Dilemmas and Challenges With Living Donor Transplantation in Adult Hemophagocytic Lymphohistiocytosis-related Acute Liver Failure: A Case Report and Review of the Literature.

Acute liver failure (ALF) is a lethal consequence of acute hepatic injury characterized by altered mental status and coagulation abnormalities associated with high mortality. While viral hepatitis and drug-induced liver injury are the most common causes of ALF, a significant fraction remains indeterminate. Hemophagocytic lymphohistiocytosis (HLH) as a cause of liver failure has been described in the pediatric population but is infrequently described in adults. Identification and treatment of triggers in conjunction with immunosuppression is the mainstay of treatment in HLH. However, once ALF sets in a setting of HLH, the prognosis is grave. Liver transplantation has been described as a salvage procedure in HLH; however, the data on this approach in adult HLH are sparse. Multiple challenges exist in ascertaining an accurate diagnosis, use of immunosuppression in the face of fulminant liver failure, dilemmas in LT decisions, and uncertainties of outcomes. We describe a case of adult HLH with an unknown trigger who underwent living donor transplantation and had an adverse outcome and review the literature on adult HLH with ALF requiring LT.