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 52
Chronic Active Epstein-Barr Disease 52
Caebv Infection 52
Cebv 52

Classifications:



Summaries for Chronic Active Epstein-Barr Virus Infection

NIH Rare Diseases : 52 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 lymphoproliferative syndrome and hemophagocytic lymphohistiocytosis. 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 Lenalidomide and Angiogenesis Inhibitors have been mentioned in the context of this disorder. Affiliated tissues include t cells, liver and lymph node, and related phenotypes are cardiovascular system and hematopoietic 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 305)
# Related Disease Score Top Affiliating Genes
1 lymphoproliferative syndrome 31.3 IL2 IL10 CTLA4
2 hemophagocytic lymphohistiocytosis 31.1 IL10 IFNG CTLA4
3 exanthem 31.0 IL2 IL10 CTLA4
4 acute graft versus host disease 30.8 IL2 IL10 IFNG
5 graft-versus-host disease 30.7 IL2 IL10 IFNG
6 chronic fatigue syndrome 30.6 IL2 IL10 IFNG
7 allergic hypersensitivity disease 30.6 IL2 IL10 IFNG CTLA4
8 myocarditis 30.5 IL2 IL10 IFNG CTLA4
9 uveitis 30.5 IL2 IL10 IFNG CTLA4
10 combined t cell and b cell immunodeficiency 30.5 IL2 IL10 CTLA4
11 vasculitis 30.5 THBD SELE CTLA4
12 alpha/beta t-cell lymphopenia with gamma/delta t-cell expansion, severe cytomegalovirus infection, and autoimmunity 30.5 IL10 IFNG CTLA4
13 rheumatic fever 30.4 IL2 IL10 IFNG
14 hypereosinophilic syndrome 30.3 IL2 IL10 IFNG
15 disseminated intravascular coagulation 30.2 THBD SELE IL10
16 hepatitis a 30.2 IL2 IL10 IFNG CTLA4
17 lymphopenia 30.2 IL2 IL10 IFNG CTLA4
18 peripheral nervous system disease 30.1 IL2 IL10 IFNG CTLA4
19 interstitial lung disease 30.1 TGFB1 IL10 IFNG
20 aphthous stomatitis 30.0 SELE IL2 IL10 IFNG
21 acquired immunodeficiency syndrome 30.0 SELE IL2 IL10 IFNG
22 immune deficiency disease 30.0 IL2 IL10 IFNG IDO1
23 takayasu arteritis 29.8 THBD SELE IL2 IL10
24 severe combined immunodeficiency 29.8 TGFB1 IL2 IL10 IFNG
25 thrombocytopenia 29.6 THBD SELE IL2 IL10 IFNG CTLA4
26 autoimmune disease 29.5 TGFB1 IL2 IL10 IFNG IDO1 CTLA4
27 aplastic anemia 29.1 THBD TGFB1 IL2 IL10 IFNG CTLA4
28 inflammatory bowel disease 29.1 TGFB1 SELE IL2 IL10 IFNG IDO1
29 systemic lupus erythematosus 28.9 THBD TGFB1 SELE IL2 IL10 IFNG
30 rheumatoid arthritis 28.5 TNFAIP6 TGFB1 SELE IL2 IL10 IFNG
31 pfeiffer syndrome 10.9
32 splenomegaly 10.7
33 pancytopenia 10.6
34 hemopericardium 10.5
35 pericardial effusion 10.5
36 coronary artery aneurysm 10.5
37 hydroa vacciniforme 10.5
38 pulmonary hypertension, primary, 1 10.4
39 respiratory failure 10.4
40 aneurysm 10.4
41 fixed drug eruption 10.4 IL2 IFNG
42 atopic keratoconjunctivitis 10.4 IL2 IFNG
43 ataxia and polyneuropathy, adult-onset 10.4
44 autoimmune hepatitis 10.4
45 histiocytosis 10.4
46 encephalitis 10.4
47 47,xyy 10.4
48 toxicodendron dermatitis 10.3 SELE IL2
49 oral tuberculosis 10.3 IL2 IFNG
50 autoimmune disease of cardiovascular system 10.3 IL2 CTLA4

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:

45
# Description MGI Source Accession Score Top Affiliating Genes
1 cardiovascular system MP:0005385 10.09 ATR CTLA4 IDO1 IFNG IL10 IL2
2 hematopoietic system MP:0005397 10.06 ATR CTLA4 GBP5 IDO1 IFNG IL10
3 immune system MP:0005387 10.03 ATR CTLA4 GBP5 IDO1 IFNG IL10
4 endocrine/exocrine gland MP:0005379 9.97 ATR CTLA4 IFNG IL10 IL2 LAIR1
5 digestive/alimentary MP:0005381 9.95 ATR CTLA4 IFNG IL10 IL2 TGFB1
6 liver/biliary system MP:0005370 9.76 ATR CTLA4 IFNG IL10 IL2 SELE
7 reproductive system MP:0005389 9.56 ATR IDO1 IFNG IL10 IL2 TGFB1
8 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):


# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Lenalidomide Approved Phase 2, Phase 3 191732-72-6 216326
2 Angiogenesis Inhibitors Phase 2, Phase 3
3 Antibodies Phase 2, Phase 3
4 Immunoglobulins Phase 2, Phase 3
5 Immunologic Factors Phase 2, Phase 3

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 A Clinical Trial of Programmed Cell Death Protein 1(PD1) Antibody and Lenalidomide as a Treatment for Epstein-Barr Virus-associated Hemophagocytic Lymphohistiocytosis or Chronic Active EBV Infection(CAEBV) Recruiting NCT04084626 Phase 2, Phase 3 PD1 antibody;lenalidomide
2 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
3 Genetic Studies of Chronic Active Epstein-Barr Virus Infection Enrolling by invitation NCT00032513
4 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:

40
T Cells, Liver, Lymph Node, Spleen, Testes, Bone, B Cells

Publications for Chronic Active Epstein-Barr Virus Infection

Articles related to Chronic Active Epstein-Barr Virus Infection:

(show top 50) (show all 243)
# Title Authors PMID Year
1
Chemotherapy Improved Pulmonary Arterial Hypertension in a Patient with Chronic-Active Epstein-Barr Virus Infection. 61
31956153 2020
2
Chronic active Epstein-Barr virus infection with intrapulmonary shunting: A case report. 61
31983614 2020
3
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. 61
31305266 2019
4
Concomitant PIK3CD and TNFRSF9 deficiencies cause chronic active Epstein-Barr virus infection of T cells. 61
31537641 2019
5
NK-cell post-transplant lymphoproliferative disease with chronic active Epstein-Barr virus infection-like clinical findings. 61
31398454 2019
6
A new therapy in Epstein-Barr virus-associated lymphoproliferative disease: a case report and a revision of the literature. 61
31685000 2019
7
A fatal case of Exophiala dermatitidis disseminated infection in an allogenic hematopoietic stem cell transplant recipient during micafungin therapy. 61
30679025 2019
8
Epstein-Barr virus and its association with disease - a review of relevance to general practice. 61
31088382 2019
9
Bilateral granulomatous panuveitis in two patients with T-cell type of chronic active Epstein-Barr virus infection. 61
30922271 2019
10
Fatal Chronic Active Epstein-Barr Virus Infection in a Rheumatoid Arthritis Patient Treated with Abatacept. 61
30210119 2019
11
Novel mutations of STXBP2 and LYST associated with adult haemophagocytic lymphohistiocytosis with Epstein-Barr virus infection: a case report. 61
30782130 2019
12
[Clinical features of adult-onset chronic active Epstein-Barr virus infection]. 61
31484894 2019
13
Chronic Active Epstein-Barr Virus Infection With Systemic Vasculitis and Pulmonary Arterial Hypertension in a Child. 61
31231622 2019
14
Chronic active EBV infection associated with NK cell lymphoma and hemophagocytic lymphohistiocytosis in a 27-year-old woman: A case report. 61
30633196 2019
15
[Fatal exacerbations of chronic active Epstein-Barr virus infection subsequent to cytotoxic chemotherapy]. 61
31068557 2019
16
Advances in the Study of Chronic Active Epstein-Barr Virus Infection: Clinical Features Under the 2016 WHO Classification and Mechanisms of Development. 61
30805320 2019
17
Chronic active Epstein-Barr virus infection: a bi-faceted disease with inflammatory and neoplastic elements. 61
30704352 2018
18
[Clinical features of adult patients with chronic active Epstein-Barr virus infection]. 61
30392236 2018
19
18F-FDG PET/CT With Diffusely High FDG Uptake Throughout Subcutaneous Adipose Tissues. 61
30036247 2018
20
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. 61
30285657 2018
21
STAT3 is constitutively activated in chronic active Epstein-Barr virus infection and can be a therapeutic target. 61
30123428 2018
22
[Chronic active Epstein-Barr virus infection cause pharyngeal ulcer and major blooding: a case report]. 61
29902855 2018
23
A distinct subtype of Epstein-Barr virus-positive T/NK-cell lymphoproliferative disorder: adult patients with chronic active Epstein-Barr virus infection-like features. 61
29242302 2018
24
An adult-onset case of chronic active Epstein-Barr virus infection with fulminant clinical course. 61
29426773 2018
25
Defining the elusive boundaries of chronic active Epstein-Barr virus infection. 61
29866887 2018
26
FDG-PET/CT findings of chronic active Epstein-Barr virus infection. 61
29022747 2018
27
Chronic active Epstein-Barr virus infection with cutaneous lymphoproliferation: haemophagocytosis in the skin and haemophagocytic syndrome. 61
29024023 2018
28
An Efficient and Simple Method to Establish NK and T Cell Lines from Patients with Chronic Active Epstein-Barr Virus Infection. 61
29658924 2018
29
Epstein-Barr Virus-Positive T/NK-Cell Lymphoproliferative Diseases in Chinese Mainland. 61
30356785 2018
30
Hematopoietic Stem Cell Transplantation for the Treatment of Epstein-Barr Virus-Associated T- or NK-Cell Lymphoproliferative Diseases and Associated Disorders. 61
30460216 2018
31
Epstein-Barr Virus-Associated T-Cell Lymphoproliferative Disorder Presenting as Chronic Diarrhea and Intestinal Bleeding: A Case Report. 61
30519236 2018
32
[Cerebrospinal fluid findings in chronic active Epstein-Barr virus infection with central nervous system involvement]. 61
29743394 2018
33
[Ovum collection following ovarian stimulation in patients with chronic active Epstein-Barr virus infection]. 61
29877232 2018
34
Whole transcriptome profiling reveals major cell types in the cellular immune response against acute and chronic active Epstein-Barr virus infection. 61
29259291 2017
35
Chronic active Epstein-Barr virus infection as the initial symptom in a Janus kinase 3 deficiency child: Case report and literature review. 61
29049190 2017
36
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. 61
28489771 2017
37
How we treat chronic active Epstein-Barr virus infection. 61
28210942 2017
38
Virus-specific cytotoxic T cells in chronic active Epstein-Barr virus infection. 61
28679986 2017
39
Inflammatory cytokine production in chronic active Epstein-Barr virus infection. 61
28381684 2017
40
[A case of chronic active Epstein-Barr virus infection with concomitant giant coronary artery aneurysm]. 61
27784484 2016
41
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. 61
26879589 2016
42
[An analysis of immunophenotyping of peripheral lymphocytes in adult patients with infectious mononucleosis and chronic active Epstein-Barr virus infection]. 61
27256608 2016
43
Chronic active Epstein-Barr virus infection with marked pericardial effusion successfully treated with allogeneic peripheral blood stem cell transplantation. 61
27263789 2016
44
Successful Cord Blood Stem Cell Transplantation for an Adult Case of Chronic Active Epstein-Barr Virus Infection. 61
27904117 2016
45
[A Case of Severe Chronic Active Epstein-Barr Virus Infection with Aplastic Anemia and Hepatitis]. 61
26809631 2016
46
Secondary neuroendocrine tumor after allogeneic bone marrow transplantation. 61
26711919 2015
47
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. 61
26148205 2015
48
Metachronous B-cell and T-cell post-transplant lymphoproliferative disorders with features of chronic active Epstein-Barr virus infection. 61
26178917 2015
49
Pulmonary arterial hypertension associated with chronic active Epstein-Barr virus infection. 61
25809637 2015
50
Neurological presentation of hemophagocytic lymphohistiocytosis. 61
26377985 2015

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 27)
# Super pathways Score Top Affiliating Genes
1
Show member pathways
13.21 TGFB1 IL2 IL10 IFNG GBP5 GBP1
2
Show member pathways
12.7 TGFB1 IL2 IL10 IFNG CTLA4
3
Show member pathways
12.46 TGFB1 IL2 IL10 IFNG
4
Show member pathways
12.43 TGFB1 IL2 IL10 IFNG CTLA4
5 12.39 IL2 IL10 IFNG IDO1 CTLA4
6
Show member pathways
12.12 TGFB1 IL2 IL10 IFNG
7 11.95 THBD SELE IFNG
8 11.88 TGFB1 IL2 IFNG
9
Show member pathways
11.87 TGFB1 IL2 IL10 IFNG
10 11.79 TGFB1 IL10 IFNG
11 11.77 THBD TGFB1 SELE
12 11.77 LAIR1 IL2 IL10 IFNG
13 11.74 TGFB1 IFNG CTLA4
14 11.67 SELE IL2 IFNG
15 11.66 IL2 IL10 IFNG
16
Show member pathways
11.62 IL2 IFNG CTLA4
17 11.54 TGFB1 IL2 IFNG
18
Show member pathways
11.51 SELE IL2 IFNG
19 11.47 TGFB1 IL2 IL10 IFNG
20 11.38 TGFB1 IL2 IFNG
21 11.35 IL2 IL10 IFNG
22 11.26 TGFB1 SELE IL10 IFNG
23 11.06 TGFB1 IL2 IFNG
24 11.02 SELE IL10 IFNG IDO1
25 10.96 IL2 IL10 IFNG
26 10.89 THBD TGFB1 IL2 IL10 IFNG
27 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 15)
# Name GO ID Score Top Affiliating Genes
1 adaptive immune response GO:0002250 9.81 LAIR1 IL2 IFNG CTLA4
2 immune system process GO:0002376 9.73 LAIR1 IL2 IDO1 GBP5 GBP1 CTLA4
3 negative regulation of inflammatory response GO:0050728 9.72 TNFAIP6 IL2 IL10
4 inflammatory response GO:0006954 9.63 TNFAIP6 TGFB1 SELE IL10 IDO1 GBP5
5 response to lipopolysaccharide GO:0032496 9.62 THBD SELE IL10 IDO1
6 female pregnancy GO:0007565 9.58 THBD TGFB1 IDO1
7 negative regulation of protein localization to plasma membrane GO:1903077 9.54 TGFB1 GBP1
8 negative regulation of B cell proliferation GO:0030889 9.52 IL10 CTLA4
9 negative regulation of immune response GO:0050777 9.49 TGFB1 CTLA4
10 positive regulation of interleukin-17 production GO:0032740 9.48 TGFB1 IL2
11 negative regulation of interleukin-17 production GO:0032700 9.46 TGFB1 IFNG
12 positive regulation of regulatory T cell differentiation GO:0045591 9.43 TGFB1 IL2
13 positive regulation of MHC class II biosynthetic process GO:0045348 9.37 IL10 IFNG
14 negative regulation of T cell proliferation GO:0042130 9.26 TGFB1 IL10 IDO1 CTLA4
15 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 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 LOVD
41 MedGen
43 MeSH
44 MESH via Orphanet
45 MGI
48 NCI
49 NCIt
50 NDF-RT
53 NINDS
54 Novoseek
56 OMIM
57 OMIM via Orphanet
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 TGDB
70 Tocris
71 UMLS
72 UMLS via Orphanet
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