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Angioimmunoblastic T-cell Lymphoma (AITL)

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Updated April 22, 2008

What is angioimmunoblastic T-cell lymphoma?

Angioimmunoblastic T-cell lymphoma (AITL) is an uncommon type of non-Hodgkin’s lymphoma (NHL). It arises from T-cells, a type of white blood cell that is responsible for protecting the body from infections.

What does ‘angioimmunoblastic’ mean?

The term ‘angio’ refers to blood vessels. This lymphoma has certain unusual features including an increase in the number of small abnormal blood vessels in the body parts that are affected. ‘Immunoblast’ is a term used for an immature immune cell (or lymph cell). The cancerous T-cells are immunoblasts.

Who does it affect?

Angioimmunoblastic T-cell lymphoma only makes up about 1% of all non-Hodgkin’s lymphoma. It affects older individuals, with an average age of about 60 years. It is slightly more common in men than in women.

Symptoms of AITL

There are the usual symptoms of lymphoma –- including an enlargement of lymph nodes (usually felt in the neck, armpits and groin), as well as fever, weight loss or night sweats.

In addition, angioimmunoblastic T-cell lymphoma has a number of unusual symptoms. These include skin rashes, joint pain and some blood abnormalities. These symptoms are related to what is called an ‘immune reaction’ in the body to some abnormal proteins produced by the cancer cells.

This lymphoma also has a more aggressive course than most other non-Hodgkin’s lymphomas. Involvement of the liver, spleen and bone marrow is more common. B-symptoms are also seen more frequently. These features unfortunately may signal a poorer outcome after treatment.

Diagnosis and tests for AITL

Like other lymphomas, the diagnosis of AITL is based on a lymph node biopsy. Following diagnosis, a number of tests need to be done to identify how far the lymphoma has spread. These include CT scans or a PET scan, a bone marrow test and additional blood tests.

When there are skin rashes, a biopsy may also be taken from the skin to find some characteristic features that help identify this disease.

Treatment of angioimmunoblastic T-cell lymphoma

The first treatment in this lymphoma is often directed at the ‘immune symptoms’ -– rash, joint pain and blood abnormalities. Steroids and several other agents have been found to be useful in reducing these symptoms.

Once the diagnosis is confirmed and the staging investigations completed, chemotherapy is started. CHOP is the most common chemotherapy regimen used. More than half the patients have a good initial response to chemotherapy and their disease may show a complete response.

However, a disease relapse is common and may occur within months of initial disease control. Further treatment of this lymphoma is difficult. Several approaches have been tried, including bone morrow or stem cell transplants. Disease outcomes have however remained worse than more common types of high grade lymphoma.

Sources:

Cancer: Principles and Practice of Oncology 7th Edition. Editors: VT DeVita, S Hellman and SA Rosenberg. Published by Lippincott Williams and Wilkins, 2005.

Therapy in Peripheral T/NK Neoplasms. Author JP Greer. Published in the journal Hematology in 2006.

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