The type, stage, along with other factors of a patient’s anaplastic large cell lymphoma will determine their course of treatment. All of the therapies listed below are often effective in treating the disease, however, relapse is common. Therefore, long-term follow up care is required.
Cutaneous and Systemic Anaplastic Large Cell Lymphoma
Treatment of primary cutaneous ALCL depends on the extent of skin lesions. If the disease is confined to a single lesion or area, radiation therapy or surgical excision will result in complete remission in approximately 95 percent of patients. If there are multiple lesions or relapsed disease in the skin, radiation can eradicate the skin lesions but will not reduce the likelihood of new lesions developing. Those with primary cutaneous ALCL appearing in multiple sites on the body usually need systemic treatment, which travels through the blood and affects many parts of the body. There are very few studies of primary cutaneous ALCL; however, vorinostat (Zolinza) and romidepsin (Istodax), which are histone deacetylase inhibitors (drugs that inhibit the growth of tumor cells), and bexarotene (Targretin), a retinoid (drug derived from vitamin A that targets skin cell growth), are approved for cutaneous T-cell lymphoma and can be used to treat the cutaneous form of ALCL. Although primary cutaneous ALCL tends to relapse in about 40 percent of cases, the long-term prognosis remains excellent as long as relapses are confined to the skin.
Many patients with newly diagnosed systemic ALCL respond well to common first-line chemotherapy regimens such as CHOP (cyclophosphamide, doxorubicin, vincristine, and prednisone), although long-term disease outcome varies depending on the subtype and other factors. Patients with ALK-positive disease usually respond very well to CHOP or CHOEP (CHOP plus etoposide). In contrast, patients with ALK-negative disease have slightly lower survival rates with similar treatments. In some circumstances, higher doses of chemotherapy followed by stem cell transplantation may be prescribed.
Relapse/Refractory Anaplastic Large Cell Lymphoma
Brentuximab vedotin (Adcetris) is approved by the U.S. Food and Drug Administration (FDA) for the treatment of patients with relapsed or refractory (disease does not respond to treatment) systemic ALCL after the failure of at least one prior multiagent chemotherapy regimen.
Accelerated approval was granted for the systemic ALCL indication based on overall response rate. Recent studies show similarly good activity in cutaneous T-cell lymphomas, and ongoing clinical trials are combining brentuximab vedotin with chemotherapy for people newly diagnosed with systemic ALCL.
Other treatment options for relapsed/refractory disease include romidepsin, which is FDA- approved for relapsed systemic T-cell lymphomas, including systemic ALCL. Belinostat is a newer histone deacetylase inhibitor that is approved for relapsed peripheral T-cell lymphomas, including systemic ALCL, but not for cutaneous T-cell lymphomas. Finally, pralatrexate (Folotyn) is also approved for the treatment of relapsed/refractory peripheral T-cell lymphomas.
Learn more about drug approvals for ALCL by visting the FDA Drug Updates page.
Treatments Under Investigation
New treatments for ALCL are being researched all the time. There are several new drugs currently in clinical trials that are showing promising results, including:
- Bortezomib (Velcade)
- Combintation of brentuximab vedotin and chemotherapy
- Crizotinib (Xalkori)
- Lenalidomide (Revlimid)
For additional information on all of these therapies, as well as treatments for anaplastic large cell lymphoma that are currently under investigation, view or order your free copy of the Foundation’s Anaplastic Large Cell Lymphoma Fact Sheet.
Please note: It is critical to remember that today’s scientific research is continuously evolving. Treatment options for anaplastic large cell lymphoma may change as new treatments are discovered and current treatments are improved. Therefore, it is important that patients check with their physician for any treatment updates that may have recently emerged.