| Summary: | Abstract We present a 23‐year‐old man with hemophagocytic lymphohistiocytosis (HLH) triggered by Epstein–Bar virus (EBV) infection. This patient presented with persistent fever and acute liver injury 6 weeks after having an infectious mononucleosis associated with EBV infection. He had hypofibrinogenemia, hyperferritinemia, increased soluble interleukin‐2 receptor, elevated prothrombin time, and pancytopenia. Bone marrow examination for evaluation of pancytopenia revealed that macrophages had phagocytosed mature erythrocytes. Based on these findings, we suspected an HLH triggered by EBV infection (EBV‐HLH). To distinguish from HLH triggered by malignant lymphomas accompanying EBV infection, we performed a percutaneous liver biopsy, which revealed that atypical T‐lymphocytes had infiltrated the liver tissues. The T‐lymphocytes were positive for EBV‐encoded RNA in situ hybridization, and no distinct monoclonal T‐cell receptor chain gene rearrangement was detected. These findings indicated EBV hepatitis and, accordingly, malignant lymphoma was ruled out. We finally made a diagnosis of EBV‐HLH. The patient was treated with corticosteroid and etoposide, according to HLH‐2004 guideline recommendations, and the patient's symptoms and laboratory values improved. After that, he experienced no recurrence. Prompt recognition and initiation of treatment remains the key to the survival of patients with EBV‐HLH, and the liver biopsy was helpful in making the diagnosis.
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