Chronic active Epstein-Barr virus disease

Background

• Life-threatening infection with Epstein-Barr virus involving NK and T cells

Pathophysiology

• EBV infection involving B, T, or NK cells

Epidemiology

• Most cases reported in people of Asian and North American Indigenous descent
• Most common in Japan

Clinical Manifestations

• Symptoms include fever, liver dysfunction, splenomegaly, lymphadenopathy, and thrombocytopenia
• May also have hepatomegaly, anemia, mosquito bite hypersensitivity, rashes, oral ulcers, hemophagocytic lymphohistiocytosis, coronary artery aneurysm, liver failure, lymphoma, and interstitial pneumonia
• Occasionally has uveitis, CNS disease, intestinal perforation, and myocarditis

Management

• Hematopoietic stem cell transplantation is the only curative treatment
• Symptoms may be temporarily improved with corticosteroids