Adult T-Cell Leukemia/Lymphoma:
Treatment Options

Depending on the Adult T-cell leukemia/lymphoma (ATLL)  subtype, the following treatment options are available:

Slow-growing Subtypes of ATLL

For some patients who have one of the slower-growing subtypes of ATLL with mild or no symptoms, physicians may recommend not treating the disease right away. This is called active surveillance (also known as “watchful waiting” or “observation”). In this case, patients are monitored through regular physical exams (to check for any swollen lymph nodes) or periodic imaging tests (like CT scans). If patients begin to have symptoms or signs of disease progression, treatment is initiated.

Visit our active surveillance learning center for more information on this treatment approach >

Aggressive Subtypes of ATLL

For ATLL affecting the skin, skin-directed therapies such as topical (applied to the skin surface), steroids or local radiation (applied only to the affected area in the body) may be prescribed. Common frontline (initial) therapies used to treat ATLL are the same as those used to treat other types of T-cell lymphomas. These include:

• Chemotherapy (drugs that stop the growth of or kill cancer cells).
  • CHOP (cyclophosphamide, doxorubicin, vincristine, and prednisone)
  • CHOEP (cyclophosphamide, doxorubicin, vincristine, etoposide, and prednisone)
  • Dose-adjusted EPOCH (etoposide, vincristine, doxorubicin, cyclophosphamide, and prednisone)
  • Hyper-CVAD (cyclophosphamide, vincristine, doxorubicin, and dexamethasone) alternating with high dose methotrexate and cytarabine (Cytosar)
• Chemoimmunotherapy is a combination of chemotherapy with immunotherapy (drugs that use the body’s immune system to fight cancer), such as antibody-drug conjugates (ADC)
  • An ADC is a monoclonal antibody (a protein made in the laboratory that binds to cancer cells and helps the immune system destroy them) attached to a chemotherapy drug. The monoclonal antibody in the ADC recognizes and binds to a protein on the cancer cell surface. Once the ADC is inside the cell, the chemotherapy drug separates from the ADC and kills the cancer cell by targeting cell multiplication.
  • BV-CHP is a combination of the ADC brentuximab vedotin (Adcetris) and the chemotherapy regimen cyclophosphamide, doxorubicin, and prednisone (CHP). This combination is used for the treatment of lymphomas that are positive for CD30 (molecule present on the surface of certain lymphomas)
• Antiviral therapy (treatment of infections caused by a virus).
  • Zidovudine (Retrovir, AZT) in combination with interferonalpha, to treat the underlying HTLV-1 infection (in patients with slow-growing ATLL).
• Stem cell transplantation (SCT) may be appropriate in some patients following remission (disappearance of signs and symptoms). During this procedure the patient is treated with high-dose chemotherapy or radiation and then receives healthy stem cells to restore the immune system and the bone marrow’s ability to make new blood cells.
  • Autologous SCT (uses patient’s own stem cells cells)
  • Allogeneic SCT (uses stem cells from a related or unrelated donor)

Treatments Under Investigation for ATLL

Several new drugs are being studied in clinical trials for ATLL, as single-agent therapy or as part of a combination therapy regimen, including the following:

• Bortezomib (Velcade)
• Tolinapant (ASTX660)
• Mogamulizumab (Poteligeo)
• Valemetostat (DS-3201b)

It is critical to remember that today’s scientific research is always evolving. Treatment options may change as new treatments are discovered and current treatments are improved. Therefore, it is important that patients check with their physician or with the Lymphoma Research Foundation for any treatment updates that may have recently appeared.

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