Sickle cell disease (SCD) frequently affects the liver; if
acute liver failure (ALF) develops, the only potentially effective therapeutic option is
liver transplantation (LT). Only 12 patients for whom LT was performed for SCD-related ALF have been described so far. We report a retrospective series of 6 adult patients with SCD (3 men and 3 women, median age = 40.1 years) who underwent emergency LT. The indication for LT was ALF complicating
cirrhosis in 5 patients (
hepatitis C/
iron overload-induced
cirrhosis in 3 and
iron overload-induced
cirrhosis in 2); one patient had
autoimmune hepatitis. The median follow-up was 52.7 months (0.5-123 months). The 1-, 3-, 5-, and 10-year survival rates were 83.3%, 66.7%, 44.4%, and 44.4%, respectively. One patient died of hepatocellular failure precipitated by hyperacute allograft rejection on post-LT day 10. Soon after LT, 2 patients developed
seizures; in 1 case, the
seizures were a complication of early
calcineurin inhibitor-induced
leukoencephalopathy. Four long-term survivors benefited from specific management of SCD; specifically, the
hemoglobin S fraction was maintained below 30% and the total
hemoglobin level was maintained between 8 and 10 g/dL. Two patients had mild vaso-occlusive crises. Three patients experienced a recurrence of hepatitis C virus (HCV)
infection; 2 of these patients experienced reversible neurological complications while they were receiving
antiviral treatment. Carefully selected patients with SCD may benefit from emergency LT. However, such patients seem to be particularly susceptible to neurological complications after LT. In contrast, severe SCD-related crises do not seem to recur if specific management is provided. Outcomes may be improved if the neurological complications can be minimized; for example, the administration of a
calcineurin inhibitor can be delayed, and the management of HCV
infection recurrence can be improved.