title: An Unusual Cause of Acute Liver Failure: Three Cases of Hemophagocytic Lymphohistiocytosis Presenting at a Transplant Center.
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.
authors: Hospital Medicine, Kaiser Permanente Panorama City Medical Center, Panorama City, California.
authors: Amanda
authors: A
authors: Schneier
authors: Department of Pathology, Icahn School of Medicine at Mount Sinai, New York, New York.
authors: Ashley E
authors: AE
authors: Stueck
authors: Department of Pathology, Icahn School of Medicine at Mount Sinai, New York, New York.
authors: Bruce
authors: B
authors: Petersen
authors: Department of Pathology, Icahn School of Medicine at Mount Sinai, New York, New York.
authors: Swan N
authors: SN
authors: Thung
authors: Department of Medicine, Icahn School of Medicine at Mount Sinai, New York, New York.
authors: Ponni
authors: P
authors: Perumalswami
mesh_terms: D000328:Adult; D001706:Biopsy; D001856:Bone Marrow Examination; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D017114:Liver Failure, Acute / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology*; D051359:Lymphohistiocytosis, Hemophagocytic / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy; D008875:Middle Aged; D011237:Predictive Value of Tests; D016896:Treatment Outcome