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- Title
Fatal Epstein-Barr virus infection in a case of familial hemophagocytic lymphohistiocytosis with syntaxin-11 mutation.
- Authors
Albayrak, Meryem; Kaya, Zühre; Yılmaz-Keskin, Ebru; Stadt, Udo Zur; Koçak, Ülker; Gürsel, Türkiz
- Abstract
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-yearold 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.
- Subjects
EPSTEIN-Barr virus diseases; HERPESVIRUS diseases; LYMPHOCYTES; DRUG therapy; KILLER cells; JUVENILE diseases; DISEASES
- Publication
Turkish Journal of Pediatrics, 2009, Vol 51, Issue 4, p371
- ISSN
0041-4301
- Publication type
Case Study