Peripheral T-Cell Lymphoma (PTCL)
Lymphoma is the most common blood cancer. The two main forms of lymphoma are Hodgkin lymphoma and non-Hodgkin lymphoma (NHL). Lymphoma occurs when cells of the immune system called lymphocytes, a type of white blood cell, grow and multiply uncontrollably. Cancerous lymphocytes can travel to many parts of the body, including the lymph nodes, spleen, bone marrow, blood, or other organs, and form a mass called a tumor. The body has two main types of lymphocytes that can develop into lymphomas: B-lymphocytes (B-cells) and T-lymphocytes (T-cells).
Peripheral T-cell lymphoma (PTCL) consists of a group of rare and usually aggressive (fast-growing) NHLs that develop from mature T-cells. Most T-cell lymphomas are PTCLs, which collectively account for about 10 percent to 15 percent of all NHL cases in the United States.
PTCLs are sub-classified into various subtypes, each of which are typically considered to be separate diseases based on their distinct clinical differences. Most of these subtypes are very rare; the three most common subtypes of PTCL, peripheral T-cell lymphoma not otherwise specified (PTCL-NOS), anaplastic large-cell lymphoma (ALCL), and angioimmunoblastic T-cell lymphoma (AITL), account for approximately 70 percent of all PTCLs in the United States.
Peripheral T-cell Lymphoma Not Otherwise Specified(PTCL NOS) refers to a group of diseases that do not fit into any of the other subtypes of PTCL. PTCL-NOS is the most common PTCL subtype, making up about one quarter of all PTCLs. It is also the most common of all the T-cell lymphomas. The term PTCL can be confusing as it can refer to the entire spectrum of mature T-cell lymphomas, but it can also refer to the specific PTCL-NOS subtype. Although most patients with PTCL-NOS are diagnosed with their disease confined to the lymph nodes, sites outside the lymph nodes, such as the liver, bone marrow, gastrointestinal tract, and skin, may also be involved. This group of PTCLs is aggressive and requires combination chemotherapy upon diagnosis.
Anaplastic Large-Cell Lymphoma (ALCL) is an aggressive T-cell lymphoma, accounting for about three percent of all lymphomas in adults (about 15 percent to 20 percent of all PTCLs) and between 10 percent and 30 percent of all lymphomas in children. ALCL can appear in the skin or in other organs throughout the body (systemic ALCL). ALCL has several different subtypes, each with different expected outcomes and treatment options. For more information, see the Lymphoma Research Foundation's (LRF) ALCL Fact Sheet.
Angioimmunoblastic T-Cell Lymphoma (AITL) is an aggressive T-cell lymphoma that accounts for about two percent of all NHL cases (about 10 percent to 15 percent of all PTCLs) in the United States. This type of lymphoma often responds to milder therapies, such as steroids, although it often progresses and requires chemotherapy and other medications. In advanced cases, bone marrow transplantation may be used. For more information, see LRF's AITL Fact Sheet.
Cutaneous T-cell Lymphomas (CTCL) are a group of lymphomas that originate in the skin. CTCLs are a subset of PTCL because they are lymphomas of mature T-cells. However, these lymphomas are generally less aggressive, have a different prognosis, and have different treatment approaches than the aggressive PTCLs.
The most common subtype of CTCL is mycosis fungoides. It is generally an indolent (slow-growing) cancer that starts in the skin, appearing as a scaly, red rash in areas of the body that are not usually exposed to the sun. Sézary syndrome is an advanced, variant form of mycosis fungoides. It affects both the skin and the peripheral blood, and it can cause widespread itching, reddening and peeling of the skin as well as skin tumors. For more information, see LRF's CTCL Fact Sheet.
Enteropathy-Type T-Cell LymphomaEnteropathy-type T-cell Lymphoma is an extremely rare subtype that appears in the intestines and is strongly associated with celiac disease.
Nasal NK/T-Cell Lymphoma involves natural killer (NK) cells, which are closely related to and often have features that overlap with T-cells. Although this aggressive lymphoma is very rare in the United States, it is more common in Asia and parts of Latin America, leading researchers to suspect that some ethnic groups may be more prone to this cancer. This type of lymphoma is associated with the Epstein-Barr virus and often involves the nasal area, trachea, gastrointestinal tract, or skin.
Hepatosplenic Gamma-Delta T-Cell Lymphomais an extremely rare and aggressive disease that starts in the liver or spleen.
As with other rare cancers, patients with enteropathy-type, NK/Tcell, or hepatosplenic gamma-delta T-cell lymphomas should discuss treatment options and potential clinical trials with their medical team.
For most subtypes of PTCL, the frontline treatment regimen is typically a combination chemotherapy, such as CHOP (cyclophosphamide, doxorubicin, vincristine, prednisone), EPOCH (etoposide, vincristine, doxorubicin, cyclophosphamide, prednisone), or other multi-drug regimen. Because most patients with PTCL will relapse, some oncologists recommend giving high-dose chemotherapy followed by an autologous (in which patients receive their own stem cells) stem cell transplant to some patients who had a good response to their initial chemotherapy. While promising, there is no firm clinical data to support that undergoing a transplant in this setting is better than not undergoing a transplant. For more information, see LRF's Transplant in Lymphoma factsheet.
In fall 2009, the U.S. Food and Drug Administration (FDA) approved pralatrexate (Folotyn) for the treatment of patients with relapsed (disease returns after treatment) or refractory (disease does not respond to treatment) PTCL. Pralatrexate was the first drug approved specifically for patients with PTCL. Clinical trials are in development to see if pralatrexate is effective when combined with other drugs commonly used in the treatment of both T-cell and B-cell lymphomas, including gemcitabine (Gemzar) and bexarotene (Targretin).
In 2009, the FDA approved romidepsin (Istodax) for the treatment of relapsed or refractory CTCL. In 2011, romidepsin was also approved for the treatment of relapsed or refractory PTCL. Current and planned clinical trials are testing the effectiveness of romidepsin in combination with chemotherapies commonly used in PTCL, including CHOP and ICE (ifosfamide, carboplatin, etoposide).
Also in 2011, the FDA approved brentuximab vedotin (Adcetris) for the treatment of relapsed or refractory systemic ALCL. Brentuximab vedotin in combination with other treatments, including CHOP and bendamustine (Treanda), are currently underway, and the drug is also being tested in other PTCL types.
Relapsed patients may also be treated with combination chemotherapy programs such as ICE followed by an autologous or allogeneic (in which patients receive stem cells from a donor) stem cell transplant. For more information, see LRF's Transplant in Lymphoma factsheet.
Gemcitabine appears effective against some forms of relapsed PTCL and is often given in combination with other chemotherapies, including vinorelbine (Navelbine) and doxorubicin (Doxil) in a regimen called GND. Other chemotherapy regimens used for relapsed or refractory PTCLs include DHAP (dexamethasone, cytarabine, cisplatin) and ESHAP (etoposide, methylprednisolone, cytarabine, and cisplatin).
Many new drugs are being studied in clinical trials for the treatment of PTCL, including:
Vaccine therapy is also being investigated in clinical trials. 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 LRF or with their physician for any treatment updates that may have recently emerged.
Clinical trials are crucial in identifying effective drugs and determining optimal doses for lymphoma patients. Because peripheral T-cell lymphomas are rare, clinical trial enrollment is crucial to establishing more effective, less toxic treatments. Patients interested in participating in a clinical trial should talk to their physician or contact LRF's Helpline for an individualized clinical trial search by calling (800) 500-9976 or email email@example.com.
PTCL is generally characterized by multiple disease relapses after responses to a variety of treatments. Patients in remission should have regular visits with a physician who is familiar with their medical history as well as with the treatments they have received. Medical tests, such as blood tests and computed axial tomography (CAT) scans, may be required at various times during remission to evaluate the need for additional treatment. Some treatments can cause long-term effects or late effects, which can vary based on duration and frequency of treatments, age, gender, and overall health at the time of treatment. Doctors will also check for these effects during follow-up care.
Survivors and their caregivers are encouraged to keep copies of all medical records and test results as well as information on the types, amounts, and duration of all treatments received. This documentation will be important for keeping track of any effects resulting from treatment or potential disease recurrences. For further information, please review our fact sheet on survivorship issues.
A lymphoma diagnosis often triggers a range of feelings and concerns. In addition, cancer treatment can cause physical discomfort. Support groups and online message boards can help patients connect with other people who have lymphoma. One-to-one peer support programs, such as LRF's Lymphoma Support Network, match lymphoma patients (or caregivers) with volunteers who have gone through similar experiences.
The Lymphoma Research Foundations (LRF) T-Cell Lymphoma Transportation Assistance Fund grant program provides financial assistance to T-cell lymphoma patients who are uninsured or have adequate medical insurance but struggle to pay for transportation costs (e.g., mileage, parking, tolls, gas, train tickets, etc.) to get to and from treatment.
LRF offers a wide range of resources that address treatment options, the latest research advances, and ways to cope with all aspects of lymphoma. LRF also provides many educational activities, from in-person meetings to teleconferences and webcasts. For more information about any of these resources, visit the website at www.lymphoma.org or www.FocusOnPTCL.org. You can also contact the Helpline at (800) 500-9976 or firstname.lastname@example.org.
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