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Acute liver injury secondary to hemophagocytic lymphohistiocytosis triggered by Epstein–Barr virus infection

机译:Epstein-Barr病毒感染引发急性肝损伤血液活性淋巴管引发

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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.
机译:我们展示了一名23岁的男子,患有血糖淋巴管激毒症(HLH),由Epstein-Bar病毒(EBV)感染引发。这种患者患有持续的发烧和急性肝损伤6?有与EBV感染相关的传染性单核细胞杂核糖后的急性肝损伤。他具有脱氧纤维生血症,高温血症,升高的白细胞介素-2受体,升高的凝血酶原时间和刺激性血症。骨髓考试评估胰腺病症揭示了巨噬细胞吞噬成熟的红细胞。基于这些发现,我们怀疑受EBV感染(EBV-HLH)引发的HLH。为了区分伴随EBV感染的恶性淋巴瘤引发的HLH,我们进行了经皮肝活检,这揭示了非典型T淋巴细胞渗透肝组织。 T淋巴细胞对EBV编码的RNA原位杂交是阳性的,并且检测到没有明显的单克隆T细胞受体链基因重排。这些发现表明EBV肝炎,因此,排除了恶性淋巴瘤。我们终于诊断了EBV-HLH。根据HLH-2004的指南建议,患者用皮质类固醇和依托泊苷治疗,患者的症状和实验室值改善了。之后,他没有经历过重复。迅速识别和对治疗的启动仍然是EBV-HLH患者存活的关键,肝脏活组织检查有助于诊断。

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