Extranodal NK T Cell Lymphoma

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Extranodal NK T Cell Lymphoma
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Overview

Extranodal NK T-cell lymphoma is a rare, aggressive type of non-Hodgkin lymphoma that can start in T cells, but develops most often in natural killer cells. It is seen more commonly in Asia, Central America and South America than in North America. It affects men more often than women. The average age at diagnosis of Extranodal NK T-cell lymphoma is 60. It represents about 75% of all nasal lymphomas. It is strongly associated with the Epstein-Barr virus infection, especially in people of Asian ancestry. It is labeled as extranodal type of lymphoma because it develops in organs or tissues other than the lymph nodes. It usually affects the nose, most often the areas of nasal passages, the paranasal sinuses or the upper part of the throat. The lesion causes local destruction of cartilage, bone and soft tissues.

Extranodal NK T-cell lymphoma can also affect other organs or tissues. It may be called extranasal type when it affects skin, kidneys, testicles, soft tissues, brain or spinal cord, respiratory tract, eyes, adrenal glands and gastrointestinal tract.

Causes:

The mechanism of disease etiology and pathogenesis remains unclear for Extranodal NK T cell Lymphoma, and studies are needed to explore underlying molecular pathology and etiological factors. Epstein-Barr virus (EBV) is found in most cases of Extranodal NK T cell Lymphoma, suggesting an oncogenic role, but patients display biclonal or polyclonal populations of malignant cells. Persons may be having genetic predisposition to develop the disease in association with environmental factors.

Signs and symptoms:

Extranodal NK T-cell lymphoma may give rise to blockage in the nose, swelling of the face, discharge from the nose and nosebleeds. Other prominent signs and symptoms can include unexplained fever, drenching night sweats, hepatomegaly (abnormally large liver), weakness, fatigue, dizziness, pancytopenia (lower than normal number of RBCs (red blood cells), WBCs (white blood cells) and platelets in the blood) and unexplained weight loss. Perforation of nasal septum has been reported in 40% of cases.

Diagnosis:

A diagnosis of Extranodal NK T-cell lymphoma requires a thorough medical history, careful clinical examination, imaging studies, biopsy of tumor tissue, and a bone marrow biopsy. Imaging studies, such as CT scan, PET scan, MRI scan can be performed to assess the type and extent of lesion with spread of the tumor. Biopsy is followed by histopathological examination of the lesion, which typically exhibits pleomorphic cellular picture with many large or immunoblast-like cells and only few lymphocytes. Angiocentric distribution of the tumor cells and angiodestruction is a characteristic feature.

Management:

Extranodal NK T-cell lymphoma is usually treated with radiation therapy and chemotherapy & the type of treatment will depend on the site and type of disease.

When the disease is confined to the nose, external beam radiation therapy can be performed alone. Chemotherapy may be given after radiation therapy, and sometimes, it may be given before radiation therapy in some cases. mANY Combinations of chemotherapy drugs may be used to treat extranodal NK T-cell lymphoma, such as;

L-asparaginase, etoposide, ifosfamide, cisplatin and dexamethasone

VIPD: etoposide, ifosfamide, carboplatin and dexamethasone

AspaMetDex: L-asparaginase, methotrexate and dexamethasone

SMILE: dexamethasone, methotrexate, ifosfamide, L-asparaginase and etoposide

Chemoradiation combines chemotherapy with radiation therapy in which chemotherapy is given during the same time period as radiation therapy. Chemoradiation is used to treat extranodal NK T-cell lymphoma when it is at an advanced stage. Extranodal NK T-cell lymphomathat starts in the testicles or paranasal sinuses can spread to the CNS. CNS prophylaxis aimed at preventing the lymphoma cells from entering the brain or spinal cord. CNS prophylaxis is given in the form of intrathecal chemotherapy, which means injection of chemotherapy medicine directly into the cerebrospinal fluid (CSF). Methotrexate is the most commonly used drug for CNS prophylaxis.