Chronic active Epstein-Barr virus infection
Prevalence estimates on Rare Medical Network websites are calculated based on data available from numerous sources, including US and European government statistics, the NIH, Orphanet, and published epidemiologic studies. Rare disease population data is recognized to be highly variable, and based on a wide variety of source data and methodologies, so the prevalence data on this site should be assumed to be estimated and cannot be considered to be absolutely correct.
Age of Onset
Autosomal dominant ?A pathogenic variant in only one gene copy in each cell is sufficient to cause an autosomal dominant disease
Autosomal recessive ?Pathogenic variants in both copies of each gene of the chromosome are needed to cause an autosomal recessive disease and observe the mutant phenotype
X-linked dominant ?X-linked dominant inheritance, sometimes referred to as X-linked dominance, is a mode of genetic inheritance by which a dominant gene is carried on the X chromosome.
X-linked recessive ?Pathogenic variants in both copies of a gene on the X chromosome cause an X-linked recessive disorder
Mitochondrial or multigenic ?Mitochondrial genetic disorders can be caused by changes (mutations) in either the mitochondrial DNA or nuclear DNA that lead to dysfunction of the mitochondria and inadequate production of energy.
Multigenic or multifactor ?Inheritance involving many factors, of which at least one is genetic but none is of overwhelming importance, as in the causation of a disease by multiple genetic and environmental factors.
Other Names (AKA)
CEBV; CAEBV infection; Chronic active Epstein-Barr disease
Chronic active Epstein-Barr
Some people with fatigue alone are mistakenly thought to have CAEBV. Very specific testing looking for the level of EBV DNA is necessary to diagnose CAEBV.
Signs and symptoms include:
- Enlarged liver (hepatomegaly)
- Enlarged spleen (splenomegaly)
- Nerve damage
- Liver failure
About 95% of people become infected by Epstein-Barr virus (EBV) sometime in their life and most never have any health problems. Some people with EBV will develop infectious mononucleosis or other illnesses, and will recover with no other problems. Only rarely will an EBV infection develop into CAEBV. Over time, CAEBV can lead to failure of the
This table lists symptoms that people with this disease may have. For most diseases, symptoms will vary from person to person. People with the same disease may not have all the symptoms listed. This information comes from a database called the Human Phenotype Ontology (HPO) . The HPO collects information on symptoms that have been described in medical resources. The HPO is updated regularly. Use the HPO ID to access more in-depth information about a symptom.
|Medical Terms||Other Names||
|Percent of people who have these symptoms is not available through HPO|
Permanent enlargement of the airways of the lungs
Decreased immune function
|Recurrent respiratory infections||
Frequent respiratory infections
Multiple respiratory infections
respiratory infections, recurrent
Susceptibility to respiratory infections
[ more ]
Increased spleen size
Specialists involved in the care of someone with CAEBV may include:
Infectious disease specialist Immunologist Hematologist
These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.
- You can obtain information on this topic from the Centers for Disease Control and Prevention (CDC). The CDC is recognized as the lead federal agency for developing and applying disease prevention and control, environmental health, and health promotion and education activities designed to improve the health of the people of the United States.
- Medscape Reference provides information on this topic. You may need to register to view the medical textbook, but registration is free.
- Online Mendelian Inheritance in Man (OMIM) is a catalog of human genes and genetic disorders. Each entry has a summary of related medical articles. It is meant for health care professionals and researchers. OMIM is maintained by Johns Hopkins University School of Medicine.
- PubMed is a searchable database of medical literature and lists journal articles that discuss Chronic active Epstein-Barr virus infection. Click on the link to view a sample search on this topic.
- Cohen JI, Jaffe ES, Dale JK, et al. Characterization and treatment of chronic active Epstein-Barr virus disease: a 28-year experience in the United States. Blood. June 2, 2011; 117(22):5835-49. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3112034/.
- Aronson MD, Auwaerter PG. Infectious mononucleosis. UpToDate. Waltham, MA: UpToDate; Updated May 28, 2019; https://www.uptodate.com/contents/infectious-mononucleosis.
- Kimura H, Cohen JI. Chronic Active Epstein-Barr Virus Disease. Front Immunol. December 22, 2017; 8:1867:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5770746/.
- About Epstein-Barr Virus (EBV). Centers of Disease Control and Prevention (CDC). 2015; https://www.cdc.gov/epstein-barr/about-ebv.html.
- Loebel M, Eckey M, Sotzny F et al. Serological profiling of the EBV immune response in Chronic Fatigue Syndrome using a peptide microarray. PLoS ONE. 12(6):e0179124. https://doi.org/10.1371/journal.pone.0179124.
- Bollard CM, Cohen JL. How I treat T-cell chronic active Epstein-Barr virus disease. Blood. Jun 28, 2018; 131(26):2899-2905. https://www.ncbi.nlm.nih.gov/pubmed/29712633.
- Sullivan JL. Clinical manifestations and treatment of Epstein-Barr virus infection. UpToDate. Updated May 20, 2019; https://www.uptodate.com/contents/clinical-manifestations-and-treatment-of-epstein-barr-virus-infection.