Fatal Epstein-Barr virus infection in a case of familial hemophagocytic lymphohistiocytosis with syntaxin-11 mutation.

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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; Zur Stadt, Udo; Koçak, Ulker; Gürsel, Türkiz.

In: TURKISH J PEDIATR, Vol. 51, No. 4, 4, 2009, p. 371-374.

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@article{2cc379d77f984aebaa7c6f34d9c997ec,
title = "Fatal Epstein-Barr virus infection in a case of familial hemophagocytic lymphohistiocytosis with syntaxin-11 mutation.",
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-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.",
author = "Meryem Albayrak and Z{\"u}hre Kaya and Ebru Yilmaz-Keskin and {Zur Stadt}, Udo and Ulker Ko{\c c}ak and T{\"u}rkiz G{\"u}rsel",
year = "2009",
language = "Deutsch",
volume = "51",
pages = "371--374",
journal = "TURKISH J PEDIATR",
issn = "0041-4301",
publisher = "Turkish Journal of Pediatrics",
number = "4",

}

RIS

TY - JOUR

T1 - Fatal Epstein-Barr virus infection in a case of familial hemophagocytic lymphohistiocytosis with syntaxin-11 mutation.

AU - Albayrak, Meryem

AU - Kaya, Zühre

AU - Yilmaz-Keskin, Ebru

AU - Zur Stadt, Udo

AU - Koçak, Ulker

AU - Gürsel, Türkiz

PY - 2009

Y1 - 2009

N2 - 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.

AB - 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.

M3 - SCORING: Zeitschriftenaufsatz

VL - 51

SP - 371

EP - 374

JO - TURKISH J PEDIATR

JF - TURKISH J PEDIATR

SN - 0041-4301

IS - 4

M1 - 4

ER -