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Semin Liver Dis 2016; 36(01): 099-106
DOI: 10.1055/s-0036-1571299
Diagnostic Problems in Hepatology
Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

An Unusual Cause of Acute Liver Failure: Three Cases of Hemophagocytic Lymphohistiocytosis Presenting at a Transplant Center

Amanda Schneier
1   Hospital Medicine, Kaiser Permanente Panorama City Medical Center, Panorama City, California
,
Ashley E. Stueck
2   Department of Pathology, Icahn School of Medicine at Mount Sinai, New York, New York
,
Bruce Petersen
2   Department of Pathology, Icahn School of Medicine at Mount Sinai, New York, New York
,
Swan N. Thung
2   Department of Pathology, Icahn School of Medicine at Mount Sinai, New York, New York
,
Ponni Perumalswami
3   Department of Medicine, Icahn School of Medicine at Mount Sinai, New York, New York
› Author Affiliations
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Publication History

Publication Date:
12 February 2016 (online)

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Abstract

Acquired hemophagocytic lymphohistiocytosis (HLH) is a rare life-threatening disorder of the immune system. Hemophagocytic lymphohistiocytosis has been associated with infections, autoimmune disorders, and malignancy. This case series describes three patients admitted to an academic liver transplant center from February 2014 to February 2015 with acute liver failure (ALF) who were ultimately diagnosed with HLH. All cases were female patients (44 to 53 years of age) transferred for workup of ALF. All developed fevers and cytopenias and underwent rapid evaluation for liver transplant by a multidisciplinary team. A complete workup for ALF was negative for intrinsic liver disease and none had significant alcohol or toxin exposure. The patients had liver biopsies showing diffuse lobular necroinflammation, of which two had evidence of hemophagocytosis on histopathology. The diagnosis of HLH was made by bone marrow biopsy featuring histiocytes with hemophagocytosis. All cases were treated with chemotherapy, but died during their hospitalization. Hemophagocytic lymphohistiocytosis can present as ALF in adult patients. Given the low success rate of treatment, early diagnosis is critical. Therefore, a high degree of suspicion should be exercised in patients with unexplained ALF.

Keywords

acute liver failure - hemophagocytic lymphohistiocytosis - hemophagocytic syndrome - acute hepatitis
 

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