Professor, Internal Medicine, Division of Oncology and Hematology, University of Nebraska Medical Center
What is peripheral T-cell lymphoma (PTCL)?
PTCL refers to a group of approximately 30 different, heterogeneous types of lymphomas, ranging from very slow-growing (indolent) to very fast-growing (aggressive) types.
The most common type is PTCL Not Otherwise Specified (PTCL-NOS), which likely has multiple different subtypes; we are trying to better understand PTCL-NOS using genetic analyses and testing in specialized clinical trials whether these genetic differences correspond to differences in treatment response. The second most common PTCL type is angioimmunoblastic T-cell lymphoma (AITL). Both of those types are a bit more difficult to treat with chemotherapy.
The third most common type is anaplastic large cell lymphoma (ALCL), which in turn has two subtypes based on the presence or absence of one of several abnormal anaplastic lymphoma kinase (ALK) proteins. The two subtypes are referred to as ALK-positive and ALK-negative ALCL. ALCL is typically easier to treat with chemotherapy than PTCL-NOS and AITL.
How common is PTCL?
PTCL comprises about 10% of all non-Hodgkin lymphoma (NHL) in the United States; it’s more common in Asia, comprising up to 25%-30% of NHL.
How is PTCL typically treated?
Treatment is highly variable depending on the type of lymphoma. It can be treated with localized radiation therapy if it is confined to one site on the skin, or it can be treated with various types of chemotherapy alone or in combination. Stem cell transplant or clinical trials are other potential options.
What are some current main areas of research for PTCL?
Many of the standard chemotherapy drugs we use for other types of lymphomas do not work very well for patients with PTCL, so we’re always looking for new treatment opportunities for these patients. Most of the current research focuses on identifying pathways that are activated in PTCL cells and new drugs that inhibit these pathways.
There are several new drugs that have been approved over the past few years that target some of those pathways, such as pralatrexate (Folotyn), which is a folate inhibitor that is administered through the vein; about 30% of patients with PTCL respond to pralatrexate. Another drug that has recently been approved for PTCL is romidepsin (Istodax), a histone deacetylase inhibitor that is also administered through the vein and has about a 30% response rate. Finally, brentuximab vedotin (Adcetris) is a novel therapy just approved last year for lymphomas that contain a protein called CD30 on the surface of the tumor cells; most ALCL types contain this protein, and about 30% of all patients with PTCL have CD30-positive tumors. This drug is an antibody that targets the CD30 tumor cells and is attached to a type of chemotherapy. So, this is a way to target chemotherapy to the tumor cells.
Other novel drugs are in development that target other pathways. Ultimately, I suspect we will use combinations of these agents.
Do you recommend clinical trial involvement to your patients?
Yes. Particularly with T-cell lymphoma, the outcomes with standard chemotherapy are not as good as we’d like, so we’re always trying to optimize treatment and are looking for different agents or different combinations that provide better outcomes. Basically with every patient with lymphoma, we discuss clinical trial options. I think that should be part of the conversation with every patient with PTCL.
How are you involved with the Lymphoma Research Foundation (LRF)? Do you recommend that your patients use LRF resources?
I’m on LRF’s Scientific Advisory Board. I think LRF is an excellent organization. It helps to provide patients with information on lymphoma in general and also specific to their subtype. It also provides many types of support and assists patients identify clinical trials or specialty physicians in their area. Especially with these really rare lymphoma types, I think that’s important to do. I’m very supportive of their mission.
Updated: March 14, 2012