Non-Hodgkin lymphoma

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Adult T-cell leukemia lymphoma

Adult T-cell leukemia lymphoma (ATLL) can develop in adults who are infected with the human T-cell leukemia lymphoma virus type 1 (HTLV-1). With this type of NHL, the lymphoma cells are often found in the blood, so it is sometimes also called a leukemia.

This type of NHL is uncommon in North America. Most cases of ATLL occur in places where the HTLV-1 virus is more common such as southern Japan, the Caribbean and parts of Africa.

Subtypes of adult T-cell leukemia/lymphoma

There are 4 subtypes of ATLL.

Acute ATLL is the most common subtype. The lymphoma cells are in the blood and the disease acts like an acute leukemia. It grows quickly and affects the skin and bones, as well as the lymph nodes, liver and spleen. Symptoms include:

  • rashes, raised patches or lumps on the skin
  • larger than normal lymph nodes
  • larger than normal liver and spleen
  • higher than normal white blood cell, lymphocyte and T cell counts
  • higher than normal calcium level in the blood
  • unexplained fever, drenching night sweats and unexplained weight loss

Lymphomatous ATLL is rare. It grows quickly, but not as fast as acute ATLL. Lymphomatous ATLL may affect tissues around the brain and spinal cord. It can cause larger than normal lymph nodes, but the numbers of lymphocytes and T cells in the blood are not higher than normal.

Chronic ATLL grows slowly. It can affect the skin, lungs, lymph nodes, liver or spleen. It causes higher than normal numbers of lymphocytes and T cells in the blood.

Smouldering ATLL grows slowly. It can affect the skin or the lungs. It causes abnormal T cell counts, but the number of lymphocytes in the blood is not higher than normal.

The chronic and smouldering subtypes of ATLL usually grow and spread less quickly than the other types, but they may change into acute ATLL.

Treatments

There is no standard treatment plan for ATLL. The treatments offered mainly depend on the subtype of ATLL.

Chemotherapy

Chemotherapy is most often used to treat acute and lymphomatous ATL/L. The most common chemotherapy combination used is CHOP, which is a combination of:

  • cyclophosphamide(Cytoxan, Procytox)
  • doxorubicin(Adriamycin)
  • vincristine(Oncovin)
  • prednisone

Lymphomatous ATLL can affect tissues around the brain and spinal cord, so chemotherapy drugs may be injected directly into the fluid-filled space around the brain and spinal cord (called intrathecal chemotherapy).

Antiretroviral drugs and interferon

Zidovudine (Retrovir, AZT) is an antiretroviral drug commonly used to treat HIV infection. Zidovudine and interferon alfa (Intron A, Wellferon) may be used to treat the HTLV-1 virus. This combination of drugs is used to treat all subtypes of ATLL. It can be effective against this cancer when it doesn’t respond to chemotherapy.

Watchful waiting

Some people with chronic or smouldering ATLL may not need to be treated right away if their only symptom is mild swelling of the lymph nodes. They will be offered watchful waiting (also called active surveillance). The healthcare team will carefully monitor the person with ATL/L and start treatment when symptoms appear or it becomes acute ATLL.

Radiation therapy

Radiation therapy may be used to treat chronic and smouldering ATLL that affects the skin.

Stem cell transplant

A stem cell transplant may be offered to some people with ATLL, especially those who respond to chemotherapy. An allogeneic transplant is the type used most often if there is a suitable donor.

allogeneic

Referring to DNA, cells, tissues or organs taken (harvested) from a donor to be given to a recipient who is a close, but not identical, genetic match.

For example, an allogeneic stem cell transplant takes blood or bone marrow from a donor (usually a first-degree relative) and gives it to a recipient.

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