The Broad Spectrum of EBV Disease
Medical Author: Melissa Conrad Stoppler, MD
Medical Editor: William C. Shiel Jr., MD, FACP, FACR
The Epstein-Barr virus (EBV), a
member of the herpesvirus family, is found
throughout the world. Studies show that up to 95% of all adults have antibodies
against this common virus, meaning that they were infected at some point in
their lives. Even though most infections with EBV go unnoticed or produce only
very mild symptoms, in some cases, it can be associated with the development of
serious conditions, including several types of cancer. Even mild or
non-life-threatening infection with EBV can, occasionally, be associated with
the development of serious complications from the infection. Although the virus
typically targets lymphocytes, a particular blood cell involved in the immune
response, almost all organs systems can ultimately be affected by EBV infection.
EBV is transmitted by close person-to-person contact. Primary, or initial,
infection with EBV may not produce symptoms or there can be a number of
different symptoms, especially in young children. The manifestations of primary
EBV infection include:
- Infectious mononucleosis (IM): This is the most common medical condition
associated with EBV. IM is characterized by extreme fatigue, tonsillitis and/or
inflamed throat (pharyngitis), enlarged, tender lymph nodes in the neck, and
moderate to high fever. Although the fever and sore throat typically
resolve within two weeks, fatigue may persist for months after the
- Other mild childhood illnesses: EBV infection in
young children has also been linked to ear infections, diarrhea, other
gastrointestinal symptoms, and cold symptoms in addition to
the classic symptoms of IM.
- In rare cases, primary infection
with EBV has been associated with neurologic disturbances including
Guillain-Barre syndrome and meningoencephalitis. Other rare manifestations of
primary infection are abnormalities of the blood or coagulation systems such as
anemia, thrombotic thrombocytopenic
and disseminated intravascular coagulation (DIC).
Complications of primary EBV infection are not common but may be
life-threatening if not treated. Rupture of the spleen (which may be enlarged in
patients with IM) occurs in one to two cases per 1,000 cases of IM, almost always in
males. A second potentially fatal but treatable complication is obstruction of
the airway due to enlargement of lymph nodes and swelling of inflamed tissues
surrounding the airway. EBV generally persists throughout life in most people
who are infected and rarely causes any problems. In some cases, however, EBV has
been linked to the development of cancers and serious conditions, including the
- Burkitt's lymphoma: This tumor
is the most common childhood malignancy in equatorial Africa. Tumors are
characteristically located in the jaw. Genetic studies have shown that in
equatorial Africa (where over 95% of children have been infected with EBV by
age 3), the vast majority of Burkitt lymphomas originate from an EBV-infected
lymphocyte, a type of blood cell targeted by the virus.
lymphoma : In
1987, the finding of EBV genetic material was reported in up to 50% of cases
of Hodgkin lymphoma, also known as Hodgkin's disease, in
certain geographic areas and patient populations.
- Nasopharyngeal carcinoma:
This cancer is rare in almost all populations, but it occurs as one of the
most common cancers in southern China. Numerous studies have shown that these
cancers are related to EBV infection and that EBV genetic material is
contained in these cancers. Still, the majority of people in China who have
been infected with EBV do not develop nasopharyngeal carcinoma.
- EBV-associated non-Hodgkin lymphomas (usually B-cell
lymphomas) have also been described in people infected with the HIV virus.
- Rare cases of T-cell lymphomas have also been linked
to EBV infection.
- Post-transplant lymphoproliferative disease (PTLD)
refers to a category of conditions that develop in people taking
immunosuppressive medications following an organ transplant. The EBV virus has
been implicated in the majority of cases of PTLD. Manifestations can vary,
ranging from an increased number of lymphocytes in the bloodstream to
blood-cell malignancies such as B-cell lymphoma.
- Rare hematologic conditions: EBV has also been found
to be associated with the development of certain rare conditions causing
abnormal growth and function of specific components of the blood. Examples of
these conditions are hemophagocytic