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 (NHL) 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".)
INDICATIONS FOR TREATMENT
As mentioned above, there are four clinical variants of adult T cell leukemia-lymphoma (ATL): acute, lymphoma-type, chronic, and smoldering [1,2]. These 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]. The five-year survival rate of patients with chronic and smoldering ATL was 47 percent in one study .
As an example, a retrospective evaluation of 26 patients with smoldering ATL reported that 14 (54 percent) were alive without tumor progression at median follow-up of 6.5 years . For those who died, the median time to transformation to a more aggressive variant was 38 months.