Anaplastic Large Cell Lymphoma: Treatment Options

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.