National Center for Advancing and Translational Sciences Genetic and Rare Diseases Information Center, a program of the National Center for Advancing and Translational Sciences

Adult T-cell leukemia/lymphoma


Other Names:
ATLL; Adult T-cell leukaemia; Adult T-cell leukaemia/lymphoma (HTLV-1 positive); ATLL; Adult T-cell leukaemia; Adult T-cell leukaemia/lymphoma (HTLV-1 positive); Adult T-cell lymphoma See More
Categories:
Adult T-cell leukemia/lymphoma (ATL) is is a rare and aggressive T-cell lymphoma that is linked to infection by the human T-cell lymphotropic virus 1 (HTLV-1).[1][2] The exact mechanism by which HTLV-I infection causes the ATL is unknown. The clinical features of ATL include generalized swelling of the lymph nodes (lymphadenopathy), increased liver and spleen size (hepatosplenomegaly), immunosuppression, high levels of calcium in the blood, lytic bone lesions (spots that appear as “holes” on a standard bone x-ray), and skin lesions.  There are four basic clinical variants of ATL: acute (60% of cases), lymphomatous (20 % of cases), chronic (10% of cases) and smoldering (10% of cases).[1] The best treatment for these patients is unclear and patients should be enrolled in clinical trials whenever possible. Medication may include CHOP chemotherapy (cyclophosphamide, doxorubicin, vincristine and prednisone) or EPOCH (etoposide, vincristine, doxorubicin, cyclophosphamide and prednisone). In some patients, a bone marrow transplant may be recommended.[2][3]
Last updated: 9/13/2016

These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.

In-Depth Information

  • Orphanet is a European reference portal for information on rare diseases and orphan drugs. Access to this database is free of charge.

Questions sent to GARD may be posted here if the information could be helpful to others. We remove all identifying information when posting a question to protect your privacy. If you do not want your question posted, please let us know.

  1. Freedman AS & Aster JC. Clinical manifestations, pathologic features, and diagnosis of adult T cell leukemia-lymphoma. UpToDate. March 29, 2016; http://www.uptodate.com/contents/clinical-manifestations-pathologic-features-and-diagnosis-of-adult-t-cell-leukemia-lymphoma.
  2. O’Connor O A. Getting the Facts. Lymphoma Research Foundation. http://www.lymphoma.org/atf/cf/%7B0363cdd6-51b5-427b-be48-e6af871acec9%7D/htlv.pdf.
  3. Matsuoka M & Tobinai K. Treatment and prognosis of adult T cell leukemia-lymphoma. UpToDate. July 05, 2016; http://www.uptodate.com/contents/treatment-and-prognosis-of-adult-t-cell-leukemia-lymphoma.