MCID: CHR276
MIFTS: 43

Chronic Active Epstein-Barr Virus Infection

Categories: 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 that begins as a primary Epstein-Barr virus (EBV) infection. In this type of infection, the body makes too many lymphocytes (lymphoproliferative disease) for a period of more than 6 months duration. Lymphocytes are a type of white blood cell. They are an important part of the immune system because they help fight off diseases and protect the body from infection by producing antibodies against viruses or bacteria and regulating immune responses. In CAEBV there are many antibodies against EBV in the blood.   Most people (about 95% of adults) get infected with EBV at some point in their lives, and never have any health problems. However, EBV can cause infectious mononucleosis and other illnesses, and has a role in various autoimmune diseases and some types of cancer. While most infections occurring during childhood do not cause any symptoms, EBV infection in adolescents or young adults can often result in mononucleosis. After an EBV infection, the virus becomes latent (inactive) in the body, and, in some cases, the virus may reactivate. This does not always cause symptoms, but people with weakened immune systems are more likely to develop symptoms if EBV reactivates.In rare cases, people infected with EBV develop chronic active EBV virus infection (CAEBV) without apparent immunodeficiency. Most cases of CAEBV have been reported from Japan. These patients have some of the complications found in otherwise-healthy patients with acute EBV infection, but unlike healthy patients, these complications persist and progress. Symptoms of CAEBV most often include fever, liver dysfunction, an enlarged spleen (splenomegaly), swollen lymph nodes (lymphadenopathy), and low numbers of platelets (thrombocytopenia) as well as high EBV-DNA load in the blood. Other features that appear in more than 10% of patients include enlarged liver (hepatomegaly), anemia, hypersensitivity to mosquito bites, rash, oral ulcers, hemophagocytic syndrome, coronary artery aneurysms, liver failure, lymphoma, and interstitial pneumonia. While the cause is yet unknown, researchers have identified defects in T cells or natural killer (NK) cells activity which results in a decreased defense against the EBV in people with CAEBV. It is important to note that the fatigue and malaise from acute infectious mononucleosis (IM) varies from mild symptoms lasting only a few weeks, to more severe symptoms of fatigue that can persist for several months, or even up to a year or more in up to 10% of patients (which may be considered a less severe form of chronic EBV infection).  The persistence of fatigue that is seen in some patients after acute IM would lead some people to believe that EBV may also cause cases of chronic fatigue syndrome (CFS).  However, no convincing link has been found between EBV and CFS. Hematopoietic stem cell transplantation has shown promise in the treatment of CAEBV.  

MalaCards based summary : Chronic Active Epstein-Barr Virus Infection, also known as chronic active epstein-barr disease, is related to chronic fatigue syndrome 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 Cytokine Signaling in Immune system and Toll-like Receptor Signaling Pathway. The drugs alemtuzumab and Fludarabine have been mentioned in the context of this disorder. Affiliated tissues include t cells, nk cells and liver, 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 143)
# Related Disease Score Top Affiliating Genes
1 chronic fatigue syndrome 30.6 IL10 IL2 TGFB1
2 hematopoietic stem cell transplantation 30.3 CTLA4 IFNG IL10 IL2
3 autoimmune lymphoproliferative syndrome 30.3 CTLA4 IL10 IL2
4 uveitis 30.2 CTLA4 IFNG IL10
5 inflammatory bowel disease 28.8 CTLA4 IFNG IL10 IL2 SELE TGFB1
6 systemic lupus erythematosus 28.8 CTLA4 IFNG IL10 IL2 SELE THBD
7 rheumatoid arthritis 27.9 CTLA4 IFNG IL10 IL2 SELE TGFB1
8 variola major 10.8 IFNG IL2
9 autoimmune myocarditis 10.8 IFNG IL2
10 fixed drug eruption 10.8 IFNG IL2
11 berylliosis 10.8 IFNG IL2
12 juvenile myasthenia gravis 10.7 IL10 TGFB1
13 atopic keratoconjunctivitis 10.7 IFNG IL2
14 african tick-bite fever 10.7 IFNG SELE
15 inflammatory bowel disease 25, autosomal recessive 10.7 IL10 TGFB1
16 hyperlucent lung 10.6 IFNG IL10
17 strongyloidiasis 10.6 IFNG TGFB1
18 sporotrichosis 10.6 IFNG IL10
19 eosinophilia-myalgia syndrome 10.5 IFNG TGFB1
20 baylisascariasis 10.5 IFNG IL10
21 crohn's colitis 10.5 IFNG IL2
22 funisitis 10.5 IL10 SELE
23 intermediate uveitis 10.5 IFNG IL10 IL2
24 coccidiosis 10.5 IFNG IL10 IL2
25 lymphoma 10.5
26 lepromatous leprosy 10.5 IFNG IL10 IL2
27 acute graft versus host disease 10.5 IFNG IL10 IL2
28 microscopic colitis 10.5 IFNG IL2
29 herpes zoster 10.4 IFNG IL10 IL2
30 chronic graft versus host disease 10.4 IFNG IL10
31 brucellosis 10.4 IFNG IL10 IL2
32 human immunodeficiency virus infectious disease 10.4 IFNG IL10 IL2
33 leprosy 3 10.4 IFNG IL10 IL2
34 tropical spastic paraparesis 10.4 IFNG IL10 IL2
35 spondyloarthropathy 10.4 IFNG IL10 IL2
36 parasitic protozoa infectious disease 10.4 IFNG IL10 IL2
37 prostatitis 10.4 IL10 IL2 TGFB1
38 primary bacterial infectious disease 10.4 IFNG IL10 IL2
39 pericardial tuberculosis 10.4 IFNG IL10
40 bone inflammation disease 10.4 IFNG IL10 IL2
41 autoimmune disease of endocrine system 10.4 CTLA4 IFNG IL2
42 toxic shock syndrome 10.4 IFNG IL10 IL2
43 schistosomiasis 10.4 IFNG IL10 IL2
44 trichuriasis 10.4 IDO1 IL10
45 tuberculoid leprosy 10.4 IFNG IL10 TGFB1
46 graft-versus-host disease 10.4 IFNG IL10 IL2
47 acquired immunodeficiency syndrome 10.4 IFNG IL10 IL2
48 paranasal sinus disease 10.4 IFNG IL10 TGFB1
49 tetanus 10.4 IFNG IL10 IL2
50 fascioliasis 10.4 IFNG IL10 SELE

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 GBP5 IL2 IDO1 ATR IFNG CTLA4
2 cardiovascular system MP:0005385 10.15 IL10 IDO1 IL2 ATR IFNG CTLA4
3 immune system MP:0005387 10.15 GBP5 IL2 IDO1 ATR IFNG CTLA4
4 homeostasis/metabolism MP:0005376 10.1 CTLA4 IL10 GBP5 IDO1 ATR IFNG
5 endocrine/exocrine gland MP:0005379 10.06 CTLA4 IL10 ATR IFNG TNFAIP6 TGFB1
6 digestive/alimentary MP:0005381 10.02 CTLA4 IL10 ATR IFNG TGFB1 IL2
7 integument MP:0010771 9.87 CTLA4 IL10 ATR IFNG SELE TGFB1
8 liver/biliary system MP:0005370 9.86 IL2 ATR IFNG CTLA4 IL10 SELE
9 normal MP:0002873 9.7 CTLA4 IL10 ATR IFNG SELE THBD
10 reproductive system MP:0005389 9.56 IL10 IDO1 ATR IFNG TNFAIP6 TGFB1
11 respiratory system MP:0005388 9.28 IL2 ATR IFNG CTLA4 IL10 TGFB1

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
alemtuzumab Approved, Investigational Phase 2 216503-57-0
2
Fludarabine Approved Phase 2 21679-14-1, 75607-67-9 30751
3
Prednisolone Approved, Vet_approved Phase 2 50-24-8 5755
4
Melphalan Approved Phase 2 148-82-3 4053 460612
5
Methylprednisolone Approved, Vet_approved Phase 2 83-43-2 6741
6
Prednisone Approved, Vet_approved Phase 2 53-03-2 5865
7 Prednisolone acetate Phase 2
8 Methylprednisolone acetate Phase 2
9 Methylprednisolone Hemisuccinate Phase 2
10 Cyclosporins Phase 2
11 Prednisolone hemisuccinate Phase 2
12 Prednisolone phosphate 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 Recruiting 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
T Cells, Nk Cells, Liver, Lymph Node, Spleen, Skin, Bone Marrow

Publications for Chronic Active Epstein-Barr Virus Infection

Articles related to Chronic Active Epstein-Barr Virus Infection:

(show top 50) (show all 160)
# 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
Whole transcriptome profiling reveals major cell types in the cellular immune response against acute and chronic active Epstein-Barr virus infection. ( 29259291 )
2017
5
Inflammatory cytokine production in chronic active Epstein-Barr virus infection. ( 28381684 )
2017
6
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
7
Chronic active Epstein-Barr virus infection with cutaneous lymphoproliferation: haemophagocytosis in the skin and haemophagocytic syndrome. ( 29024023 )
2017
8
How we treat chronic active Epstein-Barr virus infection. ( 28210942 )
2017
9
FDG-PET/CT findings of chronic active Epstein-Barr virus infection. ( 29022747 )
2017
10
Chronic active Epstein-Barr virus infection as the initial symptom in a Janus kinase 3 deficiency child: Case report and literature review. ( 29049190 )
2017
11
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
12
Virus-specific cytotoxic T cells in chronic active Epstein-Barr virus infection. ( 28679986 )
2017
13
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
14
Chronic active Epstein-Barr virus infection with marked pericardial effusion successfully treated with allogeneic peripheral blood stem cell transplantation. ( 27263789 )
2016
15
Successful Cord Blood Stem Cell Transplantation for an Adult Case of Chronic Active Epstein-Barr Virus Infection. ( 27904117 )
2016
16
Pulmonary arterial hypertension associated with chronic active Epstein-Barr virus infection. ( 25809637 )
2015
17
Recent Concise Viewpoints of Chronic Active Epstein-Barr Virus Infection. ( 25938379 )
2015
18
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
19
Metachronous B-cell and T-cell post-transplant lymphoproliferative disorders with features of chronic active Epstein-Barr virus infection. ( 26178917 )
2015
20
Role of latent membrane protein 1 in chronic active Epstein-Barr virus infection-derived T/NK-cell proliferation. ( 24799376 )
2014
21
Chronic active Epstein-Barr virus infection complicated with multiple artery aneurysms. ( 24939943 )
2014
22
Recurrence of chronic active Epstein-Barr virus infection presenting with myelopathy after umbilical cord blood transplantation. ( 25342015 )
2014
23
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
24
Fatal pulmonary complications in an immunodeficient child with chronic active Epstein-Barr virus infection. ( 24964240 )
2014
25
Current research on chronic active Epstein-Barr virus infection in Japan. ( 24528553 )
2014
26
PCR array analysis of gene expression profiles in chronic active Epstein-Barr virus infection. ( 24801498 )
2014
27
Possible autoimmune hepatitis induced after chronic active Epstein-Barr virus infection. ( 26183510 )
2014
28
Chronic active Epstein-Barr virus infection mimicking autoimmune hepatitis exacerbation in a patient with systemic lupus erythematosus. ( 24608961 )
2014
29
A case of chronic active Epstein-Barr virus infection mimicking adult-onset Still's disease. ( 22370999 )
2013
30
Chronic active Epstein-Barr virus infection: a novel cause of lymphocytic variant hypereosinophilic syndrome. ( 23520331 )
2013
31
Central nervous system complications and neuroradiological findings in children with chronic active Epstein-Barr virus infection. ( 23240920 )
2013
32
Chronic active Epstein-Barr virus infection with progression to large granular lymphocytic leukemia. ( 23731478 )
2013
33
Giant cell arteritis associated with chronic active Epstein-Barr virus infection. ( 23550258 )
2013
34
Plasma viral microRNA profiles reveal potential biomarkers for chronic active Epstein-Barr virus infection. ( 23687223 )
2013
35
An elderly patient with chronic active Epstein-Barr virus infection with mixed cryoglobulinemia and review of the literature. ( 22895834 )
2013
36
Chronic active Epstein-Barr virus infection-associated hydroa vacciniforme-like eruption and BehAset's-like orogenital ulcers. ( 23751255 )
2013
37
Interleukin-17A-producing T lymphocytes in chronic active Epstein-Barr virus infection. ( 23252773 )
2013
38
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
39
Recurrence of chronic active Epstein-Barr virus infection from donor cells after achieving complete response through allogeneic bone marrow transplantation. ( 22466838 )
2012
40
An adult case of severe chronic active Epstein-Barr virus infection with T-cell lymphoproliferative disorder. ( 23269893 )
2012
41
Quantification of Epstein-Barr virus DNA is helpful for evaluation of chronic active Epstein-Barr virus infection. ( 22850617 )
2012
42
Pulmonary arterial hypertension associated with chronic active Epstein-Barr virus infection. ( 21245635 )
2011
43
Elevated expression of activation-induced cytidine deaminase in T and NK cells from patients with chronic active Epstein-Barr virus infection. ( 21697063 )
2011
44
An adult case of chronic active Epstein-Barr virus infection with interstitial pneumonitis. ( 22205850 )
2011
45
Autoimmune lymphoproliferative syndrome mimicking chronic active Epstein-Barr virus infection. ( 21626105 )
2011
46
Aggressive large B-cell lymphoma in a systemic lupus erythematosus patient with chronic active Epstein-Barr virus infection: a case report. ( 22230415 )
2011
47
Clinical features of adult-onset chronic active Epstein-Barr virus infection: a retrospective analysis. ( 21491104 )
2011
48
A case of unilateral optic disc swelling with chronic active Epstein-Barr virus infection. ( 20856592 )
2010
49
Lymphomatoid granulomatosis developing from chronic active Epstein-Barr virus infection in a patient with myelodysplastic syndrome. ( 20345445 )
2010
50
Chronic active Epstein-Barr virus infection mimicking Henoch-SchAPnlein purpura. ( 21245820 )
2010

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 28)
# Super pathways Score Top Affiliating Genes
1
Show member pathways
13.21 GBP1 GBP5 IFNG IL10 IL2 TGFB1
2
Show member pathways
12.81 GBP1 GBP5 IFNG IL10 IL2 TGFB1
3
Show member pathways
12.74 CTLA4 IFNG IL10 IL2 TGFB1
4 12.37 CTLA4 IDO1 IFNG IL10 IL2
5
Show member pathways
12.3 IFNG IL10 IL2 TGFB1
6
Show member pathways
12.13 CTLA4 IFNG IL10 IL2 TGFB1
7
Show member pathways
12.1 IFNG IL10 IL2 TGFB1
8 11.95 IFNG SELE THBD
9
Show member pathways
11.94 IFNG IL10 IL2 TGFB1
10 11.87 IFNG IL2 TGFB1
11 11.78 SELE TGFB1 THBD
12 11.77 IFNG IL10 IL2 LAIR1
13 11.76 IFNG IL10 IL2
14 11.76 IFNG IL10 TGFB1
15 11.72 CTLA4 IFNG TGFB1
16 11.67 IFNG IL2 SELE
17 11.65 IFNG IL10 IL2
18
Show member pathways
11.61 CTLA4 IFNG IL2
19 11.54 IFNG IL2 TGFB1
20
Show member pathways
11.51 IFNG IL2 SELE
21 11.37 IFNG IL2 TGFB1
22 11.33 IFNG IL10 IL2
23 11.25 IFNG IL10 SELE TGFB1
24 11.06 IFNG IL2 TGFB1
25 11 IDO1 IFNG IL10 SELE
26 10.96 IFNG IL10 IL2
27 10.89 IFNG IL10 IL2 TGFB1 THBD
28 10.73 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 16)
# 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 9.56 IDO1 IFNG
8 positive regulation of protein complex assembly GO:0031334 9.55 IFNG TGFB1
9 negative regulation of DNA replication GO:0008156 9.54 ATR TGFB1
10 negative regulation of protein localization to plasma membrane GO:1903077 9.52 GBP1 TGFB1
11 negative regulation of B cell proliferation GO:0030889 9.51 CTLA4 IL10
12 positive regulation of interleukin-17 production GO:0032740 9.48 IL2 TGFB1
13 negative regulation of interleukin-17 production GO:0032700 9.43 IFNG TGFB1
14 positive regulation of regulatory T cell differentiation GO:0045591 9.4 IL2 TGFB1
15 negative regulation of T cell proliferation GO:0042130 9.26 CTLA4 IDO1 IL10 TGFB1
16 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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