National Center for Advancing and Translational Sciences Genetic and Rare Diseases Information Center, a program of the National Center for Advancing and Translational Sciences

Chronic active Epstein-Barr virus infection


Other Names:
CEBV; CAEBV infection; Chronic active Epstein-Barr disease
Categories:
Chronic active Epstein-Barr virus infection (CAEBV) is a very rare complication of an Epstein Barr virus (EBV). Signs and symptoms of CAEBV may include fever, swollen lymph nodes, and an enlarged liver and/or spleen.[1][2][3] More serious complications may include anemia, nerve damage, liver failure, and/or interstitial pneumonia.[1][3] Symptoms may be constant or come and go, and tend to get worse over time. CAEBV occurs when the virus remains ‘active’ and the symptoms of an EBV infection do not go away. It is diagnosed based on the symptoms and blood tests that show EBV DNA remaining at high levels for at least 3 months. Treatment is based on managing the symptoms. The only well-documented, effective treatment for CAEBV is hematopoietic stem cell transplantation.[3]

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.[3]
Last updated: 5/19/2020
The following list includes the most common signs and symptoms in people with chronic active Epstein-Barr virus infection (CAEBV). These features may be different from person to person. Some people may have more symptoms than others and symptoms can range from mild to severe. This list also does not include every symptom or feature that has been described in this condition.[1][2][3]

Signs and symptoms include:
  • Fever
  • Swollen lymph nodes
  • Enlarged liver (hepatomegaly)
  • Enlarged spleen (splenomegaly)
  • Anemia 
  • Nerve damage
  • Liver failure
  • Pneumonia
About 95% of people become infected by Epstein-Barr virus (EBV) by adulthood, and most never have any health problems.[1] Some people with EBV will develop infectious mononucleosis or other illnesses, and will recover from their illness with no other problems.[4][5] Very rarely, an EBV infection will develop into CAEBV. Over time, CAEBV can lead to failure of the immune system which, if not treated, can lead to potentially life-threatening complications.[3]
Last updated: 5/19/2020

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.

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Medical Terms Other Names
Learn More:
HPO ID
Percent of people who have these symptoms is not available through HPO
Autosomal recessive inheritance 0000007
Bronchiectasis
Permanent enlargement of the airways of the lungs
0002110
Fever 0001945
Immunodeficiency
Decreased immune function
0002721
Pneumonia 0002090
Recurrent respiratory infections
Frequent respiratory infections
Multiple respiratory infections
respiratory infections, recurrent
Susceptibility to respiratory infections
[ more ] 		0002205
Sinusitis
Sinus inflammation
0000246
Splenomegaly
Increased spleen size
0001744
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Last updated: 7/1/2020
Chronic active Epstein-Barr virus infection (CAEBV) is a rare complication of having Epstein-Barr virus (EBV). About 95% of people will become infected with the EBV by adulthood and many will have no symptoms. CAEBV occurs when an EBV infection doesn't go away and the virus remains "active," allowing symptoms of EBV infection to persist and get worse over time.[1][3][4] It is not known why some people develop CAEBV and others do not.
Last updated: 5/19/2020
Chronic active Epstein-Barr virus infection (CAEBV) is diagnosed based on a clinical exam and high EBV DNA in the blood which persists for at least 3 months. A test known as a quantitative PCR test is used to measure the amount of EBV DNA.[3]
Last updated: 5/19/2020
Treatment for chronic active Epstein-Barr virus infection (CAEBV) includes medications to manage the symptoms. Hematopoietic stem-cell transplant is currently the only curative treatment for this condition.[6]

Specialists involved in the care of someone with CAEBV may include:
  • Infectious disease specialist
  • Immunologist
  • Hematologist
Last updated: 5/19/2020
The exact number of people who have chronic active Epstein-Barr virus infection (CAEBV) is unknown. About 95% of people will become infected with the Epstein-Barr virus by adulthood.[1] Only a small number of these people will develop CAEBV.
Last updated: 5/19/2020

If you need medical advice, you can look for doctors or other healthcare professionals who have experience with this disease. You may find these specialists through advocacy organizations, clinical trials, or articles published in medical journals. You may also want to contact a university or tertiary medical center in your area, because these centers tend to see more complex cases and have the latest technology and treatments.

If you can’t find a specialist in your local area, try contacting national or international specialists. They may be able to refer you to someone they know through conferences or research efforts. Some specialists may be willing to consult with you or your local doctors over the phone or by email if you can't travel to them for care.

You can find more tips in our guide, How to Find a Disease Specialist. We also encourage you to explore the rest of this page to find resources that can help you find specialists.

Healthcare Resources

Research helps us better understand diseases and can lead to advances in diagnosis and treatment. This section provides resources to help you learn about medical research and ways to get involved.

Clinical Research Resources

  • ClinicalTrials.gov lists trials that are related to Chronic active Epstein-Barr virus infection. Click on the link to go to ClinicalTrials.gov to read descriptions of these studies.

    Please note: Studies listed on the ClinicalTrials.gov website are listed for informational purposes only; being listed does not reflect an endorsement by GARD or the NIH. We strongly recommend that you talk with a trusted healthcare provider before choosing to participate in any clinical study.

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.

Where to Start

  • 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.

In-Depth Information

  • 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.

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  1. 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. http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3112034/.
  2. Aronson MD, Auwaerter PG. Infectious mononucleosis. UpToDate. Waltham, MA: UpToDate; Updated May 28, 2019; https://www.uptodate.com/contents/infectious-mononucleosis.
  3. 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/.
  4. About Epstein-Barr Virus (EBV). Centers of Disease Control and Prevention (CDC). 2015; https://www.cdc.gov/epstein-barr/about-ebv.html.
  5. 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. http://doi.org/10.1371/journal.pone.0179124.
  6. 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.
  7. 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.