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Chronic Active EBV Disease

MedGen UID:
927324
Concept ID:
C4289792
Disease or Syndrome
Synonyms: CAEBV Infection; Chronic Active EBV Infection; Chronic Active EBV Infection of T-and NK-Cell Type, Systemic Form; Chronic Active Epstein-Barr Virus Disease; Systemic CAEBV of T-and NK-Cell Type; Systemic Chronic Active EBV Disease; Systemic Chronic Active EBV Infection of T-Cell and NK-Cell Type

Definition

An EBV-positive T-cell/NK-cell lymphoproliferative disorder characterized by repeated infectious mononucleosis-like symptoms, a very high titer of anti-EBV antibodies, and high levels of Epstein-Barr virus nucleic acids. Patients often develop progressive cellular and humoral immunodeficiency with pancytopenia and hypogammaglobulinemia. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVChronic Active EBV Disease

Professional guidelines

PubMed

Hirai Y, Asada H, Hamada T, Kawada JI, Kimura H, Arai A, Ohshima K, Ohga S, Iwatsuki K; Committee for Guidelines for the Management of Chronic Active EBV Disease and Related Disorders (the MHLW Research Team in Japan)
J Dermatol 2023 Jul;50(7):e198-e205. Epub 2023 May 30 doi: 10.1111/1346-8138.16842. PMID: 37249004
Cohen JI, Jaffe ES, Dale JK, Pittaluga S, Heslop HE, Rooney CM, Gottschalk S, Bollard CM, Rao VK, Marques A, Burbelo PD, Turk SP, Fulton R, Wayne AS, Little RF, Cairo MS, El-Mallawany NK, Fowler D, Sportes C, Bishop MR, Wilson W, Straus SE
Blood 2011 Jun 2;117(22):5835-49. Epub 2011 Mar 31 doi: 10.1182/blood-2010-11-316745. PMID: 21454450Free PMC Article

Recent clinical studies

Etiology

Luo H, Liu D, Liu W, Jin J, Bi X, Zhang P, Gu J, Zheng M, Xiao M, Liu X, Zhou J, Wang QF
J Allergy Clin Immunol 2023 Apr;151(4):1096-1109. Epub 2022 Nov 21 doi: 10.1016/j.jaci.2022.11.012. PMID: 36423698
Sakaki S, Imadome KI, Kawano F, Nakadate H, Ishiguro A
Pediatr Int 2019 Aug;61(8):825-826. Epub 2019 Aug 21 doi: 10.1111/ped.13935. PMID: 31436003
Willemze R, Cerroni L, Kempf W, Berti E, Facchetti F, Swerdlow SH, Jaffe ES
Blood 2019 Apr 18;133(16):1703-1714. Epub 2019 Jan 11 doi: 10.1182/blood-2018-11-881268. PMID: 30635287Free PMC Article
Pillai V, Tallarico M, Bishop MR, Lim MS
Br J Haematol 2016 May;173(4):573-81. Epub 2016 Mar 15 doi: 10.1111/bjh.14044. PMID: 26992145
Cohen JI
Curr Top Microbiol Immunol 2015;390(Pt 1):241-65. doi: 10.1007/978-3-319-22822-8_10. PMID: 26424649Free PMC Article

Diagnosis

Tomomasa D, Tanita K, Hiruma Y, Hoshino A, Kudo K, Azumi S, Shiota M, Yamaoka M, Eguchi K, Ishimura M, Tanaka Y, Iwatsuki K, Okuno K, Hama A, Sakamoto KI, Taga T, Goto K, Ota H, Ichiki A, Kanda K, Miyamura T, Endo S, Ohnishi H, Sasahara Y, Arai A, Fornier B, Imadome KI, Morio T, Latour S, Kanegane H
Int J Hematol 2024 Aug;120(2):241-251. Epub 2024 May 3 doi: 10.1007/s12185-024-03786-0. PMID: 38700651
Suzuki T, Sato Y, Okuno Y, Torii Y, Fukuda Y, Haruta K, Yamaguchi M, Kawamura Y, Hama A, Narita A, Muramatsu H, Yoshikawa T, Takahashi Y, Kimura H, Ito Y, Kawada JI
J Clin Immunol 2024 Apr 20;44(4):103. doi: 10.1007/s10875-024-01701-0. PMID: 38642164
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
Sakaki S, Imadome KI, Kawano F, Nakadate H, Ishiguro A
Pediatr Int 2019 Aug;61(8):825-826. Epub 2019 Aug 21 doi: 10.1111/ped.13935. PMID: 31436003
Willemze R, Cerroni L, Kempf W, Berti E, Facchetti F, Swerdlow SH, Jaffe ES
Blood 2019 Apr 18;133(16):1703-1714. Epub 2019 Jan 11 doi: 10.1182/blood-2018-11-881268. PMID: 30635287Free PMC Article

Therapy

Pillai V, Tallarico M, Bishop MR, Lim MS
Br J Haematol 2016 May;173(4):573-81. Epub 2016 Mar 15 doi: 10.1111/bjh.14044. PMID: 26992145
Isobe Y, Aritaka N, Setoguchi Y, Ito Y, Kimura H, Hamano Y, Sugimoto K, Komatsu N
J Clin Pathol 2012 Mar;65(3):278-82. Epub 2012 Jan 13 doi: 10.1136/jclinpath-2011-200523. PMID: 22247563
Cohen JI, Jaffe ES, Dale JK, Pittaluga S, Heslop HE, Rooney CM, Gottschalk S, Bollard CM, Rao VK, Marques A, Burbelo PD, Turk SP, Fulton R, Wayne AS, Little RF, Cairo MS, El-Mallawany NK, Fowler D, Sportes C, Bishop MR, Wilson W, Straus SE
Blood 2011 Jun 2;117(22):5835-49. Epub 2011 Mar 31 doi: 10.1182/blood-2010-11-316745. PMID: 21454450Free PMC Article

Prognosis

Tomomasa D, Tanita K, Hiruma Y, Hoshino A, Kudo K, Azumi S, Shiota M, Yamaoka M, Eguchi K, Ishimura M, Tanaka Y, Iwatsuki K, Okuno K, Hama A, Sakamoto KI, Taga T, Goto K, Ota H, Ichiki A, Kanda K, Miyamura T, Endo S, Ohnishi H, Sasahara Y, Arai A, Fornier B, Imadome KI, Morio T, Latour S, Kanegane H
Int J Hematol 2024 Aug;120(2):241-251. Epub 2024 May 3 doi: 10.1007/s12185-024-03786-0. PMID: 38700651
Luo H, Liu D, Liu W, Jin J, Bi X, Zhang P, Gu J, Zheng M, Xiao M, Liu X, Zhou J, Wang QF
J Allergy Clin Immunol 2023 Apr;151(4):1096-1109. Epub 2022 Nov 21 doi: 10.1016/j.jaci.2022.11.012. PMID: 36423698
Sakaki S, Imadome KI, Kawano F, Nakadate H, Ishiguro A
Pediatr Int 2019 Aug;61(8):825-826. Epub 2019 Aug 21 doi: 10.1111/ped.13935. PMID: 31436003
Willemze R, Cerroni L, Kempf W, Berti E, Facchetti F, Swerdlow SH, Jaffe ES
Blood 2019 Apr 18;133(16):1703-1714. Epub 2019 Jan 11 doi: 10.1182/blood-2018-11-881268. PMID: 30635287Free PMC Article
Kimura H
Adv Exp Med Biol 2018;1045:459-475. doi: 10.1007/978-981-10-7230-7_21. PMID: 29896680

Clinical prediction guides

Tomomasa D, Tanita K, Hiruma Y, Hoshino A, Kudo K, Azumi S, Shiota M, Yamaoka M, Eguchi K, Ishimura M, Tanaka Y, Iwatsuki K, Okuno K, Hama A, Sakamoto KI, Taga T, Goto K, Ota H, Ichiki A, Kanda K, Miyamura T, Endo S, Ohnishi H, Sasahara Y, Arai A, Fornier B, Imadome KI, Morio T, Latour S, Kanegane H
Int J Hematol 2024 Aug;120(2):241-251. Epub 2024 May 3 doi: 10.1007/s12185-024-03786-0. PMID: 38700651
Suzuki T, Sato Y, Okuno Y, Torii Y, Fukuda Y, Haruta K, Yamaguchi M, Kawamura Y, Hama A, Narita A, Muramatsu H, Yoshikawa T, Takahashi Y, Kimura H, Ito Y, Kawada JI
J Clin Immunol 2024 Apr 20;44(4):103. doi: 10.1007/s10875-024-01701-0. PMID: 38642164
Climent F, Nicolae A, de Leval L, Dirnhofer S, Leoncini L, Ondrejka SL, Soma L, Wotherspoon A, Zamo A, Quintanilla-Martinez L, Ng SB
Virchows Arch 2023 Sep;483(3):333-348. Epub 2023 Aug 30 doi: 10.1007/s00428-023-03616-4. PMID: 37646869Free PMC Article
Yamada M, Ishikawa Y, Imadome KI
Allergol Int 2021 Oct;70(4):430-438. Epub 2021 Jul 30 doi: 10.1016/j.alit.2021.07.002. PMID: 34334322
Isobe Y, Aritaka N, Setoguchi Y, Ito Y, Kimura H, Hamano Y, Sugimoto K, Komatsu N
J Clin Pathol 2012 Mar;65(3):278-82. Epub 2012 Jan 13 doi: 10.1136/jclinpath-2011-200523. PMID: 22247563

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