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

✍ Scribed by Achille Aouba; Olivier Lambotte; Viorel Vasiliu; Marine Divine; Françoise Valensi; Bruno Varet; Ali Bazarbachi; Olivier Hermine


Publisher
John Wiley and Sons
Year
2004
Tongue
English
Weight
91 KB
Volume
76
Category
Article
ISSN
0361-8609

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✦ Synopsis


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. 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.