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Severe Chronic Active EBV Infection

MedGen UID:
1705966
•Concept ID:
C5206744
•
Disease or Syndrome
Synonym: Severe Chronic Active Epstein-Barr Virus Infection

Definition

A severe form of chronic Epstein-Barr virus infection characterized by continued intermittent fever, lymphadenopathy, thrombocytopenia, lymphocytopenia, neutropenia, polyclonal gammopathy, and greatly elevated titers for antibodies to EBV-related antigens. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVSevere Chronic Active EBV Infection

Professional guidelines

PubMed

Diagnostic and disease severity determination criteria for hydroa vacciniforme lymphoproliferative disorders and severe mosquito bite allergy.
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
Epstein - Barr virus positive T and NK-cell lymphoproliferations: Morphological features and differential diagnosis.
Montes-Mojarro IA, Kim WY, Fend F, Quintanilla-Martinez L
Semin Diagn Pathol 2020 Jan;37(1):32-46. Epub 2019 Dec 17 doi: 10.1053/j.semdp.2019.12.004. PMID: 31889602
EBV-positive diffuse large B-cell lymphoma of the elderly: 2016 update on diagnosis, risk-stratification, and management.
Castillo JJ, Beltran BE, Miranda RN, Young KH, Chavez JC, Sotomayor EM
Am J Hematol 2016 May;91(5):529-37. doi: 10.1002/ajh.24370. PMID: 27093913

Recent clinical studies

Etiology

Proposed categorization of pathological states of EBV-associated T/natural killer-cell lymphoproliferative disorder (LPD) in children and young adults: overlap with chronic active EBV infection and infantile fulminant EBV T-LPD.
Ohshima K, Kimura H, Yoshino T, Kim CW, Ko YH, Lee SS, Peh SC, Chan JK; CAEBV Study Group
Pathol Int 2008 Apr;58(4):209-17. doi: 10.1111/j.1440-1827.2008.02213.x. PMID: 18324913
Clinicopathological states of Epstein-Barr virus-associated T/NK-cell lymphoproliferative disorders (severe chronic active EBV infection) of children and young adults.
Suzuki K, Ohshima K, Karube K, Suzumiya J, Ohga S, Ishihara S, Tamura K, Kikuchi M
Int J Oncol 2004 May;24(5):1165-74. PMID: 15067338
Common Epstein-Barr virus (EBV) type-1 variant strains in both malignant and benign EBV-associated disorders.
Schuster V, Ott G, Seidenspinner S, Kreth HW
Blood 1996 Feb 15;87(4):1579-85. PMID: 8608250

Diagnosis

Proposed categorization of pathological states of EBV-associated T/natural killer-cell lymphoproliferative disorder (LPD) in children and young adults: overlap with chronic active EBV infection and infantile fulminant EBV T-LPD.
Ohshima K, Kimura H, Yoshino T, Kim CW, Ko YH, Lee SS, Peh SC, Chan JK; CAEBV Study Group
Pathol Int 2008 Apr;58(4):209-17. doi: 10.1111/j.1440-1827.2008.02213.x. PMID: 18324913
Severe chronic active EBV infection in an adult patient: case report.
Ha SY, Chung CW, Ko YH
J Korean Med Sci 2004 Jun;19(3):453-7. doi: 10.3346/jkms.2004.19.3.453. PMID: 15201515Free PMC Article
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
SH2D1A mutations in Japanese males with severe Epstein-Barr virus--associated illnesses.
Sumazaki R, Kanegane H, Osaki M, Fukushima T, Tsuchida M, Matsukura H, Shinozaki K, Kimura H, Matsui A, Miyawaki T
Blood 2001 Aug 15;98(4):1268-70. doi: 10.1182/blood.v98.4.1268. PMID: 11493483
Fulminant EBV(+) T-cell lymphoproliferative disorder following acute/chronic EBV infection: a distinct clinicopathologic syndrome.
Quintanilla-Martinez L, Kumar S, Fend F, Reyes E, Teruya-Feldstein J, Kingma DW, Sorbara L, Raffeld M, Straus SE, Jaffe ES
Blood 2000 Jul 15;96(2):443-51. PMID: 10887104

Therapy

Atypical Epstein-Barr virus infection during and after intermittent FK506 therapy.
Azuma H, Sasaki K, Miyamoto K, Tanaka K, Kato H, Uemoto S, Okuno A
Acta Paediatr Jpn 1997 Apr;39(2):222-5. doi: 10.1111/j.1442-200x.1997.tb03586.x. PMID: 9141259

Prognosis

Proposed categorization of pathological states of EBV-associated T/natural killer-cell lymphoproliferative disorder (LPD) in children and young adults: overlap with chronic active EBV infection and infantile fulminant EBV T-LPD.
Ohshima K, Kimura H, Yoshino T, Kim CW, Ko YH, Lee SS, Peh SC, Chan JK; CAEBV Study Group
Pathol Int 2008 Apr;58(4):209-17. doi: 10.1111/j.1440-1827.2008.02213.x. PMID: 18324913
Guillain-Barré syndrome and hemophagocytic lymphohistiocytosis in a patient with severe chronic active Epstein-Barr virus infection syndrome.
Takahashi K, Kunishige M, Shinohara M, Kubo K, Inoue H, Yoshino H, Asano A, Honda S, Matsumoto T, Mitsui T
Clin Neurol Neurosurg 2005 Dec;108(1):80-3. Epub 2004 Dec 24 doi: 10.1016/j.clineuro.2004.11.009. PMID: 16311154
Clinicopathological states of Epstein-Barr virus-associated T/NK-cell lymphoproliferative disorders (severe chronic active EBV infection) of children and young adults.
Suzuki K, Ohshima K, Karube K, Suzumiya J, Ohga S, Ishihara S, Tamura K, Kikuchi M
Int J Oncol 2004 May;24(5):1165-74. PMID: 15067338
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
Fulminant EBV(+) T-cell lymphoproliferative disorder following acute/chronic EBV infection: a distinct clinicopathologic syndrome.
Quintanilla-Martinez L, Kumar S, Fend F, Reyes E, Teruya-Feldstein J, Kingma DW, Sorbara L, Raffeld M, Straus SE, Jaffe ES
Blood 2000 Jul 15;96(2):443-51. PMID: 10887104

Clinical prediction guides

Analysis of IgG subclasses in chronic active Epstein-Barr virus infection.
Wakiguchi H, Hisakawa H, Hosokawa T, Kubota H, Kurashige T
Pediatr Int 2000 Feb;42(1):21-5. doi: 10.1046/j.1442-200x.2000.01175.x. PMID: 10703229
Intact antigen presentation for Epstein-Barr virus (EBV)-specific CTL by a lymphoblastoid cell line established from a patient with severe chronic active EBV infection.
Kimura H, Tsuge I, Imai S, Yamamoto M, Kuzushima K, Osato T, Morishima T
Med Microbiol Immunol 1995 Aug;184(2):63-8. doi: 10.1007/BF00221388. PMID: 7500912
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
Severe chronic active Epstein-Barr virus infection syndrome and adenovirus type-2 infection.
Okano M, Thiele GM, Purtilo DT
Am J Pediatr Hematol Oncol 1990 Summer;12(2):168-73. doi: 10.1097/00043426-199022000-00008. PMID: 2165745

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