Adult T-cell Leukemia/Lymphoma: A Problem Abroad and at Home

Hematol Oncol Clin North Am. 2017 Apr;31(2):255-272. doi: 10.1016/j.hoc.2016.11.005.


Adult T-cell leukemia/lymphoma (ATLL) is a rare T-cell disorder that is etiologically linked to chronic infection with human T-cell lymphotropic virus type 1. ATLL is divided into four subtypes: acute, lymphomatous, chronic, and smoldering. The acute and lymphomatous variants are often described clinically as the aggressive types of ATLL. Treatment strategies traditionally have focused on antiviral therapy with zidovudine and interferon-alpha and combination chemotherapy. Novel therapeutic approaches include the use of monoclonal antibodies, anti-CCR4 therapy, immunomodulatory therapy, and anti-TAX vaccines. Future research must focus on multi-institutional clinical trial participation because of the rarity of this deadly hematologic malignancy.

Keywords: Aggressive lymphoma; HTLV-1; Hypercalcemia; Leukemia; Lymphoma; Rare cancer; T-cell lymphoma.

Publication types

  • Review

MeSH terms

  • Antibodies, Monoclonal / therapeutic use
  • Antibodies, Neoplasm / therapeutic use
  • Antineoplastic Combined Chemotherapy Protocols / therapeutic use*
  • Human T-lymphotropic virus 1 / metabolism
  • Humans
  • Immunologic Factors / therapeutic use
  • Interferon-alpha / therapeutic use
  • Leukemia-Lymphoma, Adult T-Cell / epidemiology
  • Leukemia-Lymphoma, Adult T-Cell / metabolism
  • Leukemia-Lymphoma, Adult T-Cell / mortality
  • Leukemia-Lymphoma, Adult T-Cell / therapy*
  • Neoplasm Proteins / antagonists & inhibitors
  • Neoplasm Proteins / metabolism
  • Receptors, CCR4 / antagonists & inhibitors
  • Receptors, CCR4 / metabolism
  • Viral Vaccines / therapeutic use*
  • Zidovudine / therapeutic use


  • Antibodies, Monoclonal
  • Antibodies, Neoplasm
  • CCR4 protein, human
  • Immunologic Factors
  • Interferon-alpha
  • Neoplasm Proteins
  • Receptors, CCR4
  • Viral Vaccines
  • Zidovudine