A 23-year-old male presented with fever, night sweats, weight loss, and hepatosplenomegaly, initially diagnosed with chronic active Epstein-Barr virus (CAEBV) infection. Despite various treatments, his condition progressed to hemophagocytic lymphohistiocytosis (HLH) and subsequently to NK/T-cell lymphoma, confirmed by bone marrow biopsy and EBV DNA detection. The patient's disease was refractory to multiple therapies, including chemotherapy and targeted agents, leading to a fatal outcome. This case highlights the aggressive and complex nature of CAEBV, which can rapidly evolve into life-threatening hematologic malignancies.
Key note: Chronic active Epstein-Barr virus infection can rapidly progress to fatal hemophagocytic lymphohistiocytosis and NK/T-cell lymphoma, requiring early aggressive management.