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Review
. 2023 Nov;118(5):568-576.
doi: 10.1007/s12185-023-03660-5. Epub 2023 Sep 20.

Updated guidelines for chronic active Epstein-Barr virus disease

Jun-Ichi Kawada  1 , Yoshinori Ito  2 , Koichi Ohshima  3 , Masaki Yamada  4 , Shinsuke Kataoka  5 , Hideki Muramatsu  5 , Akihisa Sawada  6 , Taizo Wada  7 , Ken-Ichi Imadome  4 , Ayako Arai  8 , Keiji Iwatsuki  9 , Shouichi Ohga  10 , Hiroshi Kimura  11 ; Committee for Guidelines for the Management of Chronic Active EBV Disease, Related Disorders (the MHLW Research Team in Japan)
Affiliations
Review

Updated guidelines for chronic active Epstein-Barr virus disease

Jun-Ichi Kawada et al. Int J Hematol. 2023 Nov.

Abstract

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.

Keywords: CAEBV; Diagnostic criteria; EBV; Guidelines.

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Conflict of interest statement

The authors declare that they have no conflict of interest.

Figures

Fig. 1
Fig. 1
Algorithm for the evaluation and treatment of CAEBV. CAEBV, chronic active EBV disease; EBER, EBV-encoded RNA; HSCT, hematopoietic stem cell transplantation
See this image and copyright information in PMC

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