Angioimmunoblastic T-cell lymphoma

Angioimmunoblastic T-cell lymphoma (ATCL) is an uncommon T-cell type of non-Hodgkin lymphoma (NHL).

ATCL is more common in the elderly, and the average age at diagnosis is 62. It is slightly more common in men than in women. ATCL has been linked to different viral infections, such as the Epstein-Barr virus (EBV).

Symptoms

ATCL is usually a fast-growing (aggressive) type of NHL. It is often diagnosed at an advanced stage (stage 3 or stage 4). People with ATCL often have:

• larger than normal lymph nodes in different areas of the body
• B symptoms (unexplained fever, drenching night sweats and unexplained weight loss)
• an itchy skin rash
• joint pain
• larger than normal organs, such as the liver and spleen
• NHL that has spread to the bone marrow
• anemia

Treatments

There is no standard treatment for ATCL. Treatment is usually effective at first, but ATCL often comes back (recurs).

Corticosteroids, such as prednisone or dexamethasone (Decadron, Dexasone), may be the first treatment for ATCL. The disease often progresses while it is treated with corticosteroids. Chemotherapy is given when the disease starts to progress.

Chemotherapy

ATCL is often treated with a combination of chemotherapy drugs. A common chemotherapy combination used is CHOP, which is cyclophosphamide (Cytoxan, Procytox), doxorubicin (Adriamycin), vincristine (Oncovin) and prednisone.

Targeted therapy

Targeted therapy uses drugs to target specific molecules (such as proteins) on the surface of cancer cells. These molecules help send signals that tell cells to grow or divide. By targeting these molecules, the drugs stop cancer cells from growing and spreading while limiting harm to normal cells. Brentuximab vedotin (Adcetris) is a targeted therapy drug that targets CD30. It may be used along with chemotherapy to treat ATCL that expresses CD30.

Stem cell transplant

A stem cell transplant may be an option for some younger people with ATCL that no longer responds to treatment (called refractory disease).