The Broad Spectrum of Epstein-Barr Virus Disease (cont.)

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 following:

  • 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.

  • Hodgkin 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 lymphohistiocytosis and lymphomatoid granulomatosis.