Chronic active Epstein-Barr virus infection

Title

Other Names:

CEBV; CAEBV infection; Chronic active Epstein-Barr disease

Categories:

Viral infections

Summary Summary

Chronic active Epstein-Barr virus infection (CAEBV) is a rare, progressive disease characterized by persistent or intermittent signs and symptoms of infectious mononucleosis ("mono") as well as evidence of prolonged, active EBV infection (identified by specific blood tests).^[1] Signs and symptoms may include fever, swollen lymph nodes, enlarged liver and/or spleen, liver function test abnormalities, and low levels of certain types of blood cells in the bloodstream.^[1]^[2]^[3] Some people develop anemia, hypersensitivity to mosquito bites, rash, oral ulcers, neuropathy, liver failure, and/or interstitial pneumonia.^[2]^[3] Over time, people with CAEBV develop progressive immunodeficiency which, if not treated, can lead to potentially fatal complications such as hemophagocytic syndrome, multiorgan failure, or EBV-positive lymphoma.^[3]

CAEBV begins as a primary EBV infection.^[2] Most people (about 95% of people by adulthood) become infected with EBV at some point in their lives, and never have any health problems.^[2] However, in some people, EBV causes infectious mononucleosis and other illnesses or complications.^[4]^[5] In rare cases, people with EBV develop CAEBV, where their bodies are unable to control the infection and the virus remains "active," allowing symptoms of EBV infection to persist and progress.^[2]^[3]^[4] It currently is not known why some people develop CAEBV and others do not.^[2]^[4]

CAEBV is diagnosed based on the signs and symptoms and the results of blood tests that show EBV DNA at high levels (signifying an active infection). It is a common misconception that people with fatigue alone (and not the symptoms mentioned above) and positive serologic markers for EBV have CAEBV, as these markers at certain levels are present for life in people with prior EBV exposure, and are not markers of active infection.^[1] The only proven effective treatment for CAEBV is hematopoietic stem cell transplantation. If not treated, CAEBV can be fatal.^[3]

Last updated: 10/24/2018

Symptoms Symptoms

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 infections	Frequent infections Frequent, severe infections Increased frequency of infection infections, recurrent Predisposition to infections Susceptibility to infection [ more ]	0002719
Sinusitis	Sinus inflammation	0000246
Splenomegaly	Increased spleen size	0001744

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Do you have more information about symptoms of this disease? We want to hear from you.

Last updated: 4/1/2019

Do you have updated information on this disease? We want to hear from you.

Find a Specialist Find a Specialist

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

To find a medical professional who specializes in genetics, you can ask your doctor for a referral or you can search for one yourself. Online directories are provided by the American College of Medical Genetics and the National Society of Genetic Counselors. If you need additional help, contact a GARD Information Specialist. You can also learn more about genetic consultations from Genetics Home Reference.

Research Research

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.

Learn More Learn More

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.

GARD Answers GARD Answers

Questions sent to GARD may be posted here if the information could be helpful to others. We remove all identifying information when posting a question to protect your privacy. If you do not want your question posted, please let us know.

Have a question? Contact a GARD Information Specialist.

References References

Aronson MD, Auwaerter PG. Infectious mononucleosis. UpToDate. Waltham, MA: UpToDate; July 12, 2018; https://www.uptodate.com/contents/infectious-mononucleosis.
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/.
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. http://doi.org/10.1371/journal.pone.0179124.