enlarged liver and spleen (hepatosplenomegaly), and a lower number of red blood cells, white blood cells and platelets in the blood (pancytopenia).
Extranodal NK/T-cell lymphoma is often a fast-growing (aggressive) lymphoma. The prognosis for people with this type of lymphoma is often poor and the risk for relapse is high. People with extranodal NK/T-cell lymphoma confined to the nose or nasal passages have a better prognosis than those people with more widespread disease.
Treatment of extranodal NK/T-cell lymphoma, nasal type, is generally external beam radiation therapy, often combined with chemotherapy.
If extranodal NK/T-cell lymphoma is confined to the nose or nasal passages, external beam radiation therapy is used.
Chemotherapy is often added to radiation therapy to treat extranodal NK/T-cell lymphoma. One of the combinations of chemotherapy drugs used is CHOP:
If extranodal NK/T-cell lymphoma is more widespread, then chemotherapy may be more intense and higher doses may be used.
There is a risk that lymphomas involving the paranasal sinuses or testicles can spread to the central nervous system (CNS). CNS prophylaxis is used to try to prevent the cancer cells from entering the brain or spinal cord. Usually, this involves giving chemotherapy drugs into the cerebrospinal fluid (intrathecal chemotherapy). Methotrexate is the drug used most often for CNS prophylaxis.
A stem cell transplant (SCT) may be offered to some people with extranodal NK/T-cell lymphoma who relapse after initial treatment.
For more detailed information on specific drugs, go to sources of drug information.
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