Successful Treatment of Adult Epstein-Barr Virus-Associated Hemophagocytic Lymphohistiocytosis With Etoposide Guided by Plasma Epstein-Barr Virus DNA Monitoring: A Case Report.

Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening hyperinflammatory syndrome. Epstein-Barr virus (EBV)-associated HLH is a major subtype of secondary HLH, requiring prompt diagnosis and treatment. However, treatment is particularly challenging in patients with severe coagulopathy and hepatic dysfunction. A previously healthy 23-year-old woman presented with a 2-week history of high-grade fever and cervical lymphadenopathy. Cytopenia, elevated lactate dehydrogenase levels, marked transaminase elevations, hyperferritinemia, and disseminated intravascular coagulation were observed. EBV-associated HLH was diagnosed based on bone marrow hemophagocytosis, an elevated whole blood EBV-DNA level (6.63 log IU/mL), and serological findings consistent with primary EBV infection. Initial treatment with methylprednisolone and cyclosporine A was followed by weekly oral etoposide, with careful monitoring in the setting of severe hepatic dysfunction. Clinical symptoms and laboratory abnormalities improved within 3 weeks of starting etoposide. Plasma EBV-DNA became undetectable during treatment, and oral etoposide was discontinued after the third weekly course on Day 18, with a total cumulative dose of 750 mg, corresponding to approximately 450 mg/m Trial Registration: The authors have confirmed clinical trial registration is not needed for this submission.