Updated guidelines for chronic active Epstein–Barr virus disease

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 diagno...

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Bibliographic Details
Published in:International journal of hematology 2023-11, Vol.118 (5), p.568-576
Main Authors: Kawada, Jun-ichi, Ito, Yoshinori, Ohshima, Koichi, Yamada, Masaki, Kataoka, Shinsuke, Muramatsu, Hideki, Sawada, Akihisa, Wada, Taizo, Imadome, Ken-Ichi, Arai, Ayako, Iwatsuki, Keiji, Ohga, Shouichi, Kimura, Hiroshi
Format: Article
Language:English
Subjects:
Cell proliferation
Chemotherapy
Chronic infection
Copy number
Deoxyribonucleic acid
Diagnosis
Disease control
DNA
Epstein-Barr virus
Genomes
Guidelines
Health services
Hematology
Hematopoietic stem cells
Medicine
Medicine & Public Health
Oncology
Pathogenesis
Review
Review Article
Stem cell transplantation
Stem cells
Therapeutic applications
Transplantation
Viruses
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Summary: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.
ISSN:0925-5710
1865-3774
DOI:10.1007/s12185-023-03660-5