Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
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.

PubMed Disclaimer

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

References

    1. Cohen JI, Kimura H, Nakamura S, Ko YH, Jaffe ES. Epstein–Barr virus-associated lymphoproliferative disease in non-immunocompromised hosts: a status report and summary of an international meeting, 8–9 September 2008. Ann Oncol Off J Eur Soc Med Oncol. 2009;20(9):1472–1482. - PMC - PubMed
    1. Kimura H, Cohen JI. Chronic active Epstein–Barr virus disease. Front Immunol. 2017;8:1867. - PMC - PubMed
    1. Hirai Y, Asada H, Hamada T, Kawada JI, Kimura H, Arai A, et al. Diagnostic and disease severity determination criteria for hydroa vacciniforme lymphoproliferative disorders and severe mosquito bite allergy. J Dermatol. 2023;50(7):e198–e205. - PubMed
    1. Li W. The 5(th) edition of the World Health Organization classification of hematolymphoid tumors. In: Li W, editor. Leukemia. Brisbane (AU): Exon Publications; 2022. - PubMed
    1. Alaggio R, Amador C, Anagnostopoulos I, Attygalle AD, Araujo IBO, Berti E, et al. The 5th edition of the World Health Organization classification of haematolymphoid tumours: lymphoid neoplasms. Leukemia. 2022;36(7):1720–1748. - PMC - PubMed

MeSH terms