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Treatment and prognosis of adult T cell leukemia-lymphoma

Masao Matsuoka, MD, PhD
Ritsuro Suzuki, MD, PhD
Section Editor
Arnold S Freedman, MD
Deputy Editor
Alan G Rosmarin, MD


Adult T cell leukemia-lymphoma (ATL) is a peripheral T cell neoplasm associated with infection by the human T-lymphotropic virus, type I (HTLV-1). Although it is considered one of the highly aggressive T cell non-Hodgkin lymphoma variants, the disease course is variable and sometimes quite indolent.

Four clinical variants of ATL have been described: acute, lymphoma-type (lymphomatous), chronic, and smoldering; these appear to have differing genomic alterations and varying clinical courses, and may require different treatment.

The treatment of ATL is discussed here. The epidemiology, pathogenesis, clinical features, pathology, and diagnosis of ATL are discussed separately. (See "Clinical manifestations, pathologic features, and diagnosis of adult T cell leukemia-lymphoma".)


There are four clinical variants of adult T cell leukemia-lymphoma (ATL): acute, lymphoma-type, chronic, and smoldering [1,2]. These categories differ greatly in their presentation and prognosis. Therapy is usually offered to patients with acute, lymphoma-type, or unfavorable chronic-type ATL while patients with typical chronic or smoldering ATL are observed initially. This is principally because conventional chemotherapy does not appear to improve the survival of patients with chronic or smoldering ATL [3,4]. Stratification of patients with chronic and smoldering type ATL into prognostic categories may be useful for identifying those who might benefit from treatment using a risk-adapted therapeutic approach. (See 'Prognosis' below.)

Most patients with acute or lymphoma-type ATL have a survival without treatment measured in days to weeks. A brief description of the clinical variants of ATL is given below. This is described in more detail separately. (See "Clinical manifestations, pathologic features, and diagnosis of adult T cell leukemia-lymphoma".)

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Literature review current through: Dec 2017. | This topic last updated: Jul 27, 2017.
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