Overview of nasal natural killer (NK) T-cell lymphoma
Peripheral T-cell lymphoma (PTCL) are a group of aggressive (fast growing) T-cell non-Hodgkin lymphomas (NHL). They account for 7% of all non-Hodgkin lymphoma (NHL) cases and 10% of all lymphoma cases.
Extranodal nasal natural killer (NK) T-cell lymphoma is a rare aggressive (fast growing) T-cell non-Hodgkin lymphoma (NHL). Extranodal means that it is located outside of the lymph nodes in other organs or tissue). They are a subtype of PTCL. It develops from natural killer (NK) cells (that are similar to T-cells) or cytotoxic T-cells. NK cells usually kill cancer cells that are infected with a virus. Cytotoxic cells usually kill cancer cells or cells infected by a virus, but only after they have been tagged by other cells in the immune system.
Nasal NK T-cell lymphoma can also involve other organs (extranodal) including the gastrointestinal tract and skin.
Who is affected by nasal NK T-cell lymphoma?
Nasal NK T-cell lymphoma is more common in Asia and Central and South America. It usually develops in people around the age of 50 to 60 years. It is more common in men than women.
It is strongly linked to an infection with a virus called Epstein-Barr virus (EBV), the virus that can cause glandular fever. After being infected with the virus it stays in the body but is normally kept under control by the immune system. If it is not kept under control, it can sometimes cause genetic changes in lymphocytes that might turn into lymphoma.
Symptoms of nasal NK T-cell lymphoma
Symptoms may affect the nose, eyes or face such as:
- Blocked nose
- Swelling of the face
- Weepy eyes
Patients may also experience other symptoms such as:
- Swollen lymph nodes
- B symptoms such as weight loss, drenching night sweats and fever
- Extreme fatigue
- Rash or red lumps if the skin is affected
Diagnosis nasal NK T-cell lymphoma
A biopsy is always required for a diagnosis of lymphomaNK T-cell. A biopsy is a surgical procedure to remove part of or all of an affected lymph node or other abnormal tissue to look at it under the microscope. The biopsy can be done under local or general anaesthetic depending on what part of the body is being biopsied. The biopsy can be one of three ways:
- Fine needle aspirate
- Core needle biopsy
- Excisional node biopsy
An excisional node biopsy is the best investigative option, as it collects the most adequate amount of tissue to be able to do the necessary testing for a diagnosis.
Waiting for test results can be a difficult time. It may help to talk to your family, friends or a specialist cancer nurse.
Once a diagnosis of NK T-cell lymphoma is made, further tests are required to see where else in the body the lymphoma is spread and how much of the body is affected. This is called staging.
There are four stages from stage 1 (lymphoma in one area) through to stage 4 (lymphoma that is widespread).
- Early stage means stage 1 and some stage 2 lymphoma. This may also be referred to as ‘localised’. Stage 1 or 2 means that the lymphoma is found in one area or a few areas close together.
- Advanced stage means the lymphoma is stage 3 and stage 4, and is widespread In most cases, the lymphoma has spread to parts of the body that are far from each other.
What is the ‘grade’ of lymphoma?
Once a diagnosis of lymphoma is made, further tests are required to see where else in the body the lymphoma is spread and how much of the body is affected. This is called staging.
Staging scans and tests
Lymphomas are also often grouped as either indolent or aggressive. Indolent lymphomas are usually slow growing and aggressive lymphomas are fast growing. The grade is also referred to as the clinical behaviour of the lymphoma.
Staging scans and tests
The scans and tests needed for staging and before treatment can start may include:
- Positron emission tomography (PET) scan
- Computed tomography (CT) scan
- Bone marrow biopsy
- Lumbar puncture & magnetic resonance imaging (MRI) – If lymphoma is suspected in the brain or spinal cord
Patients may also undergo a number of baseline tests prior to any treatment commencing to check organ functions. These are often repeated during and after the treatment has completed to assess whether the treatment has affected the functioning of organs. Sometimes the treatment and follow-up care may need to be adjusted to help manage side effects. These may include:
- Physical examination
- Vital observations (blood pressure, temperature, & pulse rate)
- Heart scan
- Kidney scan
- Breathing tests
- Blood tests
It may take some time for all the necessary biopsies and tests to be done (an average of 1-3 weeks), but it is important for the doctors to have a complete picture of the lymphoma and the general health of the patient in order to make the best treatment decisions
Many of the staging and organ function tests are done again after treatment to check whether the lymphoma treatment has worked and the effect this has had on the body.
Prognosis of nasal NK T-cell lymphoma
Nasal NK T-cell lymphoma is a rare aggressive (fast growing) T-cell lymphoma. This lymphoma can often be difficult to diagnose and many patients have widespread disease at diagnosis. It often depends on how advanced (or widespread) the lymphoma is at diagnosis that can predict how well someone will go with treatment.
Treatment for nasal NK T-cell lymphoma
Once all the results from the biopsy and the staging scans have been completed, the doctor will review these to decide the best possible treatment for a patient. At some cancer centres, the doctor will also meet with a team of specialists to discuss the best treatment and this is called a multidisciplinary team (MDT)meeting.
Doctors take into consideration many factors about the lymphoma and the patient’s general health to decide when and what treatment is required.
This is based on:
- The stage of lymphoma
- Symptoms (including the size and location of the lymphoma)
- How the lymphoma is affecting the body
- Past medical history & general health
- Current physical and mental wellbeing
- Patient preferences
Treatment options often depend on the stage of the nasal NK T-cell lymphoma. For localised disease radiotherapy and chemotherapy may be used. In more advanced stages chemotherapy may be given without radiotherapy. The standard first-line chemotherapy regimens used may include:
- SMILE (dexamethasone, methotrexate, ifosfamide, L-asparaginase and etoposide)
- AspaMetDex (L-asparaginase, methotrexate and dexamethasone)
Common side effects
There are many different side effects of the treatment and these are dependent on the treatment that has been given. The treating doctor and/or cancer nurse can explain the specific side effects prior to the treatment. Some of the more common side effects of treatment may include:
- Anaemia (low red blood cells carry oxygen around the body)
- Thrombocytopenia (low platelets that help bleeding and clotting)
- Neutropenia (low white blood cells help with immunity)
- Nausea and vomiting
- Bowel problems such as constipation or diarrhoea
- Fatigue (tiredness or lack of energy
The medical team, doctor, cancer nurse or pharmacist, should provide information about:
- What treatment will be given
- What are the common and possible side effects for the treatment
- What side effects do you need to report to the medical team
- What are the contact numbers, and where to attend in case of emergency 7 days a week and 24 hours per day
Once treatment has completed, post treatment staging scans are done to review how well the treatment has worked. The scans will show the doctor if there has been a:
- Complete response (CR or no signs of lymphoma remain) or a
- Partial response (PR or there is still lymphoma present, but it has reduced in size)
If all goes well regular follow-up appointments will be made for every 3-6 months to monitor the below:
- Review the effectiveness of the treatment
- Monitor any ongoing side effects from the treatment
- Monitor for any late effects from treatment over time
- Monitor signs of the lymphoma relapsing
These appointments are also important so that the patient can raise any concerns that they may need to discuss with the medical team. A physical examination and blood tests are also standard tests for these appointments. Apart from immediately after treatment to review how the treatment has worked, scans are not usually done unless there is a reason for them. For some patient’s appointments may become less frequent over time.
Relapsed or refractory management
Nasal NK T-cell lymphoma has a very high risk of relapsing (coming back) and some are refractory (do not respond to treatment). Further treatments may include:
- Clinical trial participation
- Targeted drug therapy (immunotherapy)
- Combination chemotherapy
- Allogeneic (from a donor) stem cell transplant
Treatments under investigation
Many new therapies are currently being tested in clinical trials around the world and In Australia for both newly diagnosed and relapsed/refractory NK T-cell lymphoma including:
- Checkpoint inhibitors (PD-1 inhibitors)
What happens after treatment?
Sometimes a side effect from treatment may continue or develop months or years after treatment has completed. This is called a late effect.
This can be a challenging time for many people and some of the common concerns can be related to:
- Mental wellbeing
- Emotional health
- Work, study, and social activities
Health and wellbeing
A healthy lifestyle, or some positive lifestyle changes after treatment can be a great help after the treatment has been finished. Making small changes such as eating and increasing fitness can improve health and wellbeing and help the body to recover. There are many self-care strategies that can help during the recovery phase.