T-cell lymphomas make up about 10 percent of non-Hodgkin lymphomas (NHLs) in the United States. Subtypes include:
Lymphoblastic lymphoma/leukemia can be considered either lymphoma or leukemia, depending on how much of the bone marrow is involved. It involves precursor, not mature, T cells and is treated like acute lymphocytic leukemia.
Most T-cell NHLs are treated with combination chemotherapy or multi-drug regimens. Patients with some subtypes of T-cell lymphoma may have a bone marrow transplant after their initial therapy and first remission. Most patients who relapse are transplant candidates. Many treatments are being investigated in clinical studies, including some that target specific subtypes of T-cell lymphomas.
Peripheral T-cell lymphoma (PTCL) refers to a diverse group of rare, typically aggressive (fast-growing) T-cell lymphomas that make up the majority of T-cell NHLs in the United States. PTCL arises in the lymph tissue outside of the bone marrow, such as in the lymph nodes, spleen, gastrointestinal tract, and skin. PTCLs are further subdivided.
Angioimmunoblastic T-cell lymphoma
Angioimmunoblastic T-cell lymphoma (AITL) is an aggressive subtype and is typically diagnosed at stage III or IV.
People with AITL usually have fever, night sweats, weight loss, skin rashes and autoimmune disorders, and they often develop infections. While the cause is unknown, AITL has been linked to Epstein-Barr virus, which causes mononucleosis.
Initially this disease may be treated with steroids. It often progresses and may be treated with the typical T-cell NHL treatments as well as radiation therapy.
Anaplastic large cell lymphoma
There are three types of this rare, aggressive T-cell lymphoma:
- Primary cutaneous anaplastic large cell lymphoma (ALCL), one of several types of cutaneous lymphoma, which is often referred to as lymphoma of the skin.
- ALK-positive (ALK+) ALCL, systemic disease which expresses a protein called anaplastic lymphoma kinase (ALK)
- ALK-negative (ALK-) ALCL, systemic disease which doesn’t express ALK
ALK+ ALCL usually affects children, and ALK- ALCL is typically found in adults over age 60. Both are treated as aggressive lymphomas. Patients with ALK+ ALCL tend to have excellent outcomes with the standard multi-agent chemotherapy known as CHOP — cyclophosphamide, doxorubicin hydrochloride, vincristine and prednisone. In 2011, the new drug brentuximab vedotin (Adcetris) was approved for patients with both ALK+ and ALK- relapsed or refractory systemic ALCL.
Extranodal NK/T-cell lymphoma, nasal type
This subtype is more common in parts of Asia and South America and is associated with Epstein-Barr virus. It often involves the upper airways, such as the nose and upper throat, but it can also invade the skin and digestive tract. This is the only PTCL subtype for which radiation is the primary therapy.
Enteropathy-type T-cell lymphoma
This extremely rare subtype is an intestinal lymphoma that occurs mainly in middle-aged men with sensitivity to gluten, the main protein in wheat flour. It develops when the disease gluten-sensitive enteropathy (celiac sprue) progresses to cancer. A gluten-free diet might prevent this lymphoma.
Hepatosplenic T-cell lymphoma
People with this extremely rare, aggressive disease, which starts in the liver or spleen, experience fever, abdominal pain, weakness and swelling of the liver and spleen. This subtype is mainly seen in children and young men and in people with chronic immune-system suppression, usually due to an organ transplant. There is no standard treatment for the disease. Anthracycline-based chemotherapy is associated with short-term responses in most patients, but complete remissions are rare. Typically allogeneic bone marrow transplant (using a donor’s stem cells) is recommended during the first remission.
Subcutaneous panniculitis-like T-cell lymphoma
People with this rare, aggressive disease usually have small bumps or lesions under their skin. This disease is typically seen in people ages 30 to 50. It is sometimes associated with lupus. It is typically treated with combination chemotherapy.
Peripheral T-cell lymphoma, not otherwise specified
This name is given to T-cell lymphomas that don’t readily fit into any of the other subtypes of PTCL. These lymphomas tend to be widespread and aggressive. Most people diagnosed are in their 60s. Most are treated with aggressive chemotherapy.
This aggressive, difficult-to-treat disease affects the natural killer (NK) cells of the immune system. NK-cells are a type of lymphocyte (a white blood cell) that attacks and destroys viruses and tumor cells. People with this subtype have skin lesions similar to those associated with leukemia, cutaneous lymphomas, and other skin diseases. The disease is rare in the United States but is more common in Asia and parts of Latin America. It usually affects older adults and is associated with Epstein-Barr virus. Because of the severity of this disease, patients should be treated at an academic center that can transition them to a stem cell transplant.