Overview of angioimmunoblastic T-cell lymphoma (AITL)
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.
Angioimmunoblastic T-cell lymphoma (AITL) is an aggressive (fast growing), rare subtype of T-cell non-Hodgkin lymphoma (NHL). It is the most common subtype of peripheral T-cell lymphoma (PTCL). AITL accounts for around 20% of all T-cell lymphomas. AITL affects the lymph nodes, known as a ‘nodal’ lymphoma.
AITL often requires urgent treatment due to the aggressive behaviour of the disease. AITL is initially treated with chemotherapy and patients usually achieve remission (no signs of cancer detected) after the first-line treatment. However, a high percentage of patient’s relapse (the lymphoma returns) soon after. There are currently many research studies for AITL, and these trials are investigating ways to treat and improve outcomes for patients.
Who is affected by angioimmunoblastic T-cell lymphoma (AITL)?
People who are most commonly diagnosed with AITL are usually aged between 60-70 years. It can occur in other ages and is rarely reported in children. AITL occurs equally in both men and women. People are often diagnosed with a more advanced (widespread) disease.
The cause of AITL is unknown. Yet it has been linked to the Epstein-Barr virus (EBV) that commonly causes glandular fever, and the herpesvirus 6(HHV-6).
What are the symptoms of angioimmunoblastic T-cell lymphoma (AITL)?
The most common symptoms of AITL are painless swollen lymph nodes. Other symptoms may include:
- Swollen lymph nodes in the neck, groin and armpit.
- Skin rash that may or may not be itchy
- High level of antibodies (immunoglobulins) in the blood. This may cause low red blood cells, low platelets, painful swollen joints, inflammation of blood vessels and thyroid problems.
- Swollen liver and spleen
B symptoms are what doctors call the following symptoms and can include:
- Night sweats (drenching sleepwear & bedding)
- Persistent fevers
- Unexplained weight loss
- Itchy body
It is important to tell the doctor about all symptoms as it can influence the decision about the type of treatment and when to start.
Diagnosis and staging of AITL
A biopsy is always required for a diagnosis of follicular lymphoma (FL). 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.
Staging of AITL
Once a diagnosis of follicular lymphoma (FL) 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?
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. Follicular lymphoma is a low-grade 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.
What is the prognosis of AITL?
Angioimmunoblastic T-cell lymphoma (AITL) often requires urgent treatment due to the aggressive behaviour of the disease. Patients usually achieve remission (no signs of cancer detected) after initial first-line treatment. However, a high percentage of patient’s relapse (the lymphoma returns) soon after. There are currently many research studies for AITL and these trials are investigating ways to treat and improve the outcomes of patients.
Treatment for angioimmunoblastic T-cell lymphoma (AITL)
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
If treatment is needed, the patient’s type and severity of the symptoms, age, overall health and degree of the thickness of the blood, will help determine which treatment is selected.
Angioimmunoblastic T-cell lymphoma (AITL) is an aggressive lymphoma and treatment should be started soon after diagnosis. The current standard first-line treatment for AITL is chemotherapy. Common chemotherapy regimens used:
- CHOP (cyclophosphamide, doxorubicin, vincristine and prednisolone)
- CHOEP (CHOP plus etoposide)
- High dose chemotherapy followed by autologous stem cell transplant (ASCT)
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.
Common side effects from treatment
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
Relapsed or refractory AITL
For some patients, initial treatment is effective, and the lymphoma does not return after treatment, however for some patients the lymphoma returns (relapses) or in rare cases does not respond to initial treatment (refractory). If this happens there are other treatments that can be successful:
- High dose chemotherapy and autologous stem cell transplantation (own stem cells)
- High dose chemotherapy and allogeneic stem cell transplantation (donor stem cells)
- Combination chemotherapy
- Biological medicines such a romidepsin, brentuximab or pralatrexate
- Clinical trial participation
Treatments under investigation
Many new individual and combination medicines are currently being tested in clinical trials in Australia and around the world for both newly diagnosed and relapsed/refractory AITL and include:
- Alemtuzumab (Campath™)
- Belinostat (Beleodaq™)
- Brentuximab (Adcetris™)
- Lenalidomide (Revlimid™)
- Nivolumab (Opdivo™)
- Venetoclax (Venclexa™)
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.