title: Hemophagocytic syndrome as a presenting sign of transformation of smoldering to acute adult T-cell leukemia/lymphoma: efficacy of anti-retroviral and interferon therapy.
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-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.
authors.affiliation: Department of Haematology, Hôpital Necker-Enfants Malades, Paris, France. achille.aouba@wanadoo.fr
authors.forename: Achille
authors.initials: A
authors.lastname: Aouba
authors.forename: Olivier
authors.initials: O
authors.lastname: Lambotte
authors.forename: Viorel
authors.initials: V
authors.lastname: Vasiliu
authors.forename: Marine
authors.initials: M
authors.lastname: Divine
authors.forename: Françoise
authors.initials: F
authors.lastname: Valensi
authors.forename: Bruno
authors.initials: B
authors.lastname: Varet
authors.forename: Ali
authors.initials: A
authors.lastname: Bazarbachi
authors.forename: Olivier
authors.initials: O
authors.lastname: Hermine
mesh_terms: D000970:Antineoplastic Agents / Q000627:therapeutic use; D000998:Antiviral Agents / Q000627:therapeutic use*; D004359:Drug Therapy, Combination; D005260:Female; D015614:Histiocytosis / Q000209:etiology*; D006801:Humans; D016898:Interferon-alpha / Q000627:therapeutic use*; D019259:Lamivudine / Q000627:therapeutic use; D015459:Leukemia-Lymphoma, Adult T-Cell / Q000188:drug therapy* / Q000473:pathology*; D008875:Middle Aged; D015215:Zidovudine / Q000627:therapeutic use
chemical_list: D000970:Antineoplastic Agents; D000998:Antiviral Agents; D016898:Interferon-alpha; D019259:Lamivudine; D015215:Zidovudine