Angioimmunoblastic T-cell lymphoma (AITL) and follicular T-cell lymphoma (FTCL)

This information page is about angioimmunoblastic T-cell lymphoma (AITL) and follicular T-cell lymphoma (FTCL). These types of lymphoma are thought to develop from the same type of cell and are treated in the same way.

On this page

What is AITL/FTCL and who gets it?

Symptoms

Treatment

Relapsed and refractory AITL/FTCL 

Research and targeted treatments

Follow-up

What is AITL/FTCL and who gets it?

Angioimmunoblastic T-cell lymphoma (AITL) and follicular T-cell lymphoma (FTCL) are types of T-cell non-Hodgkin lymphoma (NHL). Our overview page explains more about T-cell lymphoma.

About 1 in every 5 to 6 cases of T-cell lymphoma diagnosed are AITL/FTCL. Although this may sound like a lot, only around 200 people a year are diagnosed with AITL/FTCL in the UK. It usually occurs in older people, with an average age of around 60. It affects slightly more men than women.

These types of lymphoma are thought to develop from the same type of helper T cells – follicular helper T cells. Pathologists (doctors who study diseased tissues under a microscope) can do tests that identify these cells.

There is currently little difference in the way T-cell lymphomas are treated. However, as research into the biology of lymphoma continues, more specific targeted therapy for certain types of T-cell lymphoma may become available. People with AITL/FTCL may be asked to take part in a clinical trial to help develop new treatments.   

Although they develop from the same type of cell, AITL and FTCL samples may look different under a microscope. Samples from AITL biopsies show a characteristic pattern with lots of small, new blood vessels together with the lymphoma cells under a microscope. The term ‘angio’ in the name refers to these blood vessels. FTCL samples don’t usually have this characteristic pattern.

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Symptoms

AITL/FTCL can cause some characteristic symptoms.

AITL/FTCL is often very fast-growing and causes systemic symptoms (symptoms that affect your whole body). You might feel generally unwell. The lymphoma can affect how well your immune system works and you might frequently get infections. In addition, the cancerous lymphocytes can produce abnormal antibodies (proteins that usually fight infections). These antibodies cause your immune system to react against your normal cells (such as your blood cells), which is called an ‘autoimmune’ reaction.

AITL/FTCL can be difficult to diagnose because the symptoms are similar to those of non-cancerous inflammatory conditions and other cancers. You need a biopsy of a swollen lymph node (gland) to confirm the diagnosis.

AITL/FTCL typically causes:

  • swollen lymph nodes, often in several places
  • fever
  • skin rash, sometimes with itching
  • anaemia (low red blood cells), which might make you feel tired or short of breath.

You might have other symptoms like abdominal (tummy) discomfort and sometimes swelling, which can be caused by a swollen spleen (an organ of your immune system) or liver, or fluid collecting in your abdomen. Some people have excess fluid around their lungs (pleural effusion), which can cause breathing difficulties.

B symptoms’ (fevers, night sweats and weight loss) are common in people with AITL and FTCL. These symptoms often appear together.

Most people with AITL have advanced disease (stage 3 or 4) when they are diagnosed. People with FTCL are more likely to be diagnosed at an earlier stage (stage 1 or 2) and may have fewer systemic symptoms.

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Treatment

AITL and FTCL are treated in the same way.

T-cell lymphomas are rare, so it is difficult to determine which treatment gives the best outcome. You might be asked if you would like to take part in a clinical trial to help test new and current treatments.

AITL/FTCL can be difficult to treat and it is common for the lymphoma to relapse (come back).

Occasionally, people without troublesome symptoms might be treated with steroids alone to begin with.

The vast majority of people with AITL/FTCL are treated with chemotherapy. The most common chemotherapy regimen (combination of drugs) used for AITL/FTCL is CHOP, which is made up of:

  • cyclophosphamide
  • doxorubicin (hydroxydaunorubicin
  • vincristine (Oncovin®)
  • prednisolone (a steroid).

Chemotherapy is given in cycles. The drugs are given on certain days, which are followed by a break in treatment for your body to recover before the next cycle begins.

Your medical team should monitor you closely as it is common for people with AITL/FTCL to have problems with infections. Contact your medical team immediately if you have any symptoms of infection, including but not limited to:

  • fever
  • shivering
  • chills and sweating
  • feeling generally unwell, confused or disoriented
  • earache, cough, sore throat or mouth
  • redness and swelling around skin sores, injuries to intravenous lines
  • diarrhoea
  • a burning or stinging sensation when passing urine
  • unusual vaginal discharge or itching
  • unusual stiffness of the neck and discomfort around bright lights.

The cancerous T-cells in AITL/FTCL can cause the body to produce abnormal numbers of B cells. An antibody treatment that destroys B cells, like rituximab, is commonly given together with or after chemotherapy.  

If you are well enough, and your lymphoma has been reduced by chemotherapy, your doctor is likely to recommend you have more chemotherapy together with a stem cell transplant. This is to give you a better chance of staying in remission (no evidence of lymphoma) for a long time. ICE chemotherapy (ifosfamide, carboplatin and etoposide) is often used before a stem cell transplant, but your doctor might suggest a different regimen.

Other treatment options include:

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Relapsed or refractory AITL/FTCL

It is common for AITL/FTCL to relapse (come back) after being in remission. Sometimes, the lymphoma is refractory (doesn’t respond) to treatment. In these cases, your doctor might consider:

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Research and targeted treatments

There are several targeted drugs that can be effective in treating AITL/FTCL, either on their own or together with chemotherapy. These are usually only available if you are taking part in a clinical trial because they are still being investigated. Find out more about clinical trials and search for a trial that might be suitable for you at Lymphoma TrialsLink.

Drugs being tested in AITL/FTCL, but not yet approved in the UK, include:

  • cell signal blockers that disrupt signals that keep cancer cells alive or allow them to grow. They act on different pathways in cells
    • HDAC inhibitors like belinostat (Beleodaq®) or romidepsin (Istodax®), both of which are approved in the US for use in people with relapsed or refractory T-cell lymphoma.
    • PI3-K inhibitors like duvelisib
    • PD1 or PD-L1 inhibitors like nivolumab and avelumab.
  • immunomodulatory drugs like lenalidomide (Revlimid®) and thalidomide. These affect the activity of the immune system in several different ways. They can help the immune system to attack the lymphoma cells and prevent the lymphoma from growing. They also prevent the blood vessel formation seen in AITL.
  • antibody treatment, given together with or after chemotherapy. If it is given after chemotherapy, this is called ‘consolidation therapy’. Antibodies under investigation include alemtuzumab (Campath®) and bevacizumab (Avastin®). Bevacizumab targets an important molecule that is involved in the formation of new blood vessels, such as the abnormal blood vessels that form in AITL.
  • proteasome inhibitors, like bortezomib (Bortezomib Accord or Velcade®) and carfilozomib. These disrupt the balance of proteins in cells, causing them to die.

New drugs are being developed and tested all the time. This is not intended to be a comprehensive list of drugs in development.

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Follow-up

When your treatment is finished, you have regular follow-up appointments at the hospital. You are usually seen every 1–3 months at first, then every 6 months after a year or so. Your follow-up usually stops after around 3 years if you remain in remission.

You should contact your medical team or GP if you develop any symptoms of lymphoma or have other concerns between your appointments. Your specialist might bring your appointment forward if they think they need to see you sooner.

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Further reading

Related content

Lymphoma TrialsLink

Find out more about clinical trials and search for a trial that might be suitable for you.