Adult hemophagocytic lymphohistiocytosis is a lifethreatenning disease which has hepatic manifestations mimicking acute hepatitis or can present with fulminant hepatic failure. The undelying pathophysiology is immune dysregulation causing a hyperinflammatory state. Clues to diagnosis include extremely high ferritin levels, whereas definitive diagnosis is usually made by bone marrow, as opposed to liver biopsy. Even with early and appropriate treatment with weekly dexamethasone and etoposide, mortality remains high.
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