Angioimmunoblastic T-Cell Lymphoma Symptoms and Treatment

Angioimmunoblastic T-cell lymphoma (AITL) is an uncommon type of non-Hodgkin lymphoma (NHL). It arises from T-cells, which is the type of white blood cell that is responsible for protecting the body from infections. It may be abbreviated ATCL and was formerly called angioimmunoblastic lymphadenopathy.

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). They are the cells that normally mature into lymphocytes, but a certain number of them normally live in the bone marrow, ready to proliferate when more lymphocytes are needed. If instead they proliferate in their immature form and don't mature, they are cancerous. The cancerous T-cells in AITL disease are immunoblasts.

Who Does It Affect?

Angioimmunoblastic T-cell lymphoma only makes up about 1% of all non-Hodgkin lymphoma. It affects older individuals, with an average age at diagnosis of about 60 years. It is slightly more common in men than in women.

Symptoms

In AITL, there are the usual symptoms of lymphoma which include an enlargement of the 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, set off by some abnormal proteins produced by the cancer cells. Often infections are seen because the disease weakens the immune system.

This lymphoma also has a more aggressive course than most other non-Hodgkin’s lymphomas. The 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

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

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. However, 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 marrow or stem cell transplants. Disease outcomes have however remained worse than more common types of high-grade lymphoma.