What is the first treatment given for PTCL?
Currently PTCL is treated similarly to B-cell lymphomas. However, in recent years, scientists have developed techniques to better recognize the different types of lymphomas, such as PTCL. It is now understood that PTCL behaves differently from B-cell lymphomas and therapies are being developed that specifically target these types of lymphoma.
Anthracycline-containing chemotherapy regimens are commonly offered as the initial therapy.
With the exception of ALK-positive ALCL, anthracycline-based chemotherapies have not been shown to be as effective against most PTCL subtypes as they are for other types of lymphoma. Therefore, National Comprehensive Cancer Network (NCCN) guidelines recommend that patients seek participation in a clinical trial for the initial treatment of PTCL. In a clinical trial, patients generally will receive either the current standard of treatment or an experimental drug. If a clinical trial is not available, the following is a list of treatment regimens that are often recommended for PTCL in alphabetic order:
- CHOP (cyclophosphamide, doxorubicin, vincristine, prednisone)
- EPOCH (etoposide, prednisone, vincristine, cyclophosphamide, doxorubicin)
- HyperCVAD (cyclophosphamide, vincristine, doxorubicin, and dexamethasone) alternating with high-dose methotrexate and cytarabine
- When patients enter remission following chemotherapy, it is recommended that consolidation therapy be considered. Treatments typically pursued include high-dose chemotherapy plus stem cell transplant.
- The exceptions are:
- Patients determined to be at low risk for a relapse or recurrence, and
- Patients with the ALK-1-positive ALCL subtype, which typically has a good prognosis and therefore may not require stem cell transplant when in remission.
Studies have shown that some patients who receive stem cell transplants as part of their first-line therapy for PTCL can experience prolonged remissions. However, in the population of PTCL patients, only a select few are eligible for stem cell transplants. It is also important to note that much of the data regarding stem cell transplants are generated from small, uncontrolled trials.
Treatments Under Investigation
Many new drugs are being studied in clinical trials for the treatment of PTCL, including:
- Alisertib (MLN8237)
- Bendamustine (Treanda)
- Bortezomib (Velcade)
- GDP (gemcitabine, dexamethasone, and cisplatin)
- Lenalidomide (Revlimid)
- Nivolumab (Opdivo)
- Panobinostat (Farydak)
- Pembrolizumab (Keytruda)
- PI3K inhibitors
Participating in Clinical Trials
Clinical trials are crucial in identifying effective drugs and determining optimal doses for lymphoma patients. Because peripheral T-cell lymphomas comprise such a rare group of diseases, clinical trial enrollment is crucial to establishing more effective, less toxic treatments. The rarity of these diseases also means that the most novel treatments are often only available through clinical trials. Patients interested in participating in a clinical trial should talk to their physician. Contact the Lymphoma Research Foundation’s Helpline for an individualized clinical trial search by calling (800) 500-9976 or emailing firstname.lastname@example.org.
For additional information on these therapies, as well as treatments for mantle cell lymphoma that are currently under investigation, view or order your free copy of the Lymphoma Research Foundation's Peripheral T-Cell Lymphoma Fact Sheet.
Please note: It is critical to remember that today's scientific research is continuously 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 (LRF) for any treatment updates that may have recently emerged.