CEBV
MCID: CHR276
MIFTS: 43

Chronic Active Epstein-Barr Virus Infection (CEBV)

Categories: Blood diseases, Immune diseases, Rare diseases

Aliases & Classifications for Chronic Active Epstein-Barr Virus Infection

MalaCards integrated aliases for Chronic Active Epstein-Barr Virus Infection:

Name: Chronic Active Epstein-Barr Virus Infection 53
Chronic Active Epstein-Barr Disease 53
Caebv Infection 53
Cebv 53

Classifications:



Summaries for Chronic Active Epstein-Barr Virus Infection

NIH Rare Diseases : 53 Chronic active Epstein-Barr virus infection (CAEBV) is a rare, progressive disease characterized by persistent or intermittent signs and symptoms of infectious mononucleosis ("mono") as well as evidence of prolonged, active EBV infection (identified by specific blood tests). Signs and symptoms may include fever, swollen lymph nodes, enlarged liver and/or spleen, liver function test abnormalities, and low levels of certain types of blood cells in the bloodstream. Some people develop anemia, hypersensitivity to mosquito bites, rash, oral ulcers, neuropathy, liver failure, and/or interstitial pneumonia. Over time, people with CAEBV develop progressive immunodeficiency which, if not treated, can lead to potentially fatal complications such as hemophagocytic syndrome, multiorgan failure, or EBV-positive lymphoma.  CAEBV begins as a primary EBV infection. Most people (about 95% of people by adulthood) become infected with EBV at some point in their lives, and never have any health problems. However, in some people, EBV causes infectious mononucleosis and other illnesses or complications. In rare cases, people with EBV develop CAEBV, where their bodies are unable to control the infection and the virus remains "active," allowing symptoms of EBV infection to persist and progress. It currently is not known why some people develop CAEBV and others do not. CAEBV is diagnosed based on the signs and symptoms and the results of blood tests that show EBV DNA at high levels (signifying an active infection). It is a common misconception that people with fatigue alone (and not the symptoms mentioned above) and positive serologic markers for EBV have CAEBV, as these markers at certain levels are present for life in people with prior EBV exposure, and are not markers of active infection. The only proven effective treatment for CAEBV is hematopoietic stem cell transplantation. If not treated, CAEBV can be fatal.    

MalaCards based summary : Chronic Active Epstein-Barr Virus Infection, also known as chronic active epstein-barr disease, is related to viral infectious disease and hematopoietic stem cell transplantation. An important gene associated with Chronic Active Epstein-Barr Virus Infection is IL2 (Interleukin 2), and among its related pathways/superpathways are Innate Immune System and Cytokine Signaling in Immune system. The drugs Melphalan and Methylprednisolone hemisuccinate have been mentioned in the context of this disorder. Affiliated tissues include liver, t cells and testes, and related phenotypes are hematopoietic system and cardiovascular system

Related Diseases for Chronic Active Epstein-Barr Virus Infection

Diseases related to Chronic Active Epstein-Barr Virus Infection via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 145)
# Related Disease Score Top Affiliating Genes
1 viral infectious disease 32.0 IFNG IL10 IL2
2 hematopoietic stem cell transplantation 30.6 CTLA4 IFNG IL10 IL2
3 hemophagocytic lymphohistiocytosis 30.5 CTLA4 IFNG IL10
4 uveitis 30.4 CTLA4 IFNG IL10
5 autoimmune lymphoproliferative syndrome 30.4 CTLA4 IL10 IL2
6 chronic fatigue syndrome 30.3 IL10 IL2 TGFB1
7 arthritis 30.1 IFNG IL10 TGFB1 TNFAIP6
8 inflammatory bowel disease 29.8 CTLA4 IFNG IL10 IL2 SELE TGFB1
9 systemic lupus erythematosus 29.8 CTLA4 IFNG IL10 IL2 SELE TGFB1
10 rheumatoid arthritis 29.6 CTLA4 IFNG IL10 IL2 SELE TGFB1
11 lymphoma 10.4
12 hepatitis 10.4
13 autoimmune hepatitis 10.3
14 hydroa vacciniforme 10.3
15 pulmonary hypertension, primary, 1 10.2
16 leukemia 10.2
17 lymphocytic leukemia 10.2
18 histiocytosis 10.2
19 myocarditis 10.2
20 vasculitis 10.2
21 lupus erythematosus 10.2
22 encephalitis 10.2
23 polymyositis 10.2
24 virus associated hemophagocytic syndrome 10.2
25 hemophagocytic syndrome associated with an infection 10.2
26 variola major 10.2 IFNG IL2
27 fixed drug eruption 10.2 IFNG IL2
28 cephalosporin allergy 10.2 IFNG IL10
29 juvenile myasthenia gravis 10.2 IL10 TGFB1
30 hyperlucent lung 10.2 IFNG IL10
31 berylliosis 10.2 IFNG IL2
32 sporotrichosis 10.2 IFNG IL10
33 inflammatory bowel disease 25, autosomal recessive 10.2 IL10 TGFB1
34 nickel allergic contact dermatitis 10.2 IFNG IL10
35 gaucher disease, type ii 10.2 IL10 IL2
36 baylisascariasis 10.2 IFNG IL10
37 african tick-bite fever 10.2 IFNG SELE
38 penicillin allergy 10.2 IFNG IL10
39 atopic keratoconjunctivitis 10.2 IFNG IL2
40 autoimmune disease of endocrine system 10.2 CTLA4 IFNG IL2
41 intermediate uveitis 10.2 IFNG IL10 IL2
42 coccidiosis 10.2 IFNG IL10 IL2
43 acute graft versus host disease 10.2 IFNG IL10 IL2
44 lepromatous leprosy 10.2 IFNG IL10 IL2
45 autoimmune uveitis 10.2 IFNG IL10 IL2
46 testicular disease 10.2 IFNG IL10 IL2
47 herpes zoster 10.2 IFNG IL10 IL2
48 scleritis 10.2 IFNG SELE
49 brucellosis 10.2 IFNG IL10 IL2
50 tropical spastic paraparesis 10.2 IFNG IL10 IL2

Graphical network of the top 20 diseases related to Chronic Active Epstein-Barr Virus Infection:



Diseases related to Chronic Active Epstein-Barr Virus Infection

Symptoms & Phenotypes for Chronic Active Epstein-Barr Virus Infection

MGI Mouse Phenotypes related to Chronic Active Epstein-Barr Virus Infection:

46 (show all 11)
# Description MGI Source Accession Score Top Affiliating Genes
1 hematopoietic system MP:0005397 10.17 ATR CTLA4 GBP5 IDO1 IFNG IL10
2 cardiovascular system MP:0005385 10.15 ATR CTLA4 IDO1 IFNG IL10 IL2
3 immune system MP:0005387 10.15 ATR CTLA4 GBP5 IDO1 IFNG IL10
4 homeostasis/metabolism MP:0005376 10.1 ATR CTLA4 GBP5 IDO1 IFNG IL10
5 endocrine/exocrine gland MP:0005379 10.06 ATR CTLA4 IFNG IL10 IL2 LAIR1
6 digestive/alimentary MP:0005381 10.02 ATR CTLA4 IFNG IL10 IL2 TGFB1
7 integument MP:0010771 9.87 ATR CTLA4 IFNG IL10 SELE TGFB1
8 liver/biliary system MP:0005370 9.86 ATR CTLA4 IFNG IL10 IL2 SELE
9 normal MP:0002873 9.7 ATR CTLA4 IFNG IL10 LAIR1 SELE
10 reproductive system MP:0005389 9.56 ATR IDO1 IFNG IL10 IL2 TGFB1
11 respiratory system MP:0005388 9.28 ATR CTLA4 IFNG IL10 IL2 SELE

Drugs & Therapeutics for Chronic Active Epstein-Barr Virus Infection

Drugs for Chronic Active Epstein-Barr Virus Infection (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show all 12)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Melphalan Approved Phase 2 148-82-3 4053 460612
2
Methylprednisolone hemisuccinate Approved Phase 2 2921-57-5
3
Prednisone Approved, Vet_approved Phase 2 53-03-2 5865
4
Fludarabine Approved Phase 2 75607-67-9, 21679-14-1 30751
5
Prednisolone Approved, Vet_approved Phase 2 50-24-8 5755
6
Prednisolone phosphate Approved, Vet_approved Phase 2 302-25-0
7
Methylprednisolone Approved, Vet_approved Phase 2 83-43-2 6741
8
alemtuzumab Approved, Investigational Phase 2 216503-57-0
9
Prednisolone hemisuccinate Experimental Phase 2 2920-86-7
10 Methylprednisolone acetate Phase 2
11 Prednisolone acetate Phase 2
12 Cyclosporins Phase 2

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 Reduced Intensity Conditioning for Hemophagocytic Syndromes or Selected Primary Immune Deficiencies (BMT CTN 1204) Completed NCT01998633 Phase 2
2 CASPALLO: Allodepleted T Cells Transduced With Inducible Caspase 9 Suicide Gene Active, not recruiting NCT00710892 Phase 1
3 Genetic Studies of Chronic Active Epstein-Barr Disease Enrolling by invitation NCT00032513
4 Surveillance and Tracking the Outcomes of Chronic Latent EBV Infection Not yet recruiting NCT03491605

Search NIH Clinical Center for Chronic Active Epstein-Barr Virus Infection

Genetic Tests for Chronic Active Epstein-Barr Virus Infection

Anatomical Context for Chronic Active Epstein-Barr Virus Infection

MalaCards organs/tissues related to Chronic Active Epstein-Barr Virus Infection:

41
Liver, T Cells, Testes, Lymph Node, Spleen, Bone, Lung

Publications for Chronic Active Epstein-Barr Virus Infection

Articles related to Chronic Active Epstein-Barr Virus Infection:

(show top 50) (show all 165)
# Title Authors Year
1
Defining the elusive boundaries of chronic active Epstein-Barr virus infection. ( 29866887 )
2018
2
An Efficient and Simple Method to Establish NK and T Cell Lines from Patients with Chronic Active Epstein-Barr Virus Infection. ( 29658924 )
2018
3
An adult-onset case of chronic active Epstein-Barr virus infection with fulminant clinical course. ( 29426773 )
2018
4
Fatal Chronic Active Epstein-Barr Virus Infection in a Rheumatoid Arthritis Patient Treated with Abatacept. ( 30210119 )
2018
5
STAT3 is constitutively activated in chronic active Epstein-Barr virus infection and can be a therapeutic target. ( 30123428 )
2018
6
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. ( 30285657 )
2018
7
Whole transcriptome profiling reveals major cell types in the cellular immune response against acute and chronic active Epstein-Barr virus infection. ( 29259291 )
2017
8
Inflammatory cytokine production in chronic active Epstein-Barr virus infection. ( 28381684 )
2017
9
Chronic active Epstein-Barr virus infection associated with hemophagocytic syndrome and extra-nodal natural killer/T-cell lymphoma in an 18-year-old girl: A case report. ( 28489771 )
2017
10
Chronic active Epstein-Barr virus infection with cutaneous lymphoproliferation: haemophagocytosis in the skin and haemophagocytic syndrome. ( 29024023 )
2017
11
How we treat chronic active Epstein-Barr virus infection. ( 28210942 )
2017
12
FDG-PET/CT findings of chronic active Epstein-Barr virus infection. ( 29022747 )
2017
13
Chronic active Epstein-Barr virus infection as the initial symptom in a Janus kinase 3 deficiency child: Case report and literature review. ( 29049190 )
2017
14
A distinct subtype of Epstein Barr virus positive T/NK-cell lymphoproliferative disorder: Adult patients with chronic active Epstein Barr virus infection-like features. ( 29242302 )
2017
15
Virus-specific cytotoxic T cells in chronic active Epstein-Barr virus infection. ( 28679986 )
2017
16
Early relapse of severe chronic active Epstein-Barr virus infection with posterior reversible encephalopathy syndrome after reduced intensity stem cell transplantation with umbilical cord blood. ( 26879589 )
2016
17
Chronic active Epstein-Barr virus infection with marked pericardial effusion successfully treated with allogeneic peripheral blood stem cell transplantation. ( 27263789 )
2016
18
Successful Cord Blood Stem Cell Transplantation for an Adult Case of Chronic Active Epstein-Barr Virus Infection. ( 27904117 )
2016
19
Pulmonary arterial hypertension associated with chronic active Epstein-Barr virus infection. ( 25809637 )
2015
20
Recent Concise Viewpoints of Chronic Active Epstein-Barr Virus Infection. ( 25938379 )
2015
21
Chronic active Epstein-Barr virus infection with cutaneous and sinus lymphoproliferation in a white female patient with 25A years' follow-up: an original case report. ( 26148205 )
2015
22
Metachronous B-cell and T-cell post-transplant lymphoproliferative disorders with features of chronic active Epstein-Barr virus infection. ( 26178917 )
2015
23
Role of latent membrane protein 1 in chronic active Epstein-Barr virus infection-derived T/NK-cell proliferation. ( 24799376 )
2014
24
Chronic active Epstein-Barr virus infection complicated with multiple artery aneurysms. ( 24939943 )
2014
25
Recurrence of chronic active Epstein-Barr virus infection presenting with myelopathy after umbilical cord blood transplantation. ( 25342015 )
2014
26
An elderly patient with chronic active Epstein-Barr virus infection with severe hydroa vacciniforme-like eruptions associated with I+I^T-cell proliferation. ( 24628134 )
2014
27
Fatal pulmonary complications in an immunodeficient child with chronic active Epstein-Barr virus infection. ( 24964240 )
2014
28
Current research on chronic active Epstein-Barr virus infection in Japan. ( 24528553 )
2014
29
PCR array analysis of gene expression profiles in chronic active Epstein-Barr virus infection. ( 24801498 )
2014
30
Possible autoimmune hepatitis induced after chronic active Epstein-Barr virus infection. ( 26183510 )
2014
31
Chronic active Epstein-Barr virus infection mimicking autoimmune hepatitis exacerbation in a patient with systemic lupus erythematosus. ( 24608961 )
2014
32
A case of chronic active Epstein-Barr virus infection mimicking adult-onset Still's disease. ( 22370999 )
2013
33
Chronic active Epstein-Barr virus infection: a novel cause of lymphocytic variant hypereosinophilic syndrome. ( 23520331 )
2013
34
Central nervous system complications and neuroradiological findings in children with chronic active Epstein-Barr virus infection. ( 23240920 )
2013
35
Chronic active Epstein-Barr virus infection with progression to large granular lymphocytic leukemia. ( 23731478 )
2013
36
Giant cell arteritis associated with chronic active Epstein-Barr virus infection. ( 23550258 )
2013
37
Plasma viral microRNA profiles reveal potential biomarkers for chronic active Epstein-Barr virus infection. ( 23687223 )
2013
38
An elderly patient with chronic active Epstein-Barr virus infection with mixed cryoglobulinemia and review of the literature. ( 22895834 )
2013
39
Chronic active Epstein-Barr virus infection-associated hydroa vacciniforme-like eruption and BehAset's-like orogenital ulcers. ( 23751255 )
2013
40
Interleukin-17A-producing T lymphocytes in chronic active Epstein-Barr virus infection. ( 23252773 )
2013
41
Epstein-Barr virus-positive cytotoxic T-cell lymphoma followed by chronic active Epstein-Barr virus infection-associated T/NK-cell lymphoproliferative disorder: a case report. ( 24012167 )
2013
42
Recurrence of chronic active Epstein-Barr virus infection from donor cells after achieving complete response through allogeneic bone marrow transplantation. ( 22466838 )
2012
43
An adult case of severe chronic active Epstein-Barr virus infection with T-cell lymphoproliferative disorder. ( 23269893 )
2012
44
Quantification of Epstein-Barr virus DNA is helpful for evaluation of chronic active Epstein-Barr virus infection. ( 22850617 )
2012
45
Pulmonary arterial hypertension associated with chronic active Epstein-Barr virus infection. ( 21245635 )
2011
46
Elevated expression of activation-induced cytidine deaminase in T and NK cells from patients with chronic active Epstein-Barr virus infection. ( 21697063 )
2011
47
An adult case of chronic active Epstein-Barr virus infection with interstitial pneumonitis. ( 22205850 )
2011
48
Autoimmune lymphoproliferative syndrome mimicking chronic active Epstein-Barr virus infection. ( 21626105 )
2011
49
Aggressive large B-cell lymphoma in a systemic lupus erythematosus patient with chronic active Epstein-Barr virus infection: a case report. ( 22230415 )
2011
50
Clinical features of adult-onset chronic active Epstein-Barr virus infection: a retrospective analysis. ( 21491104 )
2011

Variations for Chronic Active Epstein-Barr Virus Infection

Expression for Chronic Active Epstein-Barr Virus Infection

Search GEO for disease gene expression data for Chronic Active Epstein-Barr Virus Infection.

Pathways for Chronic Active Epstein-Barr Virus Infection

Pathways related to Chronic Active Epstein-Barr Virus Infection according to GeneCards Suite gene sharing:

(show all 31)
# Super pathways Score Top Affiliating Genes
1
Show member pathways
13.9 CTLA4 GBP1 GBP5 IFNG IL10 IL2
2
Show member pathways
13.23 GBP1 GBP5 IFNG IL10 IL2 TGFB1
3
Show member pathways
12.84 GBP1 GBP5 IFNG IL10 IL2 TGFB1
4
Show member pathways
12.74 CTLA4 IFNG IL10 IL2 TGFB1
5
Show member pathways
12.46 IFNG IL10 IL2 TGFB1
6 12.41 CTLA4 IDO1 IFNG IL10 IL2
7
Show member pathways
12.17 CTLA4 IFNG IL10 IL2 TGFB1
8
Show member pathways
12.13 IFNG IL10 IL2 TGFB1
9 11.97 IFNG SELE THBD
10
Show member pathways
11.95 IFNG IL10 IL2 TGFB1
11
Show member pathways
11.89 IFNG IL10 IL2 TGFB1
12 11.88 IFNG IL2 TGFB1
13 11.8 IFNG IL10 IL2 LAIR1
14 11.78 SELE TGFB1 THBD
15 11.76 IFNG IL10 IL2
16 11.76 IFNG IL10 TGFB1
17 11.72 CTLA4 IFNG TGFB1
18 11.67 IFNG IL2 SELE
19 11.66 IFNG IL10 IL2
20
Show member pathways
11.62 CTLA4 IFNG IL2
21 11.55 IFNG IL2 TGFB1
22
Show member pathways
11.52 IFNG IL2 SELE
23 11.47 IFNG IL10 IL2 TGFB1
24 11.39 IFNG IL2 TGFB1
25 11.36 IFNG IL10 IL2
26 11.25 IFNG IL10 SELE TGFB1
27 11.08 IFNG IL2 TGFB1
28 11 IFNG IL10 IL2
29 11 IDO1 IFNG IL10 SELE
30 10.89 IFNG IL10 IL2 TGFB1 THBD
31 10.72 IFNG IL10 IL2 TGFB1

GO Terms for Chronic Active Epstein-Barr Virus Infection

Cellular components related to Chronic Active Epstein-Barr Virus Infection according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 extracellular space GO:0005615 9.17 IFNG IL10 IL2 SELE TGFB1 THBD

Biological processes related to Chronic Active Epstein-Barr Virus Infection according to GeneCards Suite gene sharing:

(show all 17)
# Name GO ID Score Top Affiliating Genes
1 immune system process GO:0002376 9.73 CTLA4 GBP1 GBP5 IDO1 IL2 LAIR1
2 negative regulation of inflammatory response GO:0050728 9.67 IL10 IL2 TNFAIP6
3 adaptive immune response GO:0002250 9.67 CTLA4 IFNG IL2 LAIR1
4 female pregnancy GO:0007565 9.63 IDO1 TGFB1 THBD
5 inflammatory response GO:0006954 9.63 GBP5 IDO1 IL10 SELE TGFB1 TNFAIP6
6 response to lipopolysaccharide GO:0032496 9.62 IDO1 IL10 SELE THBD
7 positive regulation of interleukin-12 production GO:0032735 9.57 IDO1 IFNG
8 positive regulation of protein complex assembly GO:0031334 9.56 IFNG TGFB1
9 protein import into nucleus, translocation GO:0000060 9.55 IFNG TGFB1
10 negative regulation of protein localization to plasma membrane GO:1903077 9.54 GBP1 TGFB1
11 negative regulation of B cell proliferation GO:0030889 9.52 CTLA4 IL10
12 negative regulation of immune response GO:0050777 9.51 CTLA4 TGFB1
13 positive regulation of interleukin-17 production GO:0032740 9.48 IL2 TGFB1
14 negative regulation of interleukin-17 production GO:0032700 9.43 IFNG TGFB1
15 positive regulation of regulatory T cell differentiation GO:0045591 9.4 IL2 TGFB1
16 negative regulation of T cell proliferation GO:0042130 9.26 CTLA4 IDO1 IL10 TGFB1
17 regulation of regulatory T cell differentiation GO:0045589 8.92 CTLA4 IFNG IL2 TGFB1

Molecular functions related to Chronic Active Epstein-Barr Virus Infection according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 cytokine activity GO:0005125 8.92 IFNG IL10 IL2 TGFB1

Sources for Chronic Active Epstein-Barr Virus Infection

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 ExPASy
19 FMA
28 GO
29 GTR
30 HGMD
31 HMDB
32 HPO
33 ICD10
34 ICD10 via Orphanet
35 ICD9CM
36 IUPHAR
37 KEGG
38 LifeMap
40 LOVD
42 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
54 NINDS
55 Novoseek
57 OMIM
58 OMIM via Orphanet
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 SNOMED-CT via Orphanet
71 TGDB
72 Tocris
73 UMLS
74 UMLS via Orphanet
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