MCID: CHR276
MIFTS: 44

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 49
Chronic Active Epstein-Barr Disease 49
Caebv Infection 49
Cebv 49

Classifications:



Summaries for Chronic Active Epstein-Barr Virus Infection

NIH Rare Diseases : 49 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.   Last updated: 8/22/2017

MalaCards based summary : Chronic Active Epstein-Barr Virus Infection, also known as chronic active epstein-barr disease, is related to alpha/beta t-cell lymphopenia with gamma/delta t-cell expansion, severe cytomegalovirus infection, and autoimmunity and hematopoietic stem cell transplantation. An important gene associated with Chronic Active Epstein-Barr Virus Infection is LAIR1 (Leukocyte Associated Immunoglobulin Like Receptor 1), and among its related pathways/superpathways are Cytokine Signaling in Immune system and Allograft rejection. The drugs Melphalan 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 141)
# Related Disease Score Top Affiliating Genes
1 alpha/beta t-cell lymphopenia with gamma/delta t-cell expansion, severe cytomegalovirus infection, and autoimmunity 30.7 CTLA4 IFNG IL10
2 hematopoietic stem cell transplantation 30.3 CTLA4 IFNG IL10 IL2
3 chronic fatigue syndrome 30.3 IL10 IL2 TGFB1
4 autoimmune lymphoproliferative syndrome 30.1 CTLA4 IL10 IL2
5 uveitis 30.1 CTLA4 IFNG IL10
6 rheumatoid arthritis 29.0 CTLA4 IFNG IL10 IL2 TGFB1 TNFAIP6
7 systemic lupus erythematosus 29.0 CTLA4 IFNG IL10 IL2 SELE TGFB1
8 inflammatory bowel disease 28.9 CTLA4 IDO1 IFNG IL10 IL2 SELE
9 variola major 10.5 IFNG IL2
10 autoimmune myocarditis 10.5 IFNG IL2
11 lymphoma 10.5
12 berylliosis 10.5 IFNG IL2
13 juvenile myasthenia gravis 10.4 IL10 TGFB1
14 hyperlucent lung 10.4 IFNG IL10
15 atopic keratoconjunctivitis 10.4 IFNG IL2
16 african tick-bite fever 10.4 IFNG SELE
17 autoimmune lymphoproliferative syndrome, type v 10.4
18 sporotrichosis 10.3 IFNG IL10
19 strongyloidiasis 10.3 IFNG TGFB1
20 baylisascariasis 10.3 IFNG IL10
21 eosinophilia-myalgia syndrome 10.3 IFNG TGFB1
22 hepatitis 10.3
23 allergic hypersensitivity disease 10.3
24 intermediate uveitis 10.3 IFNG IL10 IL2
25 coccidiosis 10.3 IFNG IL10 IL2
26 funisitis 10.3 IL10 SELE
27 lepromatous leprosy 10.3 IFNG IL10 IL2
28 acute graft versus host disease 10.3 IFNG IL10 IL2
29 herpes zoster 10.3 IFNG IL10 IL2
30 brucellosis 10.3 IFNG IL10 IL2
31 leprosy 3 10.3 IFNG IL10 IL2
32 tropical spastic paraparesis 10.3 IFNG IL10 IL2
33 human immunodeficiency virus infectious disease 10.3 IFNG IL10 IL2
34 spondyloarthropathy 10.3 IFNG IL10 IL2
35 parasitic protozoa infectious disease 10.3 IFNG IL10 IL2
36 primary bacterial infectious disease 10.3 IFNG IL10 IL2
37 bone inflammation disease 10.3 IFNG IL10 IL2
38 autoimmune disease of endocrine system 10.3 CTLA4 IFNG IL2
39 toxic shock syndrome 10.2 IFNG IL10 IL2
40 chronic graft versus host disease 10.2 IFNG IL10
41 schistosomiasis 10.2 IFNG IL10 IL2
42 tuberculoid leprosy 10.2 IFNG IL10 TGFB1
43 graft-versus-host disease 10.2 IFNG IL10 IL2
44 acquired immunodeficiency syndrome 10.2 IFNG IL10 IL2
45 thyroiditis 10.2 CTLA4 IFNG IL2
46 paranasal sinus disease 10.2 IFNG IL10 TGFB1
47 trichuriasis 10.2 IDO1 IL10
48 fascioliasis 10.2 IFNG IL10 SELE
49 autoimmune hepatitis 10.2
50 hydroa vacciniforme 10.2

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:

43 (show all 11)
# Description MGI Source Accession Score Top Affiliating Genes
1 hematopoietic system MP:0005397 10.17 THBD LAIR1 SELE TGFB1 GBP5 IL2
2 cardiovascular system MP:0005385 10.15 TGFB1 THBD SELE IDO1 IL2 ATR
3 immune system MP:0005387 10.15 THBD LAIR1 TNFAIP6 SELE TGFB1 GBP5
4 homeostasis/metabolism MP:0005376 10.1 TGFB1 IL2 THBD SELE IL10 GBP5
5 endocrine/exocrine gland MP:0005379 10.06 TGFB1 IL2 LAIR1 TNFAIP6 ATR IFNG
6 digestive/alimentary MP:0005381 10.02 TGFB1 IL2 THBD ATR IFNG CTLA4
7 integument MP:0010771 9.87 TGFB1 THBD SELE ATR IFNG CTLA4
8 liver/biliary system MP:0005370 9.86 THBD SELE TGFB1 IL2 ATR IFNG
9 normal MP:0002873 9.7 THBD LAIR1 SELE CTLA4 IL10 ATR
10 reproductive system MP:0005389 9.56 TGFB1 IL2 THBD TNFAIP6 IDO1 ATR
11 respiratory system MP:0005388 9.28 THBD TNFAIP6 SELE TGFB1 IL2 ATR

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 460612 4053
2
Fludarabine Approved Phase 2 21679-14-1, 75607-67-9 30751
3
Prednisolone Approved, Vet_approved Phase 2 50-24-8 5755
4
alemtuzumab Approved, Investigational Phase 2 216503-57-0
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) Active, not recruiting 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

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:

38
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 157)
# Title Authors Year
1
An adult-onset case of chronic active Epstein-Barr virus infection with fulminant clinical course. ( 29426773 )
2018
2
How we treat chronic active Epstein-Barr virus infection. ( 28210942 )
2017
3
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
4
Chronic active Epstein-Barr virus infection with cutaneous lymphoproliferation: haemophagocytosis in the skin and haemophagocytic syndrome. ( 29024023 )
2017
5
Chronic active Epstein-Barr virus infection as the initial symptom in a Janus kinase 3 deficiency child: Case report and literature review. ( 29049190 )
2017
6
Whole transcriptome profiling reveals major cell types in the cellular immune response against acute and chronic active Epstein-Barr virus infection. ( 29259291 )
2017
7
Virus-specific cytotoxic T cells in chronic active Epstein-Barr virus infection. ( 28679986 )
2017
8
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
9
FDG-PET/CT findings of chronic active Epstein-Barr virus infection. ( 29022747 )
2017
10
Inflammatory cytokine production in chronic active Epstein-Barr virus infection. ( 28381684 )
2017
11
Successful Cord Blood Stem Cell Transplantation for an Adult Case of Chronic Active Epstein-Barr Virus Infection. ( 27904117 )
2016
12
Chronic active Epstein-Barr virus infection with marked pericardial effusion successfully treated with allogeneic peripheral blood stem cell transplantation. ( 27263789 )
2016
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
Recent Concise Viewpoints of Chronic Active Epstein-Barr Virus Infection. ( 25938379 )
2015
15
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
16
Pulmonary arterial hypertension associated with chronic active Epstein-Barr virus infection. ( 25809637 )
2015
17
Metachronous B-cell and T-cell post-transplant lymphoproliferative disorders with features of chronic active Epstein-Barr virus infection. ( 26178917 )
2015
18
Role of latent membrane protein 1 in chronic active Epstein-Barr virus infection-derived T/NK-cell proliferation. ( 24799376 )
2014
19
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
20
Possible autoimmune hepatitis induced after chronic active Epstein-Barr virus infection. ( 26183510 )
2014
21
Chronic active Epstein-Barr virus infection complicated with multiple artery aneurysms. ( 24939943 )
2014
22
Current research on chronic active Epstein-Barr virus infection in Japan. ( 24528553 )
2014
23
Recurrence of chronic active Epstein-Barr virus infection presenting with myelopathy after umbilical cord blood transplantation. ( 25342015 )
2014
24
Fatal pulmonary complications in an immunodeficient child with chronic active Epstein-Barr virus infection. ( 24964240 )
2014
25
PCR array analysis of gene expression profiles in chronic active Epstein-Barr virus infection. ( 24801498 )
2014
26
Chronic active Epstein-Barr virus infection mimicking autoimmune hepatitis exacerbation in a patient with systemic lupus erythematosus. ( 24608961 )
2014
27
Chronic active Epstein-Barr virus infection with progression to large granular lymphocytic leukemia. ( 23731478 )
2013
28
Chronic active Epstein-Barr virus infection-associated hydroa vacciniforme-like eruption and BehAset's-like orogenital ulcers. ( 23751255 )
2013
29
Chronic active Epstein-Barr virus infection: a novel cause of lymphocytic variant hypereosinophilic syndrome. ( 23520331 )
2013
30
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
31
A case of chronic active Epstein-Barr virus infection mimicking adult-onset Still's disease. ( 22370999 )
2013
32
Giant cell arteritis associated with chronic active Epstein-Barr virus infection. ( 23550258 )
2013
33
Central nervous system complications and neuroradiological findings in children with chronic active Epstein-Barr virus infection. ( 23240920 )
2013
34
Plasma viral microRNA profiles reveal potential biomarkers for chronic active Epstein-Barr virus infection. ( 23687223 )
2013
35
Interleukin-17A-producing T lymphocytes in chronic active Epstein-Barr virus infection. ( 23252773 )
2013
36
An elderly patient with chronic active Epstein-Barr virus infection with mixed cryoglobulinemia and review of the literature. ( 22895834 )
2013
37
Recurrence of chronic active Epstein-Barr virus infection from donor cells after achieving complete response through allogeneic bone marrow transplantation. ( 22466838 )
2012
38
An adult case of severe chronic active Epstein-Barr virus infection with T-cell lymphoproliferative disorder. ( 23269893 )
2012
39
Quantification of Epstein-Barr virus DNA is helpful for evaluation of chronic active Epstein-Barr virus infection. ( 22850617 )
2012
40
Elevated expression of activation-induced cytidine deaminase in T and NK cells from patients with chronic active Epstein-Barr virus infection. ( 21697063 )
2011
41
Clinical features of adult-onset chronic active Epstein-Barr virus infection: a retrospective analysis. ( 21491104 )
2011
42
Pulmonary arterial hypertension associated with chronic active Epstein-Barr virus infection. ( 21245635 )
2011
43
Aggressive large B-cell lymphoma in a systemic lupus erythematosus patient with chronic active Epstein-Barr virus infection: a case report. ( 22230415 )
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
Lymphomatoid granulomatosis developing from chronic active Epstein-Barr virus infection in a patient with myelodysplastic syndrome. ( 20345445 )
2010
47
A case of unilateral optic disc swelling with chronic active Epstein-Barr virus infection. ( 20856592 )
2010
48
Chronic active Epstein-Barr virus infection mimicking Henoch-SchAPnlein purpura. ( 21245820 )
2010
49
M-protein-positive chronic active Epstein-Barr virus infection: features mimicking HIV-1 infection. ( 19588219 )
2009
50
Cardiovascular complications associated with chronic active Epstein-Barr virus infection. ( 19184184 )
2009

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 30)
# Super pathways Score Top Affiliating Genes
1
Show member pathways
13.23 GBP1 GBP5 IFNG IL10 IL2 TGFB1
2
Show member pathways
12.71 CTLA4 IFNG IL10 IL2 TGFB1
3
Show member pathways
12.46 IFNG IL10 IL2 TGFB1
4
Show member pathways
12.41 IFNG IL10 IL2 TGFB1
5 12.41 CTLA4 IDO1 IFNG IL10 IL2
6
Show member pathways
12.18 CTLA4 IFNG IL10 IL2 TGFB1
7
Show member pathways
12.02 IFNG IL10 IL2
8 11.96 IFNG SELE THBD
9 11.88 IFNG IL2 TGFB1
10 11.84 IFNG IL10 IL2 LAIR1
11 11.78 SELE TGFB1 THBD
12 11.76 IFNG IL10 TGFB1
13 11.73 IFNG IL10 IL2
14 11.73 CTLA4 IFNG TGFB1
15
Show member pathways
11.72 IFNG IL10 IL2 TGFB1
16 11.68 IFNG IL2 SELE
17 11.66 IFNG IL10 IL2
18
Show member pathways
11.63 CTLA4 IFNG IL2
19 11.55 CTLA4 IL10 TGFB1
20 11.54 IFNG IL2 TGFB1
21
Show member pathways
11.51 IFNG IL2 SELE
22 11.38 IFNG IL2 TGFB1
23 11.35 IFNG IL10 IL2
24 11.31 IFNG IL10 SELE TGFB1
25
Show member pathways
11.11 IFNG IL10 IL2 TGFB1
26 11.08 IFNG IL2 TGFB1
27 11.02 IFNG IL10 IL2
28 11 IDO1 IFNG IL10 SELE
29 10.89 IFNG IL10 IL2 TGFB1 THBD
30 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 adaptive immune response GO:0002250 9.67 CTLA4 IFNG IL2 LAIR1
3 female pregnancy GO:0007565 9.65 IDO1 TGFB1 THBD
4 negative regulation of inflammatory response GO:0050728 9.63 IL10 IL2 TNFAIP6
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 protein complex assembly GO:0031334 9.56 IFNG TGFB1
8 negative regulation of DNA replication GO:0008156 9.55 ATR TGFB1
9 negative regulation of protein localization to plasma membrane GO:1903077 9.54 GBP1 TGFB1
10 negative regulation of B cell proliferation GO:0030889 9.52 CTLA4 IL10
11 negative regulation of immune response GO:0050777 9.51 CTLA4 TGFB1
12 positive regulation of interleukin-17 production GO:0032740 9.49 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
16 ExPASy
18 FMA
27 GO
28 GTR
29 HGMD
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 MedGen
41 MeSH
42 MESH via Orphanet
43 MGI
45 NCI
46 NCIt
47 NDF-RT
50 NINDS
51 Novoseek
53 OMIM
54 OMIM via Orphanet
58 PubMed
60 QIAGEN
65 SNOMED-CT via HPO
66 SNOMED-CT via Orphanet
67 TGDB
68 Tocris
69 UMLS
70 UMLS via Orphanet
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