慢性活動性 EB ウイルス感染症と その類縁疾患の診 …

1 1 2016 6 19 EB 2016 2 EB CAEBV CQ CQ1 CAEBV EBV CAEBV PCR EBV DNA 1C EBV DNA / g DNA (IU/ g DNA) 2C CQ2 CAEBV CAEBV 2D CQ3 CAEBV 8 2D CQ4 CAEBV CAEBV CAEBV 2C CQ5 CAEBV CAEBV 2C CAEBV 2D CQ6 CAEBV CAEBV 2D EB EBV-HLH CQ CQ7 EBV-HLH EBV

2 EBV-HLH PCR EBV DNA 2C CQ8 EBV FA EIA EBV FA EBV 2D CQ9 EBV-HLH EBV-HLH 2D CQ10 EBV-HLH HLH EBV-HLH 2C CQ11 EBV-HLH A 2C CQ12 EBV-HLH EBV DNA EBV 2C 2C EBV-HLH EBV T/NK EBV EBV 2C 3 CQ13 EBV-HLH 2C 2D CQ CQ14 EBV EBER in situ hybridization PCR EBV DNA 2C EBV DNA / g DNA (IU/ g DNA)

3 2C CQ15 EBV 2C CQ16 2D CQ17 2D CQ18 2D CQ19 2C CQ CQ20 EBV PCR EBV DNA 2C EBV DNA / g DNA (IU/ g DNA)

4 2C CQ21 2C CQ22 2D CQ23 2D CQ24 2D CQ25 2C 4 5 6 7 8 ALT alanine transaminase , GPT AST aspartate aminotransferase , GOT BZLF1 BamHI Z fragment leftward open reading frame 1 CAEBV EB chronic active Epstein-Barr virus infection CD cluster designation CHOP Cyclophosphamide, Hydroxydaunorubicin, Oncovin vincristine.

5 Prednisone CQ clinical question early antigen EBV Epstein-Barr virus EBER Epstein-Barr virus-encoded small RNA EBER-ISH EBER in situ hybridization EBNA Epstein-Barr virus nuclear antigen EBV-HLH EB EBV-associated hemophagocytic lymphohistiocytosis EIA enzyme immunoassay DIC disseminated intravascular coagulation FA fluorescence antibody technique HIV human immunodeficiency virus HLA Human Leukocyte Antigen HLH hemophagocytic lymphohistiocytosis HDL high-density lipoproteins HSCT hematopoietic stem cell transplantation IL-2 2 interleukin-2 ISH in situ hybridization 9 LDH actate dehydrogenase LMP latent membrane protein NK natural killer OS (overall survival)

6 PCR polymerase chain reaction TCR T T- cell receptor THP-COP THP-adriamycin, Cyclophosphamide, Oncovin vincristine, Prednisone TR terminal repeat UVA ultraviolet A VCA viral capsid antigen VLDL very low density lipoprotein XLP X X- linked lymphoproliferative syndrome 10 1 1. ; EB CAEBV SHAKE 11

7 2. EB CAEBV EB EBV-HLH 4 2003 EB CAEBV 3 2014 4 CAEBV 6 8 1 12 Minds 2014

8 : : CAEBV EBV-HLH 1 1 2 3 4 5 6 COI Minds 2014 COI MDS

9 2014 12 1 CAEBV CQ EBV-HLH / 2015 3 24 EB 2015 6 2 / / CQ 8 2015 8 12 25 CQ 2015 12 3 CQ 2016 3 25 EB 2016 4 2016 6 4 13 2016 7 systematic review (SR)/meta-analysis (MA) PubMed Web The Cochran Library CENTRAL SR/MA PubMed The Cochran Library CDSR.

10 National Quality Measures Clearinghouse National Institute for Health and Care Excellence CQ CQ 2015 6 2 4 Minds 2014 Grade (A D 1) 4 1 2 2 2 Grade CAEBV 1 14 1.