Treatment of adult T-cell leukemia/lymphoma: Established paradigms and emerging directions Review


Authors: Stuver, R.; Horwitz, S. M.; Epstein-Peterson, Z. D.
Review Title: Treatment of adult T-cell leukemia/lymphoma: Established paradigms and emerging directions
Abstract: Adult T-cell leukemia/lymphoma (ATL) is a rare, aggressive subtype of peripheral T-cell lymphoma developing after many years of chronic, asymptomatic infection with the retrovirus human T-cell lymphotropic virus type 1 (HTLV-1). HTLV-1 is endemic to certain geographic areas of the world, and primary infection generally occurs in infancy through mother-to-child transmission via breastfeeding. In less than 5% of infected individuals, a decades-long pathogenic process culminates in the development of ATL. Aggressive subtypes of ATL are life-threatening and challenging to treat, with median overall survival typically less than 1 year in the absence of allogeneic hematopoietic cell transplantation (alloHCT). Owing to the rarity of this illness, prospective large-scale clinical trials have been challenging to perform, and treatment recommendations are largely founded upon limited evidence. Herein, we review the current therapeutic options for ATL, providing a broad literature overview of the foremost clinical trials and reports of this disease. We emphasize our own treatment paradigm, which is broadly based upon disease subtype, patient fitness, and intent to perform alloHCT. Finally, we highlight recent advances in understanding ATL disease biology and important ongoing clinical trials that we foresee as informative and potentially practice-changing. © 2023, The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.
Keywords: adult; prospective study; prospective studies; lymphoma; human t-lymphotropic virus 1; leukemia-lymphoma, adult t-cell; peripheral t-cell lymphoma; adult t-cell leukemia/lymphoma; humans; human; female; vertical transmission; mogamulizumab; human t-cell lymphotropic virus; adult t cell leukemia; infectious disease transmission, vertical
Journal Title: Current Treatment Options in Oncology
Volume: 24
Issue: 8
ISSN: 1527-2729
Publisher: Springer  
Date Published: 2023-08-01
Start Page: 948
End Page: 964
Language: English
DOI: 10.1007/s11864-023-01111-1
PUBMED: 37300656
PROVIDER: scopus
DOI/URL:
Notes: Review -- Source: Scopus
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  1. Steven M Horwitz
    645 Horwitz
  2. Robert Nicolais Stuver
    57 Stuver