Hemophagocytic syndrome as a presenting sign of transformation of smoldering to acute adult T-cell leukemia/lymphoma: efficacy of anti-retroviral and interferon therapy

Am J Hematol. 2004 Jun;76(2):187-9. doi: 10.1002/ajh.20065.


A 55-year-old Caribbean woman with a 6-year history of smoldering adult T-cell leukemia/lymphoma presented with clinical and biological symptoms of hemophagocytic syndrome. An extensive search for infectious diseases was negative. A lymph node biopsy showing large T-cell lymphoma (CD4-, CD25+) and findings of high LDH count and severe lymphocytosis led to the diagnosis of acute adult T-cell leukemia/lymphoma. Anti-retroviral therapy combining zidovudine, lamivudine, and interferon-alpha was started, resulting in rapid control of both hemophagocytic syndrome and symptoms of acute adult T-cell leukemia/lymphoma. Thus, we propose that adult T-cell leukemia/lymphoma must be added to the spectrum of etiologies of hemophagocytic syndrome.

Publication types

  • Case Reports

MeSH terms

  • Antineoplastic Agents / therapeutic use
  • Antiviral Agents / therapeutic use*
  • Drug Therapy, Combination
  • Female
  • Histiocytosis / etiology*
  • Humans
  • Interferon-alpha / therapeutic use*
  • Lamivudine / therapeutic use
  • Leukemia-Lymphoma, Adult T-Cell / drug therapy*
  • Leukemia-Lymphoma, Adult T-Cell / pathology*
  • Middle Aged
  • Zidovudine / therapeutic use


  • Antineoplastic Agents
  • Antiviral Agents
  • Interferon-alpha
  • Lamivudine
  • Zidovudine