Extranodal NK/T-Cell Lymphoma, Nasal type

Extranodal NK/T-Cell Lymphoma, Nasal type

Article
Ear, Nose, & Throat (ENT)
Diseases & Conditions
+3
Contributed byMaulik P. Purohit MD MPHMay 04, 2018

What are the other Names for this Condition? (Also known as/Synonyms)

  • Angiocentric T-Cell Lymphoma (Extranodal NK/T Cell Lymphoma, Nasal type)
  • ENKL (Extranodal NK/T Cell Lymphoma, Nasal type)
  • Nasal-type NK/T Cell Lymphoma

What is Extranodal NK/T-Cell Lymphoma, Nasal type? (Definition/Background Information)

  • Extranodal NK/T Cell Lymphoma, Nasal type (ENKL) is a rare type of ‘extranodal lymphoma’ that usually develops in the nose, nasal passage, and paranasal sinuses. This is the reason why it is named “nasal type,” though it may arise at other locations too
  • Extranodal lymphomas develop outside of the lymph nodes and can be found in body tissues, like the skin, tonsils, brain, bowels, and bone
  • Lymphoma is a type of blood cancer stemming from uncontrollably dividing lymphocytes (type of white blood cells). There are two types of lymphomas:
    • Hodgkin Lymphoma
    • Non-Hodgkin Lymphoma
  • NK/T Cell Lymphoma is a type of non-Hodgkin lymphoma. They are differentiated by detecting the type of proteins expressed on the surface of the cancer cells, and by the way they look under a microscope, when examined by a pathologist
  • Lymphocytes are a class of white blood cells that are part of the lymphatic system. Lymphocytes are made in bone marrow, and can develop into either B-cells or T-cells. There is also another class of lymphocytes called natural killer cells (NK cells); NK/T cells are a type of T cells. An NK/T Cell Lymphoma arises from cancerous T-cells
  • Normally, lymphocytes help generate an immune response to infections. These cells can recognize a wide variety of foreign invaders. They can also remember them and respond accordingly, if the body is infected with the same bacteria/virus ever again
  • The lymphatic system is responsible for immunity to diseases. The organs of the lymphatic system are lymph nodes, bone marrow, the spleen, thymus, and tonsils. In children, the T cells are produced in the thymus. As an individual gets older, other parts of the lymphatic system are involved. At that point, they reside in the bone marrow and the thymus shrinks down, becoming inactive
  • T-cells are responsible for recognizing cells that are altered or infected, and it includes attempting to kill cells that are cancerous too
  • Common signs and symptoms of NK/T Cell Lymphoma, Nasal type are nasal bleeding and discharge, nasal blockage, weight loss, and excessive night sweats
  • The condition is generally treated with a combination of radiation therapy and chemotherapy. However, the prognosis for Extranodal NK/T Cell Lymphoma, Nasal type is very poor, with a high risk of relapse (return of the condition after initial treatment)

Who gets Extranodal NK/T-Cell Lymphoma, Nasal type? (Age and Sex Distribution)

  • Extranodal NK/T Cell Lymphoma, Nasal type accounts for 5-10% of non-Hodgkin lymphoma cases in Asia and central & south America; but, it is very rare in the United States
  • The condition is most common in adults over the age of 50 years. It is extremely rare in children
  • Males are much more likely to develop ENKL, than females
  • There is no specific ethnic or racial predisposition, but the condition is more commonly seen in Asia, central and south America

What are the Risk Factors for Extranodal NK/T-Cell Lymphoma, Nasal type? (Predisposing Factors)

Some of the risk factors associated with Extranodal NK/T Cell Lymphoma, Nasal type include:

  • Epstein-Barr virus (EBV) is associated with all diagnoses of NK/T Cell Lymphoma, Nasal type
  • Exposure to chemicals, such as pesticides and fertilizers, as well as smoking and diet, has been suggested as links to other forms of non-Hodgkin lymphoma

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases one’s chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Extranodal NK/T-Cell Lymphoma, Nasal type? (Etiology)

  • The exact cause of Extranodal NK/T Cell Lymphoma, Nasal type is not completely understood
  • However, it is observed that there is a universal correlation with being infected with the Epstein Barr virus; the virus that causes mononucleosis infection

What are the Signs and Symptoms of Extranodal NK/T-Cell Lymphoma, Nasal type?

Signs and symptoms of Extranodal NK/T-Cell Lymphoma, Nasal type include:

  • Facial swelling
  • Nasal bleeding and discharge, nasal blockage
  • Immune system dysfunction
  • Anemia (low red blood cell count)
  • Thrombocytopenia (low platelet counts)
  • Liver and spleen enlargement
  • Loss of appetite and weight loss
  • Fatigue
  • High temperatures and excessive night sweats
  • ‘Non-painful’ swelling of lymph nodes in the neck, armpits, or groin can be present

How is Extranodal NK/T-Cell Lymphoma, Nasal type Diagnosed?

It often takes multiple tests to diagnose lymphoma. It is critical that the specific sub-type of lymphoma be diagnosed correctly, in order to create a proper treatment plan. Extranodal NK/T Cell Lymphoma, Nasal type diagnosis involves:

  • A physical examination and a complete medical history by a doctor can help determine, if there is a risk of lymphoma
  • Biopsies of the nose or face at the site of involvement are critical in diagnosing ENKL
  • NK/T Cell Lymphoma usually does not involve the lymph nodes. Biopsies of lymph nodes that may be enlarged can be taken and examined in a lab, to determine if the cells are malignant or benign. The biopsies may be performed under general or local anesthesia. If necessary, an entire lymph node may be removed to help determine the sub-type of lymphoma
  • Blood tests to evaluate red and white blood cells, as well as platelet counts
  • Lumbar puncture to determine, if NK/T Cell Lymphoma, Nasal type involves the brain
  • Bone marrow biopsies may be performed, to determine if the bone marrow is involved
  • X-rays of chest and abdomen, to look for enlarged lymph nodes, liver, or spleen
  • Whole body CT-PET scan - to determine the spread of lymphoma, by determining the size and metabolic rate (a reflection of uncontrolled growth) of lymph nodes, throughout the body. This can also determine if the cancer has spread to other organ systems
  • If neurologic symptoms are present, then brain MRIs are used to determine, if cancer has spread to the brain, or to the tissue that covers the brain (meninges)

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Extranodal NK/T-Cell Lymphoma, Nasal type?

The possible complications from Extranodal NK/T-Cell Lymphoma, Nasal type include:

  • NK/T Cell Lymphoma, Nasal type is extremely aggressive and can spread to other locations including the skin, testicles, soft tissue, kidneys, brain, nervous system, respiratory tract, and the gastrointestinal tract
  • Treatments can cause secondary health problems, such as infections, secondary forms of cancer, and heart disease
  • There is a high risk of relapse and a low percent of long-term survival

How is Extranodal NK/T-Cell Lymphoma, Nasal type Treated?

Once a definitive diagnosis of NK/T Cell Lymphoma, Nasal type has been made, staging is performed to determine how far the cancer has spread. The stage can describe:

  • How many lymph nodes are affected (if any)
  • Their locations in the body
  • And, if other organs are being affected

Staging is important because different treatment regimens are necessary, depending on the progression of the lymphoma.

  • Stage 1E: Single extranodal site involvement (generally the nose or nasal passage), and there may or may not be extension into nearby structures
  • Stage 2E: In addition to stage 1 symptoms, there can be involvement of the lymph nodes and additional sites, including the head and neck
  • Stage 3E: This rare stage includes stage 1 symptoms, involvement of lymph nodes above and below the diaphragm, and spleen involvement may be observed
  • Stage 4: Stage 1 disease symptoms, involvement of other organs, including the liver; and involvement of the bone marrow

A combination of treatments is used to most effectively treat this cancer. The aggressiveness of the treatment is determined by the disease progression.

Chemotherapy: This approach uses a combination of drugs to kill the cancerous cells and is most commonly used for NK/T Cell Lymphoma, Nasal type that has spread beyond the nasal passages.

  • Commonly, the drug combination CHOP is used first, which includes doxorubicin, vincristine, cyclophosphamide, and prednisolone
  • There can be severe side effects including fatigue, nausea, hair loss, anemia, high risk of infection, and drug-specific reactions
  • Many T-cell lymphomas can be resistant to chemotherapy. It can also damage healthy cells
  • Chemotherapy can be administered as a pill, liquid, shot, or intravenously. Although, this may work initially, and then stop being effective
  • Chemotherapy can be administered prophylactically into the cerebral spinal fluid in the spinal cord, to prevent the spread of cancer to the central nervous system

Radiation: Radiation therapy is the use of high-energy radiation waves, to kill cancer cells by destroying their DNA.

  • This treatment is commonly used, when ENKL is confined to the nasal passages and can be used in combination with chemotherapy
  • The radiation may be administered by a machine placed outside the body, or by putting a radioactive material inside the body. An external beam targeted at the nasal passages is most commonly used for NK/T Cell Lymphoma, Nasal type
  • The side effects of radiation therapy include nausea, vomiting, fatigue, pain, risk of (a different type of) cancer later in life, and a risk of heart disease
  • Radiation can damage healthy cells in addition to cancer cells, causing further complications

Stem cell transplant: Blood stem cell transplants from stem cells, harvested from a compatible donor, or from the patient before treatment, can be used to help treat ENKL. This is more frequently used, if the cancer has relapsed or does not respond to other treatment methods.

Supportive treatment: Steroids, blood transfusions, anti-nausea medications, and antibiotics, may also be used. In combination with other treatment measures, these can help combat the symptoms of immune deficiency.

Clinical trials: There are some new treatment options, currently on clinical trials that can be considered for some patients, depending on their risk factors.

How can Extranodal NK/T-Cell Lymphoma, Nasal type be Prevented?

  • Currently, there are no definitive ways to prevent Extranodal NK/T Cell Lymphoma, Nasal type
  • However, healthy diet and exercise, as well as avoidance of unnecessary exposure to chemicals, may help decrease its risk
  • Regular medical screening at periodic intervals with blood tests, scans, and physical examinations, are mandatory for those who have already endured the tumor, due to its metastasizing potential and possibility of recurrence. Often several years of active vigilance is necessary

What is the Prognosis of Extranodal NK/T-Cell Lymphoma, Nasal type? (Outcomes/Resolutions)

  • The prognosis of Extranodal NK/T Cell Lymphoma, Nasal type is generally poor. This particular type of cancer is an aggressive form of NK/T-cell Lymphoma, with a high risk of relapse
  • Without proper treatment of ENKL, the survival period is often no longer than a few months
  • With treatment, the progression of the cancer affects the prognosis. Over 60% of those with stage I diseases have a long-term remission; but those with late stage disease, often have a survival rate of a few years at the most

Additional and Relevant Useful Information for Extranodal NK/T-Cell Lymphoma, Nasal type:

T-cell lymphomas are less common than B-cell lymphomas.

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Maulik P. Purohit MD MPH picture
Approved by

Maulik P. Purohit MD MPH

Assistant Medical Director, Medical Editorial Board, DoveMed Team

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