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Chronic active Epstein-Barr virus infection

MedGen UID:
1695472
•Concept ID:
C5139190
•
Disease or Syndrome
Synonyms: CAEBV infection; Chronic active Epstein-Barr disease; Chronic Epstein Barr virus infection
 
HPO: HP:0032204

Definition

Chronic active Epstein-Barr virus (EBV) infection is an uncommon outcome of EBV infection and may present as a waxing and waning or fulminant syndrome. Unlike acute infectious mononucleosis, wherein EBV establishes lifelong infection and survives by maintaining a delicate balance with the host as a latent infection, in chronic active EBV infection the host-virus balance is disturbed. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVChronic active Epstein-Barr virus infection

Conditions with this feature

X-linked immunodeficiency with magnesium defect, Epstein-Barr virus infection and neoplasia
MedGen UID:
477076
•Concept ID:
C3275445
•
Disease or Syndrome
XMEN is an X-linked recessive immunodeficiency characterized by CD4 (186940) lymphopenia, severe chronic viral infections, and defective T-lymphocyte activation (Li et al., 2011). Affected individuals have chronic Epstein-Barr virus (EBV) infection and are susceptible to the development of EBV-associated B-cell lymphoproliferative disorders. Magnesium supplementation may be therapeutic (summary by Li et al., 2014).
See: Condition Record
X-linked immunodeficiency with magnesium defect, Epstein-Barr virus infection and neoplasia

Professional guidelines

PubMed

Sintilimab treatment for chronic active Epstein-Barr virus infection and Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis in children.
Chen R, Lin Q, Zhu Y, Shen Y, Xu Q, Tang H, Cui N, Jiang L, Dai X, Chen W, Li X
Orphanet J Rare Dis 2023 Sep 22;18(1):297. doi: 10.1186/s13023-023-02861-9. PMID: 37736751Free PMC Article
Outcomes of programmed death protein-1 inhibitors treatment of chronic active Epstein Barr virus infection: A single center retrospective analysis.
Ma Y, Zhang P, Bao Y, Luo H, Wang J, Huang L, Zheng M
Front Immunol 2023;14:1093719. Epub 2023 Mar 10 doi: 10.3389/fimmu.2023.1093719. PMID: 36969150Free PMC Article
Outcome of L-DEP regimen for treatment of pediatric chronic active Epstein-Barr virus infection.
Ma H, Zhang L, Wei A, Yang J, Wang D, Zhang Q, Zhao Y, Chen S, Lian H, Zhang L, Zhou C, Qin M, Li Z, Wang T, Zhang R
Orphanet J Rare Dis 2021 Jun 10;16(1):269. doi: 10.1186/s13023-021-01909-y. PMID: 34112210Free PMC Article

Recent clinical studies

Etiology

Sintilimab treatment for chronic active Epstein-Barr virus infection and Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis in children.
Chen R, Lin Q, Zhu Y, Shen Y, Xu Q, Tang H, Cui N, Jiang L, Dai X, Chen W, Li X
Orphanet J Rare Dis 2023 Sep 22;18(1):297. doi: 10.1186/s13023-023-02861-9. PMID: 37736751Free PMC Article
PD-1 blockade and lenalidomide combination therapy for chronic active Epstein-Barr virus infection.
Song Y, Wang J, Wang Y, Wu L, You Y, Song D, Chen L, Meng G, He X, Cui T, Wang X, Yin Q, Wang Z
Clin Microbiol Infect 2023 Jun;29(6):796.e7-796.e13. Epub 2023 Jan 24 doi: 10.1016/j.cmi.2023.01.017. PMID: 36702399
Clinical Features and Prognostic Factors of Children with Chronic Active Epstein-Barr Virus Infection: A Retrospective Analysis of a Single Center.
Chen S, Wei A, Ma H, Zhang L, Lian H, Zhao Y, Zhang Q, Cheng F, Wang D, Wang T, Li Z, Zhang R
J Pediatr 2021 Nov;238:268-274.e2. Epub 2021 Jul 11 doi: 10.1016/j.jpeds.2021.07.009. PMID: 34260897
Chronic active Epstein-Barr virus infection involving gastrointestinal tract mimicking inflammatory bowel disease.
Xu W, Jiang X, Chen J, Mao Q, Zhao X, Sun X, Zhong L, Rong L
BMC Gastroenterol 2020 Aug 5;20(1):257. doi: 10.1186/s12876-020-01395-9. PMID: 32758149Free PMC Article
Epstein-Barr virus and its association with disease - a review of relevance to general practice.
Fugl A, Andersen CL
BMC Fam Pract 2019 May 14;20(1):62. doi: 10.1186/s12875-019-0954-3. PMID: 31088382Free PMC Article

Diagnosis

Sintilimab treatment for chronic active Epstein-Barr virus infection and Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis in children.
Chen R, Lin Q, Zhu Y, Shen Y, Xu Q, Tang H, Cui N, Jiang L, Dai X, Chen W, Li X
Orphanet J Rare Dis 2023 Sep 22;18(1):297. doi: 10.1186/s13023-023-02861-9. PMID: 37736751Free PMC Article
Updated guidelines for chronic active Epstein-Barr virus disease.
Kawada JI, Ito Y, Ohshima K, Yamada M, Kataoka S, Muramatsu H, Sawada A, Wada T, Imadome KI, Arai A, Iwatsuki K, Ohga S, Kimura H; Committee for Guidelines for the Management of Chronic Active EBV Disease, Related Disorders (the MHLW Research Team in Japan)
Int J Hematol 2023 Nov;118(5):568-576. Epub 2023 Sep 20 doi: 10.1007/s12185-023-03660-5. PMID: 37728704Free PMC Article
Chronic active Epstein-Barr virus: a multidisciplinary approach.
Wauchope J, Dorris CB, Smith CP, Hanna B
BMJ Case Rep 2020 Dec 21;13(12) doi: 10.1136/bcr-2020-236287. PMID: 33370940Free PMC Article
Epstein-Barr virus and its association with disease - a review of relevance to general practice.
Fugl A, Andersen CL
BMC Fam Pract 2019 May 14;20(1):62. doi: 10.1186/s12875-019-0954-3. PMID: 31088382Free PMC Article
Recent Concise Viewpoints of Chronic Active Epstein-Barr Virus Infection.
Okano M
Curr Pediatr Rev 2015;11(1):5-9. doi: 10.2174/1573396311666150501002809. PMID: 25938379

Therapy

Outcomes of programmed death protein-1 inhibitors treatment of chronic active Epstein Barr virus infection: A single center retrospective analysis.
Ma Y, Zhang P, Bao Y, Luo H, Wang J, Huang L, Zheng M
Front Immunol 2023;14:1093719. Epub 2023 Mar 10 doi: 10.3389/fimmu.2023.1093719. PMID: 36969150Free PMC Article
PD-1 blockade and lenalidomide combination therapy for chronic active Epstein-Barr virus infection.
Song Y, Wang J, Wang Y, Wu L, You Y, Song D, Chen L, Meng G, He X, Cui T, Wang X, Yin Q, Wang Z
Clin Microbiol Infect 2023 Jun;29(6):796.e7-796.e13. Epub 2023 Jan 24 doi: 10.1016/j.cmi.2023.01.017. PMID: 36702399
Chronic active Epstein-Barr virus infection with intrapulmonary shunting: A case report.
Wang Z, Zhang Y, Duan M, Zhang Y
J Infect Chemother 2020 May;26(5):502-505. Epub 2020 Jan 23 doi: 10.1016/j.jiac.2019.12.012. PMID: 31983614
Chronic active Epstein-Barr virus infection of T-cell type, systemic form in an African migrant: case report and review of the literature on diagnostics standards and therapeutic options.
Wass M, Bauer M, Pfannes R, Lorenz K, Odparlik A, Müller LP, Wickenhauser C
BMC Cancer 2018 Oct 3;18(1):941. doi: 10.1186/s12885-018-4861-0. PMID: 30285657Free PMC Article
Chronic active Epstein-Barr virus infection as the initial symptom in a Janus kinase 3 deficiency child: Case report and literature review.
Zhong L, Wang W, Ma M, Gou L, Tang X, Song H
Medicine (Baltimore) 2017 Oct;96(42):e7989. doi: 10.1097/MD.0000000000007989. PMID: 29049190Free PMC Article

Prognosis

PD-1 blockade and lenalidomide combination therapy for chronic active Epstein-Barr virus infection.
Song Y, Wang J, Wang Y, Wu L, You Y, Song D, Chen L, Meng G, He X, Cui T, Wang X, Yin Q, Wang Z
Clin Microbiol Infect 2023 Jun;29(6):796.e7-796.e13. Epub 2023 Jan 24 doi: 10.1016/j.cmi.2023.01.017. PMID: 36702399
Chronic active Epstein-Barr virus infection manifesting as coronary artery aneurysm and uveitis.
Xiao H, Hu B, Luo R, Hu H, Zhang J, Kuang W, Zhang R, Li L, Liu G
Virol J 2020 Oct 29;17(1):166. doi: 10.1186/s12985-020-01409-8. PMID: 33121509Free PMC Article
How we treat chronic active Epstein-Barr virus infection.
Sawada A, Inoue M, Kawa K
Int J Hematol 2017 Apr;105(4):406-418. Epub 2017 Feb 16 doi: 10.1007/s12185-017-2192-6. PMID: 28210942
Current research on chronic active Epstein-Barr virus infection in Japan.
Fujiwara S, Kimura H, Imadome K, Arai A, Kodama E, Morio T, Shimizu N, Wakiguchi H
Pediatr Int 2014 Apr;56(2):159-66. doi: 10.1111/ped.12314. PMID: 24528553
Overview and problematic standpoints of severe chronic active Epstein-Barr virus infection syndrome.
Okano M
Crit Rev Oncol Hematol 2002 Dec;44(3):273-82. doi: 10.1016/s1040-8428(02)00118-x. PMID: 12467967

Clinical prediction guides

Outcomes of programmed death protein-1 inhibitors treatment of chronic active Epstein Barr virus infection: A single center retrospective analysis.
Ma Y, Zhang P, Bao Y, Luo H, Wang J, Huang L, Zheng M
Front Immunol 2023;14:1093719. Epub 2023 Mar 10 doi: 10.3389/fimmu.2023.1093719. PMID: 36969150Free PMC Article
PD-1 blockade and lenalidomide combination therapy for chronic active Epstein-Barr virus infection.
Song Y, Wang J, Wang Y, Wu L, You Y, Song D, Chen L, Meng G, He X, Cui T, Wang X, Yin Q, Wang Z
Clin Microbiol Infect 2023 Jun;29(6):796.e7-796.e13. Epub 2023 Jan 24 doi: 10.1016/j.cmi.2023.01.017. PMID: 36702399
Clinical Analysis of Chronic Active Epstein-Barr Virus Infection Involving the Gastrointestinal Tract.
Wei A, Li Z, Ma H, Zhang L, Zhang J, He L, Lian H, Zhang Q, Chen S, Xu J, Wang D, Liu W, Zhang R, Wang T
Pediatr Infect Dis J 2023 Jan 1;42(1):13-19. Epub 2022 Oct 11 doi: 10.1097/INF.0000000000003734. PMID: 36476520
Clinical Features and Prognostic Factors of Children with Chronic Active Epstein-Barr Virus Infection: A Retrospective Analysis of a Single Center.
Chen S, Wei A, Ma H, Zhang L, Lian H, Zhao Y, Zhang Q, Cheng F, Wang D, Wang T, Li Z, Zhang R
J Pediatr 2021 Nov;238:268-274.e2. Epub 2021 Jul 11 doi: 10.1016/j.jpeds.2021.07.009. PMID: 34260897
Severe chronic active Epstein-Barr virus infection syndrome.
Okano M, Matsumoto S, Osato T, Sakiyama Y, Thiele GM, Purtilo DT
Clin Microbiol Rev 1991 Jan;4(1):129-35. doi: 10.1128/CMR.4.1.129. PMID: 1848476Free PMC Article

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