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

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.

Management and diagnosis of fatty liver disease.

Nonalcoholic fatty liver disease is a common cause of chronic liver disease and has been an increasingly studied topic of research as the obesity epidemic has been growing. There is a significant morbidity and mortality with uncontrolled steatohepatitis, which can progress to fibrosis, cirrhosis and hepatocellular carcinoma. The prevalence of this disease has been estimated to be roughly one-third of the western population, thought to be largely due to diet and sedentary lifestyle. Several treatments have been studied including vitamin E, insulin-sensitizing agents and ursodeoxycholic acid; however, the only treatment shown to improve the histologic changes of nonalcoholic fatty liver disease is weight loss. Given the proven benefit of weight loss, there may be reason to screen at-risk populations; however, limited availability of other disease-modifying treatments may limit the cost-benefit ratios. A better understanding of the diagnosis and management of this condition is required to alter the course of this modifiable disease.