title: Definition, prognostic factors, treatment, and response criteria of adult T-cell leukemia-lymphoma: a proposal from an international consensus meeting.
abstract: 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.
authors.affiliation: Department of Molecular Medicine and Hematology, Molecular Medicine Unit, Atomic Bomb Disease Institute, Nagasaki University Graduate School of Biomedical Science, Nagasaki, Japan. tsukasak@net.nagasaki-u.ac.jp
authors.forename: Kunihiro
authors.initials: K
authors.lastname: Tsukasaki
authors.forename: Olivier
authors.initials: O
authors.lastname: Hermine
authors.forename: Ali
authors.initials: A
authors.lastname: Bazarbachi
authors.forename: Lee
authors.initials: L
authors.lastname: Ratner
authors.forename: Juan Carlos
authors.initials: JC
authors.lastname: Ramos
authors.forename: William
authors.initials: W
authors.lastname: Harrington
authors.forename: Deirdre
authors.initials: D
authors.lastname: O'Mahony
authors.forename: John E
authors.initials: JE
authors.lastname: Janik
authors.forename: Achiléa L
authors.initials: AL
authors.lastname: Bittencourt
authors.forename: Graham P
authors.initials: GP
authors.lastname: Taylor
authors.forename: Kazunari
authors.initials: K
authors.lastname: Yamaguchi
authors.forename: Atae
authors.initials: A
authors.lastname: Utsunomiya
authors.forename: Kensei
authors.initials: K
authors.lastname: Tobinai
authors.forename: Toshiki
authors.initials: T
authors.lastname: Watanabe
mesh_terms: D000328:Adult; D006801:Humans; D015459:Leukemia-Lymphoma, Adult T-Cell / Q000145:classification / Q000401:mortality / Q000628:therapy; D011379:Prognosis