Division of Gastroenterology and Hepatology, Center for Digestive Health, Virginia Mason Franciscan Health, 1201 Ninth Ave, Seattle, WA, 98101, USA.
Center for Interventional Immunology, Benaroya Research Institute, Virginia Mason Franciscan Health, 1201 Ninth Ave, Seattle, WA, 98101, USA.
Dig Dis Sci. 2023 Aug;68(8):3205-3207. doi: 10.1007/s10620-023-07985-3. Epub 2023 Jun 8.
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.
成人噬血细胞性淋巴组织细胞增生症是一种危及生命的疾病，其肝脏表现类似于急性肝炎，也可能表现为暴发性肝衰竭。其潜在的病理生理学是免疫失调导致的过度炎症状态。诊断线索包括极高的铁蛋白水平，而明确的诊断通常是通过骨髓而不是肝活检做出的。即使早期和适当的每周地塞米松和依托泊苷治疗，死亡率仍然很高。
