Abstract
Adult T-cell leukemia–lymphoma (ATL), a rare and aggressive T-cell malignancy caused by human T-cell lymphotropic virus type 1 (HTLV-1), is associated with a poor prognosis. Evidence-based standard treatment options are lacking and outcomes are generally unsatisfactory, particularly for patients with relapsed or refractory disease. Continued research is contributing to changing treatment landscape as a number of existing and investigational agents are evaluated. We describe the epidemiology of HTLV-1 and ATL, discuss the biology behind the disease, review current treatment practices and guidelines, and provide an overview of emerging therapies in ATL, with a focus on those for relapsed or refractory disease.
Highlights
Adult T-cell leukemia–lymphoma (ATL) is a rare and aggressive peripheral T-cell neoplasm caused by human T-cell lymphotropic virus type 1 (HTLV-1) [1, 2]
The purpose of this review is to provide an overview of ATL and a brief review of current treatment guidelines, and to discuss emerging
Patients with ATL are rarely cured with currently available cytotoxic drugs
Summary
Adult T-cell leukemia–lymphoma (ATL) is a rare and aggressive peripheral T-cell neoplasm caused by human T-cell lymphotropic virus type 1 (HTLV-1) [1, 2]. ATL can present with diverse clinical features, but typically is associated with circulating leukemic cells, generalized lymph node swelling, hepatosplenomegaly, skin involvement, opportunistic infections, and hypercalcemia [3]. ATL generally has a poor prognosis, with shorter overall survival (OS) relative to other peripheral T-cell lymphomas (PTCLs) [4]. Factors contributing to poor outcomes include inherent chemoresistance and immunosuppression associated with ATL, with aggressive forms [5]. Progress has been made in understanding the biologic underpinnings of ATL, treatment outcomes generally remain unsatisfactory. The purpose of this review is to provide an overview of ATL (biology, epidemiology, diagnosis, and prognosis) and a brief review of current treatment guidelines, and to discuss emerging
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