Chronic active Epstein-Barr virus infection

Alternate names

CEBV; CAEBV infection; Chronic active Epstein-Barr disease

Definition

Chronic active Epstein-Barr virus infection (CAEBV) is a very rare complication of an Epstein Barr virus (EBV) infection.

Epidemiology

• 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.
• Only a small number of these people will develop CAEBV.

Cause

• Chronic active Epstein-Barr virus infection (CAEBV) is a rare complication of having Epstein-Barr virus (EBV).
• About 95% of people 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".
• This allows the symptoms of an EBV infection to persist and get worse over time.
• It is not known why some people develop CAEBV and others do not.

Signs and symptoms

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) 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 immune system which, if not treated, can lead to potentially life-threatening complications.

Diagnosis

• Chronic active Epstein-Barr virus infection (CAEBV) is diagnosed based on the symptoms, 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.

Treatment

Treatment for chronic active Epstein-Barr virus infection (CAEBV) includes medication to help manage the symptoms. Hematopoietic stem-cell transplant is currently the only curative treatment for this condition. Specialists involved in the care of someone with CAEBV may include:

• Infectious disease specialist
• Immunologist
• Hematologist

NIH genetic and rare disease info

• NIH info on Chronic active Epstein-Barr virus infection

Chronic active Epstein-Barr virus infection is a rare disease.

Chronic active Epstein-Barr virus infection Resources
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