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.Research output: SCORING: Contribution to journal › SCORING: Journal article › Research › peer-review
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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 -