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Non Hodgkin Mature T Cell and NK-Cell neoplasms

To view this article in the new Rare Cancers Australia Knowledgebase, click here 

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. More information can be found here.

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 the HTLV-1 virus is more common. There are 4 subtypes: smoldering, chronic, acute, and lymphoma.

  • The smoldering subtype has abnormal T-cells in the blood without an increased number of lymphocytes in the blood. This lymphoma may involve the skin or lungs, but there is no involvement of other tissues. The smoldering type grows slowly and has a good prognosis.
  • The chronic subtype also grows slowly and has a good prognosis. It has an increase in total lymphocytes and T-cells in the blood. It may involve the skin, lungs, lymph nodes, liver, and/or spleen, but nor other tissues.
  • The acute subtype acts like acute leukemia. It has high lymphocyte and T-cell counts, often along with enlargement of lymph nodes, liver, and spleen. The skin and other organs may be involved with lymphoma as well. Patients often have fever, night sweats, and/or weight loss, as well as certain abnormal blood test results.
  • The lymphoma subtype grows more quickly than the chronic and smoldering types, but not as fast as the acute type. It has enlarged lymph nodes without increased lymphocytes in the blood, and the T-cell count is not high.

Angioimmunoblastic T-cell lymphoma

This lymphoma accounts for about 3% of all lymphomas. It is more common in older adults. It tends to involve the lymph nodes as well as the spleen or liver, which can cause them to be enlarged. Patients 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 and digestive tract. It is much more common in parts of Asia and South America. Cells of this lymphoma are similar in some ways to normal natural killer (NK) cells. NK cells are lymphocytes that can respond to infections more quickly than T-cells and B-cells.

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 jejunum (the second part of the small intestine), but can also occur elsewhere in the small intestine and in the colon. EATL often affects more than one place in the intestine, and may spread to the nearby lymph nodes, as well. It can cause the intestine to become blocked (obstruction) or a hole to develop in the intestine (a perforation). If either of these occur, the symptoms can include severe abdominal (belly) pain, nausea, and vomiting. There are 2 subtypes of this lymphoma.

  • Type I EATL occurs in people with a disease called gluten-sensitive enteropathy (also known as celiac disease, celiac sprue, or sprue). Sprue is an autoimmune disease in which gluten, the main protein in wheat flour, causes the body produce antibodies that attack the lining of the intestine and other parts of the body. Type I EATL is rare among people who have had sprue since childhood, and is more often seen in people diagnosed as adults who have had sprue for a long time. It is most common in people with sprue that hasn’t been well controlled on a gluten free diet. Still, type I EATL can sometimes occur in people who didn’t realize that they had sprue before the lymphoma was found. This lymphoma is more common in men than women, and tends to occur in people in their 60s and 70s. People who do not tolerate gluten, but don’t have sprue, do not seem to have an increased risk of this type of lymphoma.
  • Type II EATL is not linked to sprue and looks different than type I under the microscope. Type II EATL is less common than type I.

Anaplastic large cell lymphoma (ALCL)

About 2% of lymphomas are of this type. It is more common in young people (including children), but it does occur in people in their 50s and 60s. It usually starts in lymph nodes and can also spread to skin. This type of lymphoma tends to be fast-growing, but many people with this lymphoma are cured with aggressive chemotherapy.

The main forms of ALCL are primary cutaneous, which only affects the skin, and systemic. Systemic ALCL is divided into 2 types based on whether a gene change is present in the lymphoma cells that causes them to make a lot of protein called anaplastic lymphoma kinase or ALK1. ALK-positive ALCL tends to occur in younger patients and tends to have a better prognosis (outlook) than the ALK-negative type.

Information has been sourced from

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