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).
T-cell lymphomas account for approximately 15 percent of all NHLs in the United States. A similar lymphocyte called a natural killer (NK) cell shares many features with T-cells. When NK cells become cancerous, the cancer is called NK or NK/T-cell lymphoma and is generally grouped with other T-cell lymphomas. There are many different forms of T-cell lymphomas, some of which are extremely rare. T-cell lymphomas can be aggressive (fast-growing) or indolent (slow-growing).
Lymphomas are often, but not always, named from a description of the normal cell that leads to cancer. The general term peripheral T-cell lymphoma (PTCL) refers to the entire group of mature or "post-thymic" T-cell lymphomas (arise from mature T-cells), which distinguishes them from the immature T-cell lymphomas such as acute lymphocytic leukemia (ALL) or lymphoblastic lymphoma. Under this broad meaning, almost all types of T-cell lymphoma fall under the category of PTCL.
Common and uncommon types of T-cell lymphoma:
- Peripheral T-cell lymphoma not otherwise specified
- Anaplastic large cell lymphoma
- Angioimmunoblastic Lymphoma
- Cutaneous T-cell lymphoma
- Adult T-cell Leukemia/Lymphoma
- Blastic NK-cell Lymphoma
- Enteropathy-type T-cell lymphoma
- Hepatosplenic gamma-delta T-cell Lymphoma
- Lymphoblastic Lymphoma
- Nasal NK/T-cell Lymphomas
- Treatment-related T-cell lymphomas
Lymphoma survivors should have regular visits with a physician who is familiar with their medical history as well as the treatments they have received.
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 of each patient at the time of treatment. The doctor will 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.
Because there are so many different types of T-cell lymphoma, treatment varies widely. Standard lymphoma therapies, including chemotherapy, radiation, stem cell transplantation, and surgery, may be effective. Patients diagnosed with the rare forms of lymphoma should consult their medical team to find promising therapies or clinical trials.
Treatments aimed at the skin, such as ultraviolet light therapy or electron beam therapy (a type of radiation that does not penetrate to internal organs), are effective for many slow-growing T-cell lymphomas that appear in the skin. Drugs that have been approved specifically for T-cell lymphomas of the skin include bexarotene (Targretin), denileukin diftitox (Ontak), romidepsin (Istodax), and vorinostat (Zolinza).
A procedure called extracorporeal photopheresis (ECPP) is approved to treat people with mycosis fungoides or Sézary syndrome. For this procedure, blood is removed from the patient and treated with ultraviolet light, and with drugs that become active when exposed to ultraviolet light. Once the blood has been treated, it is then returned back into the patient's body.
Several agents are approved for the treatment of T-cell lymphomas. Pralatrexate (Folotyn) was approved in 2009 for relapsed (recurrence of the disease) or refractory (disease that is resistant to treatment) peripheral T-cell lymphoma. Romidepsin was approved in 2009 for the treatment of relapsed or refractory CTCL and in 2011 for the treatment of relapsed or refractory peripheral T-cell lymphoma.
Treatment options for the different types of T-cell lymphomas are expanding as new treatments are discovered and current treatments are improved. For example, a pilot study of sorafenibin is examining the use of biomarkers in relapsed or refractory patients and a phase I/II study is currently investigating everolimus plus chemotherapy with CHOP in patients who were recently diagnosed with peripheral T-cell lymphomas.
Clinical trials are crucial for identifying effective new drugs and determining optimal doses for lymphoma patients. 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 emailing [email protected].