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

Amanda Schneier, Ashley E. Stueck, Bruce Petersen, Swan N. Thung, Ponni Perumalswami

Research output: Contribution to journalArticlepeer-review

13 Scopus citations

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.

Original languageEnglish
Pages (from-to)99-105
Number of pages7
JournalSeminars in Liver Disease
Volume36
Issue number1
DOIs
StatePublished - 1 Feb 2016

Keywords

  • acute hepatitis
  • acute liver failure
  • hemophagocytic lymphohistiocytosis
  • hemophagocytic syndrome

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