CEBV
MCID: CHR276
MIFTS: 37

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 very rare complication of an Epstein Barr virus (EBV). Most people (about 95% by adulthood) become infected by EBV at some point of their lives and never have any health problems. Some people with EBV will develop infectious mononucleosis or other illnesses, and will recover from their illness with no other problems. Very rarely, an EBV infection will develop into CAEBV. Signs and symptoms of CAEBV may include fever, swollen lymph nodes, enlarged liver and/or spleen, liver function test abnormalities, and low levels of certain types of blood cells. Some people develop anemia, over sensitivity to mosquito bites, rash, oral ulcers, nerve damage, liver failure, and/or interstitial pneumonia. These symptoms may occur irregularly or continuously, and tend to get worse over time. CAEBV occurs when people with an EBV infection are unable to control the infection and the virus remains "active," allowing symptoms of EBV infection to persist and progress. It is not known why some people develop CAEBV and others do not. Because this condition is so rare, it is not clear how many people with an EBV infection will go on to develop CAEBV. CAEBV is diagnosed based on the signs and symptoms and the results of blood tests that show EBV DNA at high levels, indicating an active infection. It is a common misconception that people with fatigue alone (and not the symptoms mentioned above) and positive blood markers for EBV have CAEBV. These blood markers are present for life in people with prior EBV exposure, and are not markers of an active infection of CAEBV. Over time, CAEBV can lead to progressive failure of the immune system which, if not treated, can lead to potentially fatal complications such as a blood cell disorder known as hemophagocytic syndrome, multiorgan failure, or EBV-positive lymphoma. The only well-documented, effective treatment for CAEBV is hematopoietic stem cell transplantation.

MalaCards based summary : Chronic Active Epstein-Barr Virus Infection, also known as chronic active epstein-barr disease, is related to hematopoietic stem cell transplantation and acute graft versus host disease. 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. 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 218)
# Related Disease Score Top Affiliating Genes
1 hematopoietic stem cell transplantation 31.3 IL2 IL10 IFNG CTLA4
2 acute graft versus host disease 31.1 IL2 IL10 IFNG
3 uveitis 31.1 IL10 IFNG CTLA4
4 graft-versus-host disease 30.9 IL2 IL10 IFNG
5 chronic fatigue syndrome 30.8 TGFB1 IL2 IL10
6 alpha/beta t-cell lymphopenia with gamma/delta t-cell expansion, severe cytomegalovirus infection, and autoimmunity 30.7 IL10 IFNG CTLA4
7 acquired immunodeficiency syndrome 30.7 IL2 IL10 IFNG
8 interstitial lung disease 30.7 TGFB1 IL10 IFNG
9 autoimmune lymphoproliferative syndrome 30.6 IL2 IL10 CTLA4
10 autoimmune disease 30.6 IL2 IL10 IFNG CTLA4
11 inflammatory bowel disease 30.4 TGFB1 IL2 IL10 IFNG
12 systemic lupus erythematosus 29.2 THBD TGFB1 SELE IL2 IL10 IFNG
13 rheumatoid arthritis 28.3 TNFAIP6 TGFB1 SELE IL2 IL10 IFNG
14 pfeiffer syndrome 10.9
15 lymphoproliferative syndrome 10.7
16 splenomegaly 10.7
17 hemophagocytic lymphohistiocytosis 10.6
18 pancytopenia 10.6
19 variola major 10.6 IL2 IFNG
20 fixed drug eruption 10.6 IL2 IFNG
21 vasculitis 10.6
22 cephalosporin allergy 10.6 IL10 IFNG
23 berylliosis 10.5 IL2 IFNG
24 hyperlucent lung 10.5 IL10 IFNG
25 hemopericardium 10.5
26 pericardial effusion 10.5
27 juvenile myasthenia gravis 10.5 TGFB1 IL10
28 atopic keratoconjunctivitis 10.5 IL2 IFNG
29 allergic hypersensitivity disease 10.5
30 thrombocytopenia 10.5
31 coronary artery aneurysm 10.5
32 hydroa vacciniforme 10.5
33 sporotrichosis 10.5 IL10 IFNG
34 inflammatory bowel disease 25, autosomal recessive 10.5 TGFB1 IL10
35 nickel allergic contact dermatitis 10.5 IL10 IFNG
36 african tick-bite fever 10.5 SELE IFNG
37 exanthem 10.4
38 aneurysm 10.4
39 baylisascariasis 10.4 IL10 IFNG
40 autoimmune disease of endocrine system 10.4 IL2 IFNG CTLA4
41 scleritis 10.4 SELE IFNG
42 alopecia areata 10.4 IL2 IFNG CTLA4
43 chronic graft versus host disease 10.4 IL10 IFNG
44 sezary's disease 10.4 IL2 IFNG CTLA4
45 gaucher disease, type ii 10.4 IL2 IL10
46 intermediate uveitis 10.4 IL2 IL10 IFNG
47 coccidiosis 10.4 IL2 IL10 IFNG
48 leprosy 3 10.4 IL2 IL10 IFNG
49 lepromatous leprosy 10.4 IL2 IL10 IFNG
50 pulmonary hypertension, primary, 1 10.4

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

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 CASPALLO: A Phase I Study Evaluating the Use of Allodepleted T Cells Transduced With Inducible Caspase 9 Suicide Gene After Haploidentical Stem Cell Transplantation Active, not recruiting NCT00710892 Phase 1
2 Genetic Studies of Chronic Active Epstein-Barr Virus Infection Enrolling by invitation NCT00032513
3 Study to Surveil and Track the Outcomes of Chronic Latent EBV Infection Based on Healthy Volunteers Undergoing Routine Inspection 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, Spleen, Lymph Node, Nk Cells, Bone Marrow

Publications for Chronic Active Epstein-Barr Virus Infection

Articles related to Chronic Active Epstein-Barr Virus Infection:

(show top 50) (show all 238)
# Title Authors PMID Year
1
NK-cell post-transplant lymphoproliferative disease with chronic active Epstein-Barr virus infection-like clinical findings. 38
31398454 2019
2
Chronic Active Epstein-Barr Virus Infection of T/NK-Cell Type Mimicking Classic Hodgkin Lymphoma: Clinicopathologic and Genetic Features of 8 Cases Supporting a Variant With "Hodgkin/Reed-Sternberg-like" Cells of NK Phenotype. 38
31305266 2019
3
A fatal case of Exophiala dermatitidis disseminated infection in an allogenic hematopoietic stem cell transplant recipient during micafungin therapy. 38
30679025 2019
4
Epstein-Barr virus and its association with disease - a review of relevance to general practice. 38
31088382 2019
5
Bilateral granulomatous panuveitis in two patients with T-cell type of chronic active Epstein-Barr virus infection. 38
30922271 2019
6
Fatal Chronic Active Epstein-Barr Virus Infection in a Rheumatoid Arthritis Patient Treated with Abatacept. 38
30210119 2019
7
Novel mutations of STXBP2 and LYST associated with adult haemophagocytic lymphohistiocytosis with Epstein-Barr virus infection: a case report. 38
30782130 2019
8
Chronic Active Epstein-Barr Virus Infection With Systemic Vasculitis and Pulmonary Arterial Hypertension in a Child. 38
31231622 2019
9
Chronic active EBV infection associated with NK cell lymphoma and hemophagocytic lymphohistiocytosis in a 27-year-old woman: A case report. 38
30633196 2019
10
[Fatal exacerbations of chronic active Epstein-Barr virus infection subsequent to cytotoxic chemotherapy]. 38
31068557 2019
11
Advances in the Study of Chronic Active Epstein-Barr Virus Infection: Clinical Features Under the 2016 WHO Classification and Mechanisms of Development. 38
30805320 2019
12
Chronic active Epstein-Barr virus infection: a bi-faceted disease with inflammatory and neoplastic elements. 38
30704352 2018
13
[Clinical features of adult patients with chronic active Epstein-Barr virus infection]. 38
30392236 2018
14
18F-FDG PET/CT With Diffusely High FDG Uptake Throughout Subcutaneous Adipose Tissues. 38
30036247 2018
15
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. 38
30285657 2018
16
STAT3 is constitutively activated in chronic active Epstein-Barr virus infection and can be a therapeutic target. 38
30123428 2018
17
[Chronic active Epstein-Barr virus infection cause pharyngeal ulcer and major blooding: a case report]. 38
29902855 2018
18
A distinct subtype of Epstein-Barr virus-positive T/NK-cell lymphoproliferative disorder: adult patients with chronic active Epstein-Barr virus infection-like features. 38
29242302 2018
19
Defining the elusive boundaries of chronic active Epstein-Barr virus infection. 38
29866887 2018
20
FDG-PET/CT findings of chronic active Epstein-Barr virus infection. 38
29022747 2018
21
An adult-onset case of chronic active Epstein-Barr virus infection with fulminant clinical course. 38
29426773 2018
22
Chronic active Epstein-Barr virus infection with cutaneous lymphoproliferation: haemophagocytosis in the skin and haemophagocytic syndrome. 38
29024023 2018
23
An Efficient and Simple Method to Establish NK and T Cell Lines from Patients with Chronic Active Epstein-Barr Virus Infection. 38
29658924 2018
24
Epstein-Barr Virus-Positive T/NK-Cell Lymphoproliferative Diseases in Chinese Mainland. 38
30356785 2018
25
Hematopoietic Stem Cell Transplantation for the Treatment of Epstein-Barr Virus-Associated T- or NK-Cell Lymphoproliferative Diseases and Associated Disorders. 38
30460216 2018
26
Epstein-Barr Virus-Associated T-Cell Lymphoproliferative Disorder Presenting as Chronic Diarrhea and Intestinal Bleeding: A Case Report. 38
30519236 2018
27
[Ovum collection following ovarian stimulation in patients with chronic active Epstein-Barr virus infection]. 38
29877232 2018
28
[Cerebrospinal fluid findings in chronic active Epstein-Barr virus infection with central nervous system involvement]. 38
29743394 2018
29
Whole transcriptome profiling reveals major cell types in the cellular immune response against acute and chronic active Epstein-Barr virus infection. 38
29259291 2017
30
Chronic active Epstein-Barr virus infection as the initial symptom in a Janus kinase 3 deficiency child: Case report and literature review. 38
29049190 2017
31
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. 38
28489771 2017
32
How we treat chronic active Epstein-Barr virus infection. 38
28210942 2017
33
Inflammatory cytokine production in chronic active Epstein-Barr virus infection. 38
28381684 2017
34
Virus-specific cytotoxic T cells in chronic active Epstein-Barr virus infection. 38
28679986 2017
35
[A case of chronic active Epstein-Barr virus infection with concomitant giant coronary artery aneurysm]. 38
27784484 2016
36
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. 38
26879589 2016
37
[An analysis of immunophenotyping of peripheral lymphocytes in adult patients with infectious mononucleosis and chronic active Epstein-Barr virus infection]. 38
27256608 2016
38
Chronic active Epstein-Barr virus infection with marked pericardial effusion successfully treated with allogeneic peripheral blood stem cell transplantation. 38
27263789 2016
39
Successful Cord Blood Stem Cell Transplantation for an Adult Case of Chronic Active Epstein-Barr Virus Infection. 38
27904117 2016
40
[A Case of Severe Chronic Active Epstein-Barr Virus Infection with Aplastic Anemia and Hepatitis]. 38
26809631 2016
41
Secondary neuroendocrine tumor after allogeneic bone marrow transplantation. 38
26711919 2015
42
Chronic active Epstein-Barr virus infection with cutaneous and sinus lymphoproliferation in a white female patient with 25 years' follow-up: an original case report. 38
26148205 2015
43
Metachronous B-cell and T-cell post-transplant lymphoproliferative disorders with features of chronic active Epstein-Barr virus infection. 38
26178917 2015
44
Pulmonary arterial hypertension associated with chronic active Epstein-Barr virus infection. 38
25809637 2015
45
Neurological presentation of hemophagocytic lymphohistiocytosis. 38
26377985 2015
46
Recent Concise Viewpoints of Chronic Active Epstein-Barr Virus Infection. 38
25938379 2015
47
Dehydroxymethylepoxyquinomicin selectively ablates T-CAEBV cells. 38
25553464 2015
48
Chronic active Epstein-Barr virus infection complicated with multiple artery aneurysms. 38
24939943 2014
49
[Clinical and laboratory characteristics of chronic active Epstein-Barr virus infection in children]. 38
25406547 2014
50
Role of latent membrane protein 1 in chronic active Epstein-Barr virus infection-derived T/NK-cell proliferation. 38
24799376 2014

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 TNFAIP6 TGFB1 LAIR1 IL2 IL10 IFNG
2
Show member pathways
13.23 TGFB1 IL2 IL10 IFNG GBP5 GBP1
3
Show member pathways
12.92 TGFB1 IL2 IL10 IFNG GBP5 GBP1
4
Show member pathways
12.74 TGFB1 IL2 IL10 IFNG CTLA4
5
Show member pathways
12.46 TGFB1 IL2 IL10 IFNG
6 12.41 IL2 IL10 IFNG IDO1 CTLA4
7
Show member pathways
12.31 TGFB1 IL2 IL10 IFNG CTLA4
8
Show member pathways
12.13 TGFB1 IL2 IL10 IFNG
9 11.97 THBD SELE IFNG
10
Show member pathways
11.95 TGFB1 IL2 IL10 IFNG
11
Show member pathways
11.89 TGFB1 IL2 IL10 IFNG
12 11.88 TGFB1 IL2 IFNG
13 11.8 LAIR1 IL2 IL10 IFNG
14 11.79 THBD TGFB1 SELE
15 11.79 TGFB1 IL10 IFNG
16 11.76 IL2 IL10 IFNG
17 11.74 TGFB1 IFNG CTLA4
18 11.67 SELE IL2 IFNG
19 11.66 IL2 IL10 IFNG
20
Show member pathways
11.62 IL2 IFNG CTLA4
21 11.55 TGFB1 IL2 IFNG
22
Show member pathways
11.52 SELE IL2 IFNG
23 11.47 TGFB1 IL2 IL10 IFNG
24 11.39 TGFB1 IL2 IFNG
25 11.36 IL2 IL10 IFNG
26 11.25 TGFB1 SELE IL10 IFNG
27 11.08 TGFB1 IL2 IFNG
28 11.02 SELE IL10 IFNG IDO1
29 11 IL2 IL10 IFNG
30 10.89 THBD TGFB1 IL2 IL10 IFNG
31 10.72 TGFB1 IL2 IL10 IFNG

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 TNFAIP6 THBD TGFB1 SELE IL2 IL10

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 LAIR1 IL2 IDO1 GBP5 GBP1 CTLA4
2 negative regulation of inflammatory response GO:0050728 9.67 TNFAIP6 IL2 IL10
3 adaptive immune response GO:0002250 9.67 LAIR1 IL2 IFNG CTLA4
4 female pregnancy GO:0007565 9.63 THBD TGFB1 IDO1
5 inflammatory response GO:0006954 9.63 TNFAIP6 TGFB1 SELE IL10 IDO1 GBP5
6 response to lipopolysaccharide GO:0032496 9.62 THBD SELE IL10 IDO1
7 positive regulation of protein complex assembly GO:0031334 9.56 TGFB1 IFNG
8 positive regulation of interleukin-12 production GO:0032735 9.55 IFNG IDO1
9 negative regulation of protein localization to plasma membrane GO:1903077 9.54 TGFB1 GBP1
10 negative regulation of B cell proliferation GO:0030889 9.52 IL10 CTLA4
11 negative regulation of immune response GO:0050777 9.51 TGFB1 CTLA4
12 positive regulation of interleukin-17 production GO:0032740 9.48 TGFB1 IL2
13 positive regulation of regulatory T cell differentiation GO:0045591 9.43 TGFB1 IL2
14 negative regulation of interleukin-17 production GO:0032700 9.4 TGFB1 IFNG
15 negative regulation of T cell proliferation GO:0042130 9.26 TGFB1 IL10 IDO1 CTLA4
16 regulation of regulatory T cell differentiation GO:0045589 8.92 TGFB1 IL2 IFNG CTLA4

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 TGFB1 IL2 IL10 IFNG

Sources for Chronic Active Epstein-Barr Virus Infection

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 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 TGDB
71 Tocris
72 UMLS
73 UMLS via Orphanet
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