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 that begins as a primary Epstein-Barr virus (EBV) infection. In this type of infection, the body makes too many lymphocytes (lymphoproliferative disease) for a period of more than 6 months duration. Lymphocytes are a type of white blood cell. They are an important part of the immune system because they help fight off diseases and protect the body from infection by producing antibodies against viruses or bacteria and regulating immune responses. In CAEBV there are many antibodies against EBV in the blood.^[1]

Most people (about 95% of adults) get infected with EBV at some point in their lives, and never have any health problems.^[1]^[2] However, EBV can cause infectious mononucleosis and other illnesses, and has a role in various autoimmune diseases and some types of cancer.^[3]^[4] While most infections occurring during childhood do not cause any symptoms, EBV infection in adolescents or young adults can often result in mononucleosis. After an EBV infection, the virus becomes latent (inactive) in the body, and, in some cases, the virus may reactivate. This does not always cause symptoms, but people with weakened immune systems are more likely to develop symptoms if EBV reactivates.^[1]^[3]

In rare cases, people infected with EBV develop chronic active EBV virus infection (CAEBV) without apparent immunodeficiency. Most cases of CAEBV have been reported from Japan. These patients have some of the complications found in otherwise-healthy patients with acute EBV infection, but unlike healthy patients, these complications persist and progress. Symptoms of CAEBV most often include fever, liver dysfunction, an enlarged spleen (splenomegaly), swollen lymph nodes (lymphadenopathy), and low numbers of platelets (thrombocytopenia) as well as high EBV-DNA load in the blood. Other features that appear in more than 10% of patients include enlarged liver (hepatomegaly), anemia, hypersensitivity to mosquito bites, rash, oral ulcers, hemophagocytic syndrome, coronary artery aneurysms, liver failure, lymphoma, and interstitial pneumonia. While the cause is yet unknown, researchers have identified defects in T cells or natural killer (NK) cells activity which results in a decreased defense against the EBV in people with CAEBV.^[1]

It is important to note that the fatigue and malaise from acute infectious mononucleosis (IM) varies from mild symptoms lasting only a few weeks, to more severe symptoms of fatigue that can persist for several months, or even up to a year or more in up to 10% of patients (which may be considered a less severe form of chronic EBV infection).^[5]^[6] The persistence of fatigue that is seen in some patients after acute IM would lead some people to believe that EBV may also cause cases of chronic fatigue syndrome (CFS).^[5] However, no convincing link has been found between EBV and CFS.^[7]

Hematopoietic stem cell transplantation has shown promise in the treatment of CAEBV.^[1]

Last updated: 8/22/2017

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		0002110
Fever		0001945
Pneumonia		0002090
Sinusitis	Sinus inflammation	0000246
Splenomegaly	Increased spleen size	0001744

Showing of 6 |

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

Last updated: 6/1/2018

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

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. 2011 Jun 2; 117(22):5835-49. http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3112034/. Accessed 5/11/2015.
Mononucleosis (Mono) - What Happens. WebMD. 2017; http://www.webmd.com/a-to-z-guides/tc/infectious-mononucleosis-what-happens.
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.
Rogers ME. Acute Infectious Mononucleosis: A Review for Urgent Care Physicians. American Journal of Clinical Medicine. 2012; 9(2):http://aapsus.org/wp-content/uploads/aim88.pdf.
Eligio P, Delia R, Valeria G. EBV Chronic Infections. Mediterranean Journal of Hematology and Infectious Diseases. 2010; 2(1):e2010022. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3033110/.
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.. 2017; 12(6):e0179124. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5467847/.
Chronic Fatigue Syndrome - What Happens. WebMD. 2015; http://www.webmd.com/chronic-fatigue-syndrome/chronic-fatigue-syndrome-what-happens.
Rogers ME. Acute Infectious Mononucleosis: A Review for Urgent Care Physicians. American Journal of Clinical Medicine. 2012; 9(2):http://aapsus.org/wp-content/uploads/aim88.pdf.