TY - JOUR
T1 - Updated guidelines for chronic active Epstein–Barr virus disease
AU - The Committee for Guidelines for the Management of Chronic Active EBV Disease, Related Disorders (the MHLW Research Team in Japan)
AU - Kawada, Jun ichi
AU - Ito, Yoshinori
AU - Ohshima, Koichi
AU - Yamada, Masaki
AU - Kataoka, Shinsuke
AU - Muramatsu, Hideki
AU - Sawada, Akihisa
AU - Wada, Taizo
AU - Imadome, Ken Ichi
AU - Arai, Ayako
AU - Iwatsuki, Keiji
AU - Ohga, Shouichi
AU - Kimura, Hiroshi
N1 - Publisher Copyright:
© 2023, The Author(s).
PY - 2023/11
Y1 - 2023/11
N2 - Chronic active Epstein–Barr virus disease (CAEBV), formerly named chronic active Epstein–Barr virus infection, is characterized by systemic inflammation and clonal proliferation of Epstein–Barr virus (EBV)-infected T or NK cells. As CAEBV is a potentially life-threatening illness, appropriate diagnosis and therapeutic interventions are necessary for favorable clinical outcomes. Substantial evidence regarding the pathogenesis and treatment of CAEBV has been accumulated since previous guidelines for the diagnosis of CAEBV were proposed. To reflect this evidence, we updated the guidelines for the diagnosis and treatment of CAEBV to improve clinical management of the disease. The details of the updated guidelines are presented in this report. Diagnosis of CAEBV now requires confirmation of a high copy number of EBV genome and EBV-infected T or NK cells. An EBV DNA load ≥ 10,000 IU/mL in whole blood is proposed as the diagnostic cutoff value for CAEBV in this updated guideline. A standard treatment approach for CAEBV has not been established, and hematopoietic stem cell transplantation (HSCT) is considered the only curative treatment. Chemotherapy can be administered to control disease activity before HSCT.
AB - Chronic active Epstein–Barr virus disease (CAEBV), formerly named chronic active Epstein–Barr virus infection, is characterized by systemic inflammation and clonal proliferation of Epstein–Barr virus (EBV)-infected T or NK cells. As CAEBV is a potentially life-threatening illness, appropriate diagnosis and therapeutic interventions are necessary for favorable clinical outcomes. Substantial evidence regarding the pathogenesis and treatment of CAEBV has been accumulated since previous guidelines for the diagnosis of CAEBV were proposed. To reflect this evidence, we updated the guidelines for the diagnosis and treatment of CAEBV to improve clinical management of the disease. The details of the updated guidelines are presented in this report. Diagnosis of CAEBV now requires confirmation of a high copy number of EBV genome and EBV-infected T or NK cells. An EBV DNA load ≥ 10,000 IU/mL in whole blood is proposed as the diagnostic cutoff value for CAEBV in this updated guideline. A standard treatment approach for CAEBV has not been established, and hematopoietic stem cell transplantation (HSCT) is considered the only curative treatment. Chemotherapy can be administered to control disease activity before HSCT.
KW - CAEBV
KW - Diagnostic criteria
KW - EBV
KW - Guidelines
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U2 - 10.1007/s12185-023-03660-5
DO - 10.1007/s12185-023-03660-5
M3 - Review article
C2 - 37728704
AN - SCOPUS:85171668829
SN - 0925-5710
VL - 118
SP - 568
EP - 576
JO - International Journal of Hematology
JF - International Journal of Hematology
IS - 5
ER -