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T-cell lymphomas make up less than 15% of non-Hodgkin lymphomas in the United States. These are types of lymphoma that affect T lymphocytes. There are many types of T-cell lymphoma, but they are all fairly rare.
This disease accounts for about 1% of all lymphomas. It's most common in teens or young adults, with males being affected more often than females. It can be considered either a lymphoma or a type of acute lymphoblastic leukemia (ALL), depending on how much of the bone marrow is involved (leukemias have more bone marrow involvement). The cancer cells are very early forms of T cells.
It often starts in the thymus (a small organ behind the breastbone and in front of the heart, which is where many T cells are made), and can grow into a large tumor in the mediastinum (the area between the lungs). This can cause trouble breathing and swelling in the arms and face.
This lymphoma is fast-growing, but if it hasn’t spread to the bone marrow when it is first diagnosed, the chance of curing it with chemotherapy is quite good. Often, the lymphoma form of this disease is treated in the same way as the leukemia form. For more information, see Acute Lymphocytic Leukemia (Adults).
These are uncommon types of lymphoma that develop from more mature forms of T cells.
Cutaneous T-cell lymphomas (mycosis fungoides, Sezary syndrome, and others): These lymphomas start in the skin. Skin lymphomas account for about 5% of all lymphomas. See Lymphoma of the Skin.
Adult T-cell leukemia/lymphoma: This lymphoma is caused by infection with a virus called HTLV-1. It is rare in the United States, and much more common in Japan, the Caribbean, and parts of Africa – where infection with HTLV-1 is more common. It can affect the bone marrow (where new blood cells are made), lymph nodes, spleen, liver, skin, and other organs. There are 4 subtypes:
Angioimmunoblastic T-cell lymphoma: This lymphoma accounts for about 4% of all lymphomas. It is more common in older adults. It tends to involve the lymph nodes and bone marrow as well as the spleen or liver, which can become enlarged. People with this lymphoma usually have fever, weight loss, and skin rashes and often develop infections. This lymphoma often progresses quickly. Treatment is often effective at first, but the lymphoma tends to come back (recur).
Extranodal natural killer/T-cell lymphoma, nasal type: This rare type often involves the upper airway passages, such as the nose and upper throat, but it can also invade the skin, digestive tract, and other organs. It is much more common in parts of Asia and South America. Cells of this lymphoma are similar in some ways to natural killer (NK) cells, another type of lymphocyte.
Enteropathy-associated intestinal T-cell lymphoma (EATL): EATL is a lymphoma that occurs in the lining of the intestine. This lymphoma is most common in the small intestine, but can also occur in the colon. Symptoms can include severe abdominal (belly) pain, nausea, vomiting and bleeding from the intestine.
This lymphoma occurs in some people with celiac disease (also called gluten-sensitive enteropathy). Celiac disease is an autoimmune disease in which eating gluten, a protein found mainly in wheat and barley, causes the immune system to attack the lining of the intestine and other parts of the body. It is rare among people who have had celiac disease since childhood, and is more common in people diagnosed as older adults. This lymphoma is more common in men than women.
Prior to 2016, EATL was divided into 2 subtypes: Type I and Type II. In 2016, the World Health Organization renamed Type II EATL as monomorphic epitheliotropic intestinal T cell lymphoma (MEITL) and considers it a separate disease. MEITL is not linked to celiac disease.
Anaplastic large cell lymphoma (ALCL): About 2% of lymphomas are of this type. It is more common in young people (including children), but it can also affect older adults. This type of lymphoma tends to be fast-growing, but many people with this lymphoma can be cured.
There are different forms of ALCL:
Peripheral T-cell lymphoma, not otherwise specified (PTCL, NOS): This name is given to T-cell lymphomas that don’t readily fit into any of the groups above. Most people diagnosed with these lymphomas are in their 60s. These lymphomas often involve the lymph nodes, but they can affect the skin, bone marrow, spleen, liver, and digestive tract, as well. As a group, these lymphomas tend to be widespread and grow quickly. Some patients respond well to chemotherapy, but over time these lymphomas tend to become harder to treat.
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Freedman AS, Jacobson CA, Mauch P, Aster JC. Chapter 103: Non-Hodgkin’s lymphoma. In: DeVita VT, Lawrence TS, Rosenberg SA, eds. DeVita, Hellman, and Rosenberg’s Cancer: Principles and Practice of Oncology. 10th ed. Philadelphia, Pa: Lippincott Williams & Wilkins; 2015.
National Comprehensive Cancer Network (NCCN)—Central Nervous System Cancers. V1.2018. Accessed April 26, 2018 from https://www.nccn.org/professionals/physician_gls/pdf/cns.pdf
Ondrejka S and Jagadeesh D. Enteropathy-Associated T-Cell Lymphoma. Curr Hematol Malig Rep. 2016;11(6):504-513. doi: 10.1007/s11899-016-0357-7.
Roschewski MJ, Wilson WH. Chapter 106: Non-Hodgkin Lymphoma. In: Niederhuber JE, Armitage JO, Doroshow JH, Kastan MB, Tepper JE, eds. Abeloff’s Clinical Oncology. 5th ed. Philadelphia, Pa: Elsevier; 2014.
Swerdlow SH, Campo, E, Pileri SA et al. The 2016 revision of the World Health Organization classification of lymphoid neoplasms. Blood. 2016; 127:2375-2390; doi: https://doi.org/10.1182/blood-2016-01-643569.
Last Revised: August 1, 2018
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