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Fatal Epstein-Barr virus infection in a case of familial hemophagocytic lymphohistiocytosis with syntaxin-11 mutation.
Albayrak, Meryem; Kaya, Zühre; Yilmaz-Keskin, Ebru; Stadt, Udo Zur; Koçak, Ulker; Gürsel, Türkiz.
Afiliación
  • Albayrak M; Unit of Pediatric Hematology, Department of Pediatrics, Gazi University Faculty of Medicine, Ankara, Turkey.
Turk J Pediatr ; 51(4): 371-4, 2009.
Article en En | MEDLINE | ID: mdl-19950846
Familial hemophagocytic lymphohistiocytosis (FHL) is a fatal disease of early infancy caused by defective natural killer cell activity and is characterized by fever, organomegaly, pancytopenia, and coagulopathy. Disease-causing mutations have been found in perforin, Munc 13-4 and syntaxin-11 genes. We herein describe a case of late-onset FHL with syntaxin-11 mutation in a six-year-old boy in whom only partial response was obtained by immunochemotherapy (HLH-94 protocol) and who died with persistent Epstein-Barr virus (EBV) infection. The role of EBV infection in the prognosis of FHL is discussed.
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Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Infecciones por Virus de Epstein-Barr / Linfohistiocitosis Hemofagocítica / Proteínas Qa-SNARE Tipo de estudio: Prognostic_studies Límite: Child / Humans / Male Idioma: En Revista: Turk J Pediatr Año: 2009 Tipo del documento: Article País de afiliación: Turquía Pais de publicación: Turquía
Buscar en Google
Añadir a Mi BVS
Imprimir
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PubMed Links

Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Infecciones por Virus de Epstein-Barr / Linfohistiocitosis Hemofagocítica / Proteínas Qa-SNARE Tipo de estudio: Prognostic_studies Límite: Child / Humans / Male Idioma: En Revista: Turk J Pediatr Año: 2009 Tipo del documento: Article País de afiliación: Turquía Pais de publicación: Turquía