Abstract:
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. Antiretroviral therapy combining zidovudine, lamivudine, and interferon-α 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. © 2004 Wiley-Liss, Inc.