Extranodal NK/T-cell lymphoma, nasal type

Extranodal NK/T-cell lymphoma, nasal type, is a rare, fast-growing (aggressive) type of non-Hodgkin lymphoma (NHL). It can start in T cells, but it develops most often in natural killer (NK) cells.

Extranodal NK/T-cell lymphoma, nasal type, is more common in Asia, Central America and South America than it is in North America. It occurs more often in men than women. The average age at diagnosis is 60. It is strongly linked to the Epstein-Barr virus (EBV), especially in people of Asian ancestry.

This type of NHL is called extranodal because it develops in organs or tissues other than the lymph nodes. It usually affects areas in the nose, most often the nasal passages. It can also affect the paranasal sinuses or the upper part of the throat.

Extranodal NK/T-cell lymphoma, nasal type, can also affect other organs or tissues. It may be called extranasal type when it affects the:

  • skin
  • testicles
  • soft tissues
  • kidneys
  • brain or spinal cord (called the central nervous system, or CNS)
  • respiratory tract
  • gastrointestinal (GI) tract
  • eyes
  • adrenal glands

Symptoms

Most people are diagnosed with extranodal NK/T-cell lymphoma, nasal type, at an early stage (stage 1 or 2) when it causes a blockage in the nose. This disease can also cause swelling of the face, discharge from the nose and nosebleeds. It doesn’t usually cause B symptoms (unexplained fever, drenching night sweats and unexplained weight loss).

People with extranodal NK/T-cell lymphoma, nasal type, often develop a serious condition called hemophagocytic syndrome. It causes certain parts of the immune system to become active. This condition results in:

  • fever
  • a larger than normal liver
  • a lower than normal number of red blood cells, white blood cells and platelets in the blood (called pancytopenia)

Treatments

Extranodal NK/T-cell lymphoma, nasal type, is usually treated with radiation therapy and chemotherapy. Treatments for the extranasal type will depend on where it develops.

Radiation therapy

Extranodal NK/T-cell lymphoma, nasal type that is only in the nose is treated with external beam radiation therapy.

Chemotherapy

Chemotherapy is often given after radiation therapy to treat extranodal NK/T-cell lymphoma, nasal type. It may be given before radiation therapy in some cases.

The following combinations of chemotherapy drugs may be used to treat extranodal NK/T-cell lymphoma, nasal type:

  • L-asparaginase (Kidrolase), etoposide (Vepesid, VP-16), ifosfamide (Ifex), cisplatin (Platinol AQ) and dexamethasone (Decadron, Dexasone)
  • VIPD – etoposide, ifosfamide, carboplatin (Paraplatin, Paraplatin AQ) and dexamethasone
  • SMILE – dexamethasone, methotrexate, ifosfamide, L-asparaginase and etoposide
  • AspaMetDex – L-asparaginase, methotrexate and dexamethasone

Chemoradiation

Chemoradiation is treatment that combines chemotherapy with radiation therapy. Chemotherapy is given during the same time period as radiation therapy. Some types of chemotherapy make radiation therapy more effective.

Chemoradiation is used to treat extranodal NK/T-cell lymphoma, nasal type, when it is at an advanced stage or it is stage I with poor risk factors.

Central nervous system prophylaxis

The central nervous system (CNS) is the brain and spinal cord. Extranodal NK/T-cell lymphoma, nasal type that starts in the paranasal sinuses or testicles can spread to the CNS. CNS prophylaxis is used to try to prevent the lymphoma cells from entering the brain or spinal cord.

CNS prophylaxis is usually given as intrathecal chemotherapy. This means that the chemotherapy drug is injected directly into the cerebrospinal fluid (CSF). Methotrexate is the drug used most often for CNS prophylaxis.

Expert review and references

  • American Cancer Society. Non-Hodgkin Lymphoma. 2014: http://www.cancer.org/acs/groups/cid/documents/webcontent/003126-pdf.pdf.
  • American Society of Clinical Oncology. Lymphoma Non-Hodgkin Overview. 2014: http://www.cancer.net/cancer-types/lymphoma-non-hodgkin/view-all.
  • Freedman AS, Jacobson CA, Mauch P, Aster JC . Non-Hodgkin lymphoma. DeVita VT Jr, Lawrence TS, & Rosenberg SA. Cancer: Principles and Practice of Oncology. 10th ed. Philadelphia: Wolters Kluwer Health/Lippincott Williams & Wilkins; 2015: 103:1552-1583.
  • Medeiros L J . Pathology of non-Hodgkin's and Hodgkin's lymphomas. Wiernik PH, Goldman JM, Dutcher JP, Kyle RA (eds.). Neoplastic Diseases of the Blood. 5th ed. Springer; 2013: 42: 867-918.