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Hemophagocytic lymphohistiocytosis (HLH) is a rare, life-threatening hyperinflammatory syndrome associated with high mortality. We report a case of secondary HLH associated with herpes simplex virus type 2 (HSV-2) viremia in a 21-year-old postpartum woman who developed acute liver failure. The patient presented with fever, cytopenias, and extreme hyperferritinemia (>30,000 ng/mL). Bone marrow examination demonstrated prominent hemophagocytosis. Despite treatment with dexamethasone, intravenous immunoglobulin (IVIG), and acyclovir, she developed fulminant hepatic failure with severe coagulopathy and encephalopathy, requiring urgent liver transplantation. Postoperatively, she developed refractory shock due to polymicrobial sepsis and died. The explanted liver showed massive hemorrhagic necrosis. This case highlights the diagnostic challenges of HLH presenting as acute liver failure in the postpartum period, emphasizes the importance of early recognition of HSV infection, and illustrates the substantial risks associated with liver transplantation during active hyperinflammation.
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Souza et al. (2026) studied this question.
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