Epstein-Barr Virus Associated Disorders

National Institute Of Allergy And Infectious Diseases

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Abstract

Epstein-Barr virus (EBV) is the major cause of infectious mononucleosis and is associated with both B cell cancers (Hodgkin lymphoma, Burkitt lymphoma) and epithelial cell cancers (nasopharyngeal carcinoma, gastric carcinoma). The virus typically infects epithelial cells of the oropharynx and B cells circulating in the blood. The virus establishes a latent infection in B cells where it persists for life. An unusual complication of EBV disease is chronic active Epstein-Barr virus disease (CAEBV) which is characterized by high levels of EBV predominantly in T and/or natural killer cells with lymphoproliferation, organ failure due to infiltration of tissues with virus-infected cells, hemophagocytic lymphohistiocytosis, and/or lymphoma. The disease is more common in Asia than in the United States and Europe. Although allogeneic hematopoietic stem cell transplantation (HSCT) is considered the only curative therapy for CAEBV, its efficacy and the best treatment modality to reduce disease severity prior to HSCT is unknown. In FY 2022, we retrospectively assessed an international cohort of 57 patients. This is the largest report of patients with CAEBV outside of Asia. Treatment of the disease varied widely, although most patients ultimately proceeded to HSCT. Though patients undergoing HSCT had a statistically significant improved survival than those who did not (55% vs 25%), there was still a high rate of death in both groups. Mortality was largely not affected by age, ethnicity, cell-type involvement, or disease complications, but development of lymphoma showed a trend with increased mortality (56% vs 35%). The overwhelming majority (75%) of patients who died after HSCT succumbed to relapsed disease. CAEBV remains challenging to treat when advanced disease is present. Outcomes would likely improve with better disease control strategies, earlier referral for HSCT, and close follow-up after HSCT including aggressive management of rising EBV DNA levels in the blood.