Treatment Options

The type and stage 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.

Systemic Anaplastic Large Cell Lymphoma

Chemotherapy is often given to newly-diagnosed systemic anaplastic large cell lymphoma patients. Chemotherapy is treatment with drugs (as opposed to radiation, for example). Chemotherapy for anaplastic large cell lymphoma often consists of giving several drugs together (called combination chemotherapy) in a defined way, called a treatment regimen. Drug combinations are used because different medications damage or kill cancer cells in different ways making them more vulnerable to the treatment. Although patients with newly diagnosed systemic ALCL respond well to common first-line chemotherapy regimens such as CHOP (cyclophosphamide, doxorubicin, vincristine, and prednisone), long-term disease outcome varies depending on the subtype. Patients with ALK-positive disease usually respond very well to CHOP or CHOEP (CHOP including etoposide), with a three-year overall survival rate of about 90 percent. In contrast, patients with ALK-negative disease have three-year survival rates of about 60 percent with similar treatments.

In August 2011, the United States Food and Drug Administration (FDA) approved brentuximab vedotin (ADCETRIS™) for the treatment of patients systemic anaplastic large cell lymphoma after failure of at least one combination chemotherapy regimen. It is an antibody-drug conjugate that combines an antibody and cytotoxic agent, allowing the antibody to direct the drug to a target on the surface of lymphoma cells known as CD30. Brentuximab vedotin is the first FDA-approved antibody drug conjugate directed to CD30-expressing cells.

Pralatrexate (Folotyn) is approved for treatment of relapsed/refractory (disease does not respond to treatment) peripheral T-cell lymphoma, which includes ALCL. This is based on the overall treatment response rate of patients in a clinical trial; however, clinical benefit such as progression- free survival or overall survival was not demonstrated. Pralatrexate is a folate analogue metabolic inhibitor that interferes with cellular metabolism.

Primary Cutaneous Anaplastic Large Cell Lymphoma

Primary cutaneous anaplastic large cell lymphoma is very different than systemic anaplastic large cell lymphoma, in terms of prognosis and management. 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. Radiation therapy (also called radiotherapy) uses high-energy x-rays to kill cancer cells and shrink tumors. Radiation is a most often localized, which means it only affects cancer cells in the treated area.

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. At relapse, radiation, topical treatments, mild chemotherapies, biologic therapies, or excisions for small lesions can all be used successfully.

For those with primary cutaneous anaplastic large cell lymphoma appearing in multiple sites on the body, systemic treatment is usually needed and may include:

  • Mild chemotherapy (single agents or mild combinations)
  • Bexarotene (Targretin) capsules
  • CVP (cyclophosphamide, vincristine, prednisone) chemotherapy
  • Methotrexate (Trexall), oral or injection form

Vorinostat (Zolinza) and romidepsin (Istodax), which are histone deacetylase inhibitors, and bexarotene (Targretin), a retinoid, are approved for cutaneous T-cell lymphoma and could be used to treat the cutaneous form of ALCL.

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.

FDA approved a 90-minute infusion for rituximab (Rituxan Injection, Genentech, Inc.) starting at Cycle 2 for patients with non-Hodgkin's lymphoma (NHL) who did not experience a grade 3 or 4 infusion-related adverse reaction during Cycle 1. Patients with clinically significant cardiovascular disease and high circulating lymphocyte counts (>5000/mcL) are not recommended to receive the faster infusion. More Information. October 19, 2012