Originally published as JCO Early Release 10.1200/JCO.2008.18.2428 on December 8 2008

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Definition, Prognostic Factors, Treatment, and Response Criteria of Adult T-Cell Leukemia-Lymphoma: A Proposal From an International Consensus Meeting

Kunihiro Tsukasaki, Olivier Hermine, Ali Bazarbachi, Lee Ratner, Juan Carlos Ramos, William Harrington, Jr, Deirdre O’Mahony, John E. Janik, Achiléa L. Bittencourt, Graham P. Taylor, Kazunari Yamaguchi, Atae Utsunomiya, Kensei Tobinai, Toshiki Watanabe

From the Nagasaki University, Nagasaki, Japan; Hospital Necker, University Paris V Rene Descartes and CNRS UMR 8147, Paris, France; Department of Internal Medicine, American University of Beirut, Beirut, Lebanon; Washington University, St Louis, MO; National Cancer Institute, Bethesda, MD; Federal University of Bahia, Bahia, Brazil; Imperial College London, London, United Kingdom; National Institute of Infectious Diseases; National Cancer Center Hospital; Tokyo University, Tokyo; and Imamura Bun-in Hospital, Kagoshima, Japan

Corresponding author: Kunihiro Tsukasaki, MD, PhD, Department of Molecular Medicine and Hematology, Molecular Medicine Unit, Atomic Bomb Disease Institute, Nagasaki University Graduate School of Biomedical Science, 1-12-4 Sakamoto, Nagasaki 852-8523, Japan; e-mail: tsukasak{at}net.nagasaki-u.ac.jp

Adult T-cell leukemia-lymphoma (ATL) is a distinct peripheral T-lymphocytic malignancy associated with a retrovirus designated human T-cell lymphotropic virus type I (HTLV-1). The diversity in clinical features and prognosis of patients with this disease has led to its subclassification into the following four categories: acute, lymphoma, chronic, and smoldering types. The chronic and smoldering subtypes are considered indolent and are usually managed with watchful waiting until disease progression, analogous to the management of some patients with chronic lymphoid leukemia (CLL) or other indolent histology lymphomas. Patients with aggressive ATL generally have a poor prognosis because of multidrug resistance of malignant cells, a large tumor burden with multiorgan failure, hypercalcemia, and/or frequent infectious complications as a result of a profound T-cell immunodeficiency. Under the sponsorship of the 13th International Conference on Human Retrovirology: HTLV, a group of ATL researchers joined to form a consensus statement based on established data to define prognostic factors, clinical subclassifications, and treatment strategies. A set of response criteria specific for ATL reflecting a combination of those for lymphoma and CLL was proposed. Clinical subclassification is useful but is limited because of the diverse prognosis among each subtype. Molecular abnormalities within the host genome, such as tumor suppressor genes, may account for these diversities. A treatment strategy based on the clinical subclassification and prognostic factors is suggested, including watchful waiting approach, chemotherapy, antiviral therapy, allogeneic hematopoietic stem-cell transplantation (alloHSCT), and targeted therapies.

published online ahead of print at www.jco.org on December 8, 2008.

Supported in part by the intramural Research Program of the National Cancer Institute, National Institutes of Health.

Presented in part at the 13th International Conference on Human Retrovirology: HTLV, May 22-25, 2007, Hakone, Japan, and the 49th Annual Meeting of the American Society of Hematology, December 8-11, 2007, Atlanta, GA.

Authors’ disclosures of potential conflicts of interest and author contributions are found at the end of this article.

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