Characteristics
Extranodal NK/T-cell lymphoma is a rare and aggressive type of non-Hodgkin lymphoma that develops from two types of white blood cells: natural killer (NK) cells and cytotoxic T-cells. As indicated by the word ‘extranodal’, this lymphoma grows outside of the lymphatic system. In fact, It usually occurs inside the nose, the sinuses, and in the upper airway (nasal type). However, it can also occur in the skin, digestive tract, or other organs (extranasal type)
Extranodal NK/T-cell lymphoma (nasal type) is more common in people with an Asian origin and in people coming from Central or South America, while it is very rare in the Western world. As is the case for several other lymphoma subtypes, extranodal NK/T-cell is associated with an infection by the Epstein-Barr virus, the virus that causes infectious mononucleosis.
For professionals
Uitklappen
Symptoms
People who have extranodal NK/T-cell lymphoma, nasal type, usually develop a fast-growing mass inside their nose or in the sinuses. As a result, patients develop a blocked nose and experience regular nose bleeds. In addition, this lymphoma type can cause a (painful) swelling of the face and weepy eyes as a result of pressure in the sinuses and nasal cavity.
Extranodal NK/T-cell lymphoma may also come with more universal lymphoma symptoms, such as swollen lymph nodes, B-symptoms (fever, night sweats and unexplained weight loss) and a general feeling of fatigue.
Depending on the location of the lymphoma in patients with an extranasal form of extranodal NK/T-cell lymphoma, patients may experience other symptoms. For example, when the skin is involved, patients may also develop a rash or lumps in the skin.
Diagnostic tests
The diagnosis of NK/T-cell lymphomas begins with a thorough medical history and a general physical exam. When a lymphoma is suspected, a biopsy of an affected area will be taken to confirm the presence of lymphoma cells and tests are performed to better characterize the genetic and molecular features of the lymphoma cells. In addition to this, medical imaging tests (e.g., PET, CT, MRI) are usually performed to assess the presence of lymphoma in other parts of the body.
Treatments
For patients with localized NK/T-cell lymphoma of the nose, radiotherapy is the most commonly used treatment modality. In a proportion of cases, this radiotherapy is combined with, or followed up by chemotherapy. If the disease is more advanced and has spread throughout the body, radiotherapy is no longer an option and chemotherapy is the standard of care. Of note, the chemotherapy regimens that are used for NK/T-cell lymphoma are different from the traditional regimens used other NHL subtypes (e.g., no anthracyclines, important place for L-asparaginase).
If the lymphoma comes back after therapy (relapse), or when patients do not respond to the treatment, alternative chemotherapy regimens can be used. For fit patients with relapsed/refractory disease, a stem cell transplantation can be considered. Sometimes alternative treatment options, such as targeted therapy, can be offered by participating in a clinical trial.
Want to submit input to improve quality content on this blood disorder?