How I treat adult T-cell leukemia/lymphoma

Blood. 2021 Jan 28;137(4):459-470. doi: 10.1182/blood.2019004045.

Abstract

Adult T-cell leukemia/lymphoma (ATL) is a highly aggressive T-cell malignancy that arises in a proportion of individuals who are long-term carriers of human T-lymphotropic virus type 1. The median survival of aggressive subtypes is 8 to 10 months; with chemotherapy-based approaches, overall survival has remained largely unchanged in the ∼35 years since ATL was first described. Through the use of 4 representative case studies, we highlight advances in the biological understanding of ATL and the use of novel therapies such as mogamulizumab, as well as how they are best applied to different subtypes of ATL. We discuss the implementation of molecular methods that may guide diagnosis or treatment, although we accept that these are not universally available. In particular, we acknowledge discrepancies in treatment between different countries, reflecting current drug licensing and the difficulties in making treatment decisions in a rare disease, with limited high-quality clinical trial data.

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Aged
  • Allografts
  • Antibodies, Monoclonal, Humanized / therapeutic use
  • Antineoplastic Agents, Immunological / therapeutic use
  • Antineoplastic Combined Chemotherapy Protocols / administration & dosage
  • Antineoplastic Combined Chemotherapy Protocols / therapeutic use*
  • Combined Modality Therapy
  • Cyclophosphamide / administration & dosage
  • Dexamethasone / administration & dosage
  • Doxorubicin / administration & dosage
  • Etoposide / administration & dosage
  • Female
  • Hematopoietic Stem Cell Transplantation*
  • Human T-lymphotropic virus 1 / physiology
  • Humans
  • Interferon-alpha / administration & dosage
  • Leukemia-Lymphoma, Adult T-Cell / drug therapy
  • Leukemia-Lymphoma, Adult T-Cell / radiotherapy
  • Leukemia-Lymphoma, Adult T-Cell / therapy*
  • Leukemia-Lymphoma, Adult T-Cell / virology
  • Male
  • Methotrexate / administration & dosage
  • Middle Aged
  • Practice Patterns, Physicians'
  • Prednisone / administration & dosage
  • Raltegravir Potassium / administration & dosage
  • Recurrence
  • Remission Induction
  • Therapies, Investigational*
  • Vincristine / administration & dosage
  • Virus Activation
  • Zidovudine / administration & dosage

Substances

  • Antibodies, Monoclonal, Humanized
  • Antineoplastic Agents, Immunological
  • Interferon-alpha
  • Raltegravir Potassium
  • Zidovudine
  • Vincristine
  • Etoposide
  • Dexamethasone
  • Doxorubicin
  • Cyclophosphamide
  • Prednisone
  • mogamulizumab
  • Methotrexate

Supplementary concepts

  • CHOP protocol
  • CVAD protocol
  • EPOCH protocol