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Non-Hodgkin lymphoma

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Adult T-cell leukemia/lymphoma

Adult T-cell leukemia/lymphoma (ATL/L or ATLL) is uncommon in North America. ATL/L is more common in adults who have been exposed to the human T-cell leukemia/lymphoma virus, type 1 (HTLV-1). Most cases occur in people from southern Japan and the Caribbean, but sporadicsporadic1. Occurring at random or by chance. 2. Occurring at scattered, intermittent or random intervals. cases have occurred elsewhere in the world.

Only a few people who have HTLV-1 go on to develop ATL/L. There is often a long period of time before HTLV-1 develops into ATL/L.

ATL/L is generally an aggressive disease.

Subtypes of ATL/L

There are 4 subtypes of ATL/L. The signs and symptoms and the extent of disease depend on the subtype of ATL/L.

Acute ATL/L

Acute ATL/L is the most common and most aggressive form. With acute ATL/L, lymphoma cells appear in the blood (leukemia form of ATL/L). Other signs or symptoms include:

  • skin lesions – may be rashes, raised patches (plaques), solid raised lesion (papules) or nodules
  • many enlarged lymph nodes (generalized lymphadenopathy)
  • enlarged liver and spleen (hepatosplenomegaly)
  • increased white blood cell count
  • increased calcium level in the blood (hypercalcemia)

Lymphomatous ATL/L

Lymphomatous (lymphoma-type) ATL/L is rare and also behaves aggressively. It presents with enlarged lymph nodes (lymphadenopathy), but there are usually no leukemia cells in the blood. An increased calcium level is less likely to occur with lymphomatous ATL/L than with the acute type.

Chronic ATL/L

Chronic ATL/L often presents with skin lesions and a high white blood cell count. However, unlike the acute type, there is no hypercalcemia and no leukemia cells in the blood.

Smouldering ATL/L

Smouldering ATL/L presents with skin lesions, but the white blood cell count and blood calcium levels are normal.

The chronic and smouldering subtypes of ATL/L usually behave less aggressively than the other 2 types, but may change into the acute form in about 25% of cases.

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Treatment options for ATL/L mainly depend on the subtype of ATL/L the person has.

Watchful waiting

Some people with chronic or smouldering ATL/L may not need to be treated right away if they do not have symptoms or problems (asymptomatic). The person is observed and treatment is not started until the ATL/L causes symptoms or progresses.


People with acute and lymphomatous ATL/L are often treated with chemotherapy. Combinations of chemotherapy drugs used to treat other aggressive types of non-Hodgkin lymphoma (such as CHOP) have been used to treat ATL/L.

  • CHOP – cyclophosphamide (Cytoxan, Procytox), doxorubicin (Adriamycin), vincristine (Oncovin) and prednisone (Deltasone)

ATL/L does not always respond very well to conventional chemotherapy. More intensive combinations of chemotherapy may be tried.

Antiretroviral and biological therapy drugs

Other drugs may be used to treat ATL/L. Zidovudine (Retrovir, AZT) is an antiretroviral drug used to treat HIV infection. Zidovudine and biological therapy drugs like interferon alfa (Intron A, Wellferon) may be used to treat ATL/L and have been effective in people who do not respond to chemotherapy. These 2 drugs are often used together.

Stem cell transplant

A stem cell transplant (SCT) may be offered to some people with ATL/L, especially those who respond to chemotherapy. Most often an allogeneic transplant is used in these cases if there is a suitable donor.

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For more detailed information on specific drugs, go to sources of drug information.


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