Types of T-cell Lymphoma

T-cell lymphomas make up less than 15% of non-Hodgkin lymphomas in the United States. These lymphomas start in early forms of T lymphocytes (T cells). There are many types of T-cell lymphoma, but they are all fairly rare.

T-lymphoblastic lymphoma/leukemia

Lymphoblastic lymphoma/leukemia (also known as T cell acute lymphoblastic leukemia/lymphoma) 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 thought of as either a lymphoblastic lymphoma (LBL) or a type of acute lymphoblastic leukemia (ALL), depending on how much of the bone marrow is involved (leukemias have more cancer cells in the bone marrow). The cancer cells are very early forms of T cells.

This lymphoma 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 there is a good chance of curing it with chemotherapy if it hasn’t spread to the bone marrow when it is first diagnosed. 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).

Peripheral T-cell lymphomas

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. To learn more, 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:

• The smoldering subtype tends to grow slowly. It often has no symptoms, and it tends to have a good prognosis (outlook).
• The chronic subtype also grows slowly and tends to have a good prognosis.
• The acute subtype is the most common. It grows quickly like acute leukemia, so it needs to be treated right away.
• The lymphoma subtype grows more quickly than the chronic and smoldering types, but not as fast as the acute type.

Angioimmunoblastic T-cell lymphoma (AITL): 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, skin rashes, and often develop infections. This lymphoma often progresses quickly. Treatment often works 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. It is most common in the small intestine, but it 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. This lymphoma is rare among people who have had celiac disease since childhood, but more common in people diagnosed as older adults. It is more common in men than women.

Monomorphic epitheliotropic intestinal T cell lymphoma (MEITL): This rare type of lymphoma also affects the lining of the intestines, but it 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:

• Primary cutaneous ALCL only affects the skin. This is discussed in more detail in Lymphoma of the Skin.
• Systemic ALCL can affect the lymph nodes and other organs, including the skin. Systemic ALCL is divided into 2 types based on whether the lymphoma cells have a change in the ALK gene. ALK-positive ALCL is more common in younger people and tends to have a better prognosis (outlook) than the ALK-negative type.
• Breast implant-associated ALCL is a rare type of ALCL that can develop in the breasts of women who have had implants. It seems to be more likely to happen if the implant surfaces are textured (as opposed to smooth).  

Peripheral T-cell lymphoma, not otherwise specified (PTCL, NOS): This name is given to T-cell lymphomas that don’t fit well 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 also affect the skin, bone marrow, spleen, liver, and digestive tract. As a group, these lymphomas tend to spread and grow quickly. Some of these lymphomas respond well to chemotherapy, but over time they tend to become harder to treat.