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

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‐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. Am. J. Hematol. 76:187–189, 2004. © 2004 Wiley‐Liss, Inc.