Angioimmunoblastic T-cell lymphoma

Angioimmunoblastic T-cell lymphoma (AITL) is a rare type of non-Hodgkin lymphoma (NHL).

NHL is a type of blood cancer that affects white blood cells called lymphocytes Open a glossary item. It is a cancer of the lymphatic system Open a glossary item

There are many different types of NHL. AITL is a type of peripheral T-cell lymphoma. It is fast growing (high grade).

Treatment is usually a combination of chemotherapy Open a glossary item drugs. Doctors call this combination CHOP.

What is Angioimmunoblastic T-cell lymphoma?

Angioimmunoblastic T-cell lymphoma (AITL) is a rare type of non-Hodgkin lymphoma (NHL).

Lymphoma develops when white blood cells called lymphocytes grow out of control. They move around your body in your blood and your lymphatic system. 

The lymphatic system is an important part of our immune system. It has tubes that branch through all parts of the body.

These tubes are called lymph vessels or lymphatic vessels and they carry a straw coloured liquid called lymph. This liquid circulates around the body tissues. It contains a high number of white blood cells (lymphocytes) which fight infection.

Diagram of the lymphatic system

There are two types of lymphocytes: T lymphocytes (T cells) and B lymphocytes (B cells). 

AITL develops when the body makes abnormal T lymphocytes. So it is a type of T cell lymphoma. The abnormal lymphocytes build up in the lymph nodes Open a glossary item or other body organs. They don't work properly. So they can't fight infection as normal white blood cells do.

It is fast growing, and doctors call it a high grade lymphoma.

Symptoms of angioimmunoblastic T-cell lymphoma

Symptoms can start or get worse in just a few weeks.

Painless swellings

The most common symptom is one or more painless swellings in the:

  • neck
  • armpit
  • groin

These swellings are enlarged lymph nodes Open a glossary item. They can grow very quickly.

General symptoms (B symptoms)

You might have other general symptoms such as:

  • heavy sweating at night
  • high temperatures that come and go with no obvious cause
  • losing a lot of weight (more than one tenth of your total weight)

Doctors call this group of symptoms B symptoms. Some people may also have unexplained itching. It is important to tell your doctor about any symptoms like this.

Other symptoms

Lymphoma can grow in the bone marrow. Lymphoma cells can take up the space of normal blood cells. This can cause low blood cell counts. Symptoms of this includes:

  • tiredness and shortness of breath
  • bleeding and bruising

You might have a swollen spleen Open a glossary item or liver. This can cause bloating, and you might have tummy discomfort or pain.

The lymphoma cells produce abnormal proteins and the body reacts to these. Doctors call this as an autoimmune reaction. This can cause symptoms such as:

  • skin rashes
  • inflammation of your joints

Getting diagnosed with angioimmunoblastic lymphoma

The main test to diagnose AITL is a biopsy Open a glossary item.  A doctor removes a sample from the affected area. This often means removing part or all of a swollen lymph node. They send it to the laboratory for a specialist to look at it under a microscope. 

You usually also have some blood tests.

If your doctor diagnoses AITL, you usually have further tests. These might include:

  • PET-CT scan Open a glossary item
  • a CT scan Open a glossary item
  • bone marrow test Open a glossary item to check if you have lymphoma cells in your bone marrow

Stages and grades of angioimmunoblastic T cell lymphoma

Grade

Grade describes how the cells look under a microscope. Your grade tells the doctor how quickly the lymphoma is likely to grow and spread. Doctors put NHL into 2 grades:

  • low grade (slow growing)
  • high grade (fast growing)

AITL is fast growing and doctors call it a high grade lymphoma.

Stage

You have tests to find out the number of places in your body affected by lymphoma, and where these are. This is your stage. Doctors use your stage to plan your treatment.

There are different systems for staging NHL. Your doctor can tell you more about your stage, and what it means.

There are 4 main stages of NHL (stage 1 to stage 4). Or your doctors might talk about early (limited) stage and advanced stage lymphoma. Early stage usually means either stage 1 or stage 2 lymphoma. Advanced stage usually means either stage 3 or stage 4 lymphoma. 

Treatment for angioimmunoblastic T-cell lymphoma

 Your treatment depends on:

  • where your lymphoma is (the stage)
  • your symptoms
  • your general health

The main treatments

The main treatments are:

  • a combination of chemotherapy Open a glossary item drugs 
  • a stem cell transplant Open a glossary item
  • steroids  Open a glossary item
  • targeted cancer drugs Open a glossary item as part of a clinical trial

Chemotherapy

You usually have a several chemotherapy drugs together.  A common combination of chemotherapy drugs is called CHOP. This is a mixture of chemotherapy and steroids. It includes the drugs cyclophosphamide, doxorubicin, vincristine and dexamethasone. 

Stem cell transplant

A stem cell transplant allows you to have very high doses of chemotherapy. You can have a transplant using:

  • your own stem cells (autologous stem cell transplant)
  • a donor’s stem cells (allogeneic stem cell transplant)

You might have an autologous stem cell transplant as part of your treatment for AITL. You might have it after your CHOP chemotherapy, if you responded well to the chemotherapy. This is intensive treatment so you need to be well enough to have it.

Steroids

You often have steroids together with chemotherapy for lymphoma. Sometimes you have steroids on their own to treat AITL.

Treatment when AITL doesn't go away or comes back

Lymphoma that comes back after treatment is called relapsed disease. You might have:

  • further chemotherapy treatment using different chemotherapy drugs
  • a stem cell transplant using donor stem cells - an allogenic stem cell transplant
  • targeted cancer drugs on a clinical trial

Research

Researchers around the world are looking at the treatments for NHL. Your doctor might suggest you take part in a clinical trial. 

Survival for angioimmunoblastic T-cell lymphoma

The best person to talk to you about your outlook or prognosis is your doctor. Not everyone wants to know. People cope differently with their lymphoma and want different information.

Survival depends on many factors. So no one can tell you exactly how long you will live. Your doctor might be able to give you some guide, based on their knowledge and experience.

Coping with NHL

It can be difficult finding our you have NHL. Some people find it can be particularly worrying in the run up to check up appointments.

Advice and support is available to help you cope with NHL and its treatment.

Our discussion forum Cancer Chat is a place for anyone affected by cancer. You can share experiences, stories and information with other people who know what you are going through.

  • Advances in understanding of angioimmunoblastic T-cell lymphoma
    S Chiba and others
    Leukaemia, 2020. Volume 34, Pages 2592–2606 

  • Outcomes following front-line chemotherapy in peripheral T-cell lymphoma: 10-year experience at The Royal Marsden and The Christie Hospital
    M Gleeson and others 
    Leukaemia and lymphoma, 2017. Volume 9, Pages 1-10

  • Angioimmunoblastic T-cell lymphoma: the many-faced lymphoma
    M Lunning and J Vose 
    Blood, 2017. Volume 129, Issue 9, Pages 1095-1102

  • The survival outcome of patients with relapsed/refractory peripheral T-cell lymphoma-not otherwise specified and angioimmunoblastic T-cell lymphoma
    D Chihara and others 
    British Journal of Haematology, 2017. Volume 176, Issue 5, Pages 750-758

  • Pan-London Haemato-Oncology Clinical Guidelines Lymphoid Malignancies Part 5: Less Common Lymphoid Malignancies
    South East London Cancer Alliance and others
    January 2020

  • Guidelines for the management of mature T- and naturalkiller-cell lymphomas (excluding cutaneous T-cell lymphoma):a British Society for Haematology Guideline
    C Fox and others
    British Journal of Haematology, 2022. Volume 196, Issue 3, Pages 507 - 522

  • The information on this page is based on literature searches and specialist checking. We used many references and there are too many to list here. Please contact patientinformation@cancer.org.uk with details of the particular issue you are interested in if you need additional references for this information.

Last reviewed: 
19 Apr 2024
Next review due: 
19 Dec 2024

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