Angioimmunoblastic T-cell lymphoma

Angioimmunoblastic T-cell lymphoma (AITL) is a rare type of fast-growing non-Hodgkin lymphoma (NHL). It develops when T-cells become abnormal. T-cells are white blood cells that fight infection.

AITL usually begins in the lymph nodes. The lymphoma cells build up, which makes the lymph nodes bigger. Sometimes AITL begins in other parts of the body. This is called extranodal disease.

Symptoms of AITL can include:

  • feeling unwell
  • night sweats
  • high temperatures (fevers)
  • weight loss
  • itchy skin or rash
  • swellings in the neck, armpit or groin.

To diagnose AITL, a doctor usually removes a sample of an enlarged lymph node and checks it for lymphoma cells. This is called a biopsy. You may have biopsies taken from other parts of the body. You will also have tests and scans to find out more about the lymphoma.

Most people have a combination of drugs that includes chemotherapy and steroids. Some people also have stem cell treatment. Sometimes doctors delay treatment for people with no symptoms. This is called watch and wait.

You may be invited to join a clinical trial. These test new treatments or ways of giving treatments. Your doctor will talk to you about clinical trials.

What is angioimmunoblastic T-cell lymphoma (AITL)?

It is best to read this information with our general information about non-Hodgkin lymphoma (NHL). If you have any more questions, you can ask your doctor or nurse at the hospital where you are having treatment.

Angioimmunoblastic T-cell lymphoma (AITL) is a rare type of fast-growing NHL. It develops when T-cells (also called T-lymphocytes) become abnormal. T-cells are white blood cells that fight infection.

The abnormal T-cells (lymphoma cells) usually build up in lymph nodes, but it can affect other parts of the body.


Causes and risk factors for AITL

The causes of lymphoma are not known. Like other cancers, it is not infectious and cannot be passed on to other people. It usually affects people aged 50 to 70.

We have more information about risk factors for lymphoma.


Signs and symptoms of AITL

Symptoms often include:

  • feeling generally unwell
  • itchy skin or rash
  • swellings in the neck, armpit or groin caused by lymphoma cells building up in the lymph nodes.

B symptoms

Some people also have:

  • drenching night sweats
  • high temperatures (fevers) with no obvious cause
  • unexplained weight loss.

These are called B symptoms.

Symptoms of extranodal disease

AITL may also sometimes affect other parts of the body outside the lymph nodes. This is called extranodal disease. Extranodal disease may cause different symptoms, depending on where the lymphoma is in the body. For example, some people develop a swollen tummy area (abdomen). Or if AITL is affecting the lungs, this can cause shortness of breath.


Diagnosing AITL

AITL often causes symptoms that are like other conditions. This sometimes makes diagnosis difficult. The most common test for AITL is to remove part or all of an enlarged lymph node (a <biopsy >). This may be done under local or general anaesthetic. The biopsy is then sent to a laboratory to be checked for lymphoma cells. You may also have biopsies taken from other areas of the body.

Other tests may include:

  • blood tests
  • x-rays and scans
  • bone marrow samples.

Doctors use the information from all these tests to find out more about the lymphoma, such as its stage and grade.


Staging and grading AITL

Staging

The stage of the lymphoma describes which areas of the body are affected by lymphoma. This information helps doctors plan the right treatment for you.

The stage of a lymphoma is usually described using numbers from 1 to 4. Stages 1 and 2 are also called early-stage, limited or localised lymphoma. Stages 3 and 4 are also called advanced lymphoma.

As well as giving each stage a number, doctors often add the letters A or B. A means you do not have B symptoms. B means you do have B symptoms.

Sometimes the lymphoma can affect areas outside the lymph nodes. This is called extranodal lymphoma, and the stage will include the letter E (for extranodal).

Grading

Non-Hodgkin lymphomas are divided into two groups:

  • Low-grade (indolent) lymphomas, which usually grow slowly.
  • High-grade (aggressive) lymphomas, which grow more quickly.

AITL is a high-grade, fast-growing lymphoma.


Treating AITL

Your treatment may depend on:

  • your general health
  • whether you have symptoms caused by the lymphoma.

Most people have a combination of drugs that includes chemotherapy and steroids. Some people then have stem cell treatment. Sometimes doctors delay treatment for people with no symptoms (called watch and wait), or give them steroids until they need chemotherapy.

Treatments may make all signs of the AITL disappear (called remission). But there is a risk AITL will come back. This is called recurrence or relapse. Further treatment can be given to manage AITL that comes back.

Watch and wait

If the lymphoma isn’t causing troublesome symptoms, your doctor may suggest that you delay having any treatment. Instead you have regular tests and appointments to monitor the lymphoma and check for signs that you need to start treatment. This is called watch and wait. If you develop new or worse symptoms, your doctor may talk to you about starting treatment.

Chemotherapy

Chemotherapy uses anti-cancer (cytotoxic) drugs to destroy cancer cells.

AITL is usually treated with a combination of chemotherapy and other drugs. The most commonly used drugs are called CHOP and CHOEP. Other combinations of drugs may also be used. This is often as part of a clinical trial.

CHOP is named after the initials of the drugs used. It includes:

  • the chemotherapy drugs cyclophosphamide, doxorubicin (hydroxydaunomycin) and vincristine (Oncovin®)
  • a steroid called prednisolone.

The chemotherapy drugs are given into a vein (intravenously). You take the steroid as tablets. You usually have treatment every 2 or 3 weeks. You will have up to 8 treatments over several months.

CHOEP is the same as CHOP but also includes a chemotherapy drug called etoposide.

Steroids

Steroids are drugs that are often given with chemotherapy to treat lymphomas. They help make chemotherapy more effective. They also help you feel better and can make you feel less sick.

Steroids are sometimes given on their own to treat AITL. They can help control this type of lymphoma for a time.

Targeted therapy

Targeted therapies (also called biological therapies) are drugs that use unique features of the cancer to find and treat cancer cells. These drugs only ‘target’ the cancer cells, so they have less effect on healthy cells. They may be used to treat AITL as part of a clinical trial.

Stem cell treatment (transplants)

This is an intensive treatment, so it is not suitable for everyone.

Stem cells are a type of blood cell that can make all other types of blood cells. There are two different types of stem cell treatment:

High-dose treatment with stem cell support (autologous stem cell transplant)

Some people have treatment to put the lymphoma into remission. Then some of their own stem cells are collected from their blood and stored. Then they have high doses of chemotherapy to try to destroy any remaining lymphoma cells. After this, their stem cells are returned through a drip (like a blood transfusion). The stem cells help their blood cells recover from the effects of chemotherapy.

Donor transplant (allogeneic transplant)

Some people have treatment, to put the lymphoma into remission, and are then given stem cells from another person (a donor).

Clinical trials

It is common for AITL to be treated as part of a clinical trial. Clinical trials test new treatments or new ways of giving treatments.

Your lymphoma doctor will always explain your treatment options in detail before you make any decisions


Follow-up after treatment

After treatment, you will have regular check-ups. These appointments are a good opportunity for you to talk to your doctor or nurse about any concerns you have. Your doctor will want to know how you are feeling generally, and to check you are recovering from any side effects of treatment. We have more information about follow-up.


Getting support

Everyone has their own way of dealing with illness and the different emotions they experience. You may find it helpful to talk things over with family and friends or your doctor or nurse. You can also call our cancer support specialists free on 0808 808 00 00. The organisations below also offer information and support:

  • Bloodwise offers support and information to people affected by blood cancers, including lymphoma.
  • The Lymphoma Association gives emotional support, advice and information on all aspects of Hodgkin lymphoma and non-Hodgkin lymphoma. It has a national network of people with lymphoma, and local groups.