2010 Vol. 22No. 159 41 1 EpsteinBarr hemophagocytic lymphohistiocytosishlh EB HLH 3 1 HLHFHL HLH EBV 2 X XLPSAP 3 EBVHLH EB CAEBV 2 T NK EBV HLH 1 HLH HLHFHL 2 HLH EB EB HLH EBVHLH FHLX EBVAHS EBVHLHNK T EBV CAEBV HLH EBVHLH EBVHLH 2 1 EpsteinBarr virusassociated hemophagocytic lymphohistiocytosis and inflammation Key words EB X Akihiko Maeda, Tetsuya Sato, Yoshihito Ishiura, Sumitaka Dohno, Hiroaki Hisakawa, Mikiya Fujieda, Hiroshi Wakiguchi 7838505
60 2010 1HLH 5 2 LDH TG EBV EBV EBER 2EBVHLH 1 EBV 2 LDH TG Tchol DIC EBV 1 EBV viral capsid antigen VCAIgM early antigenea 2 EBV 3 in situ hybridization EBV encoded RNAEBER 1 EBVHLH HLH 3 HLH DIC 3 1 HLHHLH EBV 2 X 3 EBVHLH EB HLH NK
2010 Vol. 22No. 161 1EBV HLH 3HLH EBV NK CTL HLH FHL1 FHL2 FHL3 FHL4 FHL5 GS2 CHS1 XLP 9q21.322 10q2122 17q25 6q24 19p 15q21 1q42.142.2 Xq25 2HLH PFR1 induction of apoptosis UNC13D Vesicle priming STX11 Vesicle transport STXBP2 Vesicle transport RAB27A Vesicle docking LYST Vesicle transport SH2D1A Signal transduction & SAP activation of lymphocytes FHLHFamilial HLH GSGriscelli syndrome CHSChediak Higashi syndrome STX syntaxin STXBP2STX binding protein 2 Munc182 HLH 3 HLH X HLH 1 HLHFHL 15 Griscelli ChediakHigashi 22009 FHL5 2 HLH 1 EBV NK CTL EBV HLH EBV HLH 4 3 EBV B NK CD8 T cytotoxic granule cytotoxic vesicle FHL2
62 2010 4 HLH 3 5Perforin LCMV HLH 4 LYSTRab27AMunc134 cytotoxic vesicle EBV 3 2004 HLH 5 Jordan 4 lymphocytic choriomeningitic virus LCMV HLH LCMV EB CTL LCH CD8 T IFN
2010 Vol. 22No. 163 6EBVHLH LCMV CD8 IFN IFN CD8 T IFN HLH EB Jordan X Xlinked lymphoproliferative syndromexlp XLP EBV EBV HLH EBV SAP SH2D1A 1998 3 57 SAP EB SAP SAP NK T SAP SAP EBV T 8 EBVHLH EBVHLH HLH 20 EB CAEBV 6 EB B T NK EBV T NK TNF IFN IL1IL6IL18 T TNF 9 CAEBV XLP SAP 10 EBVHLH T NK
64 2010 7 EBV EBV HLH HLH CAEBV EBV CD19CD4CD8 CD56 purity 90 DNA PCR EBV Copyg DNA EBV 7 EB IM B B IM T NK purity 100EBV EBV CD4 CD8 NK CD4 CD8 CAEBV IM 10100 11 HLH CD8 CD4 Kasahara 12 CAEBV CD4 T EB HLH CD8 T T NK T latent membrane proteinlmp1 LMP1 NFB SAP XLP 13 LMP1 T TNF 8 TNF Autocrine survival 14
2010 Vol. 22No. 165 8HLH EBV T 14 EBVHLH T EBV HLH EB T T HLH CAEBV In vivo HLH T CD8 in vitro T NK EB B convertant T NK EB 15 T NK In vivo in vitro T EBVHLH HHV6 CD4 HIV EBV HHV6 16 2 EBVHLH HHV6 EBV EBV HHV6 HLH HHV6 EBV EBVHHV6 CD4 T EBV T 2009 11 41 1
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