Angioimmunoblastic T-cell lymphoma (AITL) is a rare, aggressive type of non-Hodgkin lymphoma or peripheral T-cell lymphoma (PTCL), which is a cancer that affects the lymphatic system. The lymphatic system and white blood cells are part of the immune system that helps protect the body against infection and disease. Lymphomas involve cancer of the white blood cells (lymphocytes) and are classified by the type of white blood cells involved: either B-lymphocytes (B-cells) or T-lymphocytes (T-cells). In angioimmunoblastic T-cell lymphoma, a T-cell transforms into a malignant (cancerous) cell that leads to a dysfunction of the immune system. Angioimmunoblastic T-cell lymphoma mainly affects the elderly.

Symptoms of angioimmunoblastic T-cell lymphoma may include an enlargement of the lymph nodes and other lymphatic organs, such as the spleen and bone marrow; fatigue; pale skin; dark urine; malaise; persistent high fever; night sweats; unintended weight loss; edema (swelling); pleural effusion (fluid around lungs); shortness of breath; rapid heartbeat; abdominal fluid (ascites); rash; itching; liver enlargement; bruising; vasculitis (inflammation of blood vessels); neurological abnormalities, such as confusion, apathy, tinnitus (ringing in the ears), nerve inflammation (polyneuritis), eye swelling, and hearing or visual impairment; gastrointestinal abnormalities, such as ulcers, diarrhea, gastrointestinal bleeding, and intestinal polyps, inflammation of the bile ducts; and autoimmune disorders, such as autoimmune hemolytic anemia (AIHA) and immune thrombocytopenia (low platelets; ITP).

Some patients with angioimmunoblastic T-cell lymphoma may not initially receive treatment as their doctor may take a wait-and-watch approach until the cancer progresses. Treatment for aggressive angioimmunoblastic T-cell lymphoma includes combined chemotherapy, such as cyclophosphamide, doxorubicin, vincristine, prednisone (CHOP) or doxorubicin, vincristine, cyclophosphamide, etoposide, and prednisone (CHOEP); radiation therapy; higher doses of chemotherapy, followed by stem cell transplantation; corticosteroids; and multidrug chemotherapy at the end of treatment. In patients with angioimmunoblastic T-cell lymphoma with a CD30 marker on cancer cells, brentuximab vedotin (Adcetris) may be used in combination with cyclophosphamide, doxorubicin, and prednisone. Since relapse is common for angioimmunoblastic T-cell lymphoma, or for patients who are resistant to treatment, romidepsin (Istodax), belinostat (Beleodaq), or pralatrexate (Folotyn) may be administered. Other treatments following relapse may include high-dose chemotherapy followed by an autologous (patient’s) stem cell transplant or an allogeneic (donor) stem cell transplant. Additional drugs that may be used include enalidomide (Revlimid) alone or in combination with current chemotherapy regimens. Drugs used for other types of lymphoma may also be used for patients with angioimmunoblastic T-cell lymphoma, such as gemcitabine (Gemzar), bortezomib (Velcade), bendamustine (Treanda), or alemtuzumab (Campath).