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Liver Dysfunction in Adult Hemophagocytic Lymphohistiocytosis: A Narrative Review

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Abstract

Hemophagocytic lymphohistiocytosis (HLH) is a rare, life-threatening condition that has been increasingly recognized in adults and is characterized by a hyperinflammatory state due to immune dysregulation. Its nonspecific presentation, the lack of clinician familiarity given its rarity, and shared clinical features with sepsis and other syndromes can lead to a delay in diagnosis and a poor prognosis. Significant liver function abnormalities as the initial manifestation of HLH are uncommon and can range from mild elevation of aminotransferases to fulminant hepatic failure with high mortality rates. The authors encountered a case of adult HLH mimicking acute viral hepatitis in which a markedly elevated ferritin level led to a prompt diagnosis, early initiation of treatment, and a successful outcome. Clinicians, including gastroenterologists and hepatologists, are often called upon to evaluate patients with abnormal liver tests and may lack experience in the early diagnosis and management of liver dysfunction in the context of HLH. Thus, we expand our reporting to a narrative review of literature which explores the pathogenesis of HLH, challenges associated with its diagnosis, previous reports of liver disease associated with the syndrome, recommended treatments for the familial and adult variations including the role of liver transplantation, and the outcomes of these treatments.

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Acknowledgements

The authors would like to thank Dr. Dick Hwang from the Department of Pathology at Virginia Mason Franciscan Health for his assistance in acquiring the pathology images from the bone marrow biopsy and providing pathologist interpretation of these images. The authors would also like to thank Terri Davis Smith from the Center for Digestive Health at Virginia Mason Franciscan Health for her assistance in coordination and submission of the manuscript.

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  1. Division of Gastroenterology and Hepatology, Center for Digestive Health, Virginia Mason Franciscan Health, Seattle, WA, USA

    Muaaz Masood, Asma Siddique, Rajesh Krishnamoorthi & Richard A. Kozarek

  2. Center for Interventional Immunology, Benaroya Research Institute, Virginia Mason Franciscan Health, 1201 Ninth Ave, Seattle, WA, 98101, USA

    Richard A. Kozarek

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  1. Muaaz Masood
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Muaaz Masood, MD: Study concept, literature review, manuscript writing and editing. Asma Siddique, MD: Study concept, manuscript editing. Rajesh Krishnamoorthi, MD: Manuscript editing. Richard A. Kozarek, MD: Study concept, literature review, manuscript editing.

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Correspondence to Richard A. Kozarek.

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Masood, M., Siddique, A., Krishnamoorthi, R. et al. Liver Dysfunction in Adult Hemophagocytic Lymphohistiocytosis: A Narrative Review. Adv Ther 41, 553–566 (2024). https://doi.org/10.1007/s12325-023-02768-8

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