☆ 4.5 Article

Transplantation for Liver Failure in Patients With Sickle Cell Disease: Challenging But Feasible

LIVER TRANSPLANTATION (2011)

Journal

LIVER TRANSPLANTATION

Volume 17, Issue 4, Pages 381-392

Publisher

WILEY

DOI: 10.1002/lt.22257

Keywords

Ask authors/readers for more resources

Protocol

Community support

Reagent

Community support

Abstract

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. Liver Transpl 17:381-392, 2011. (C) 2011 AASLD.

Authors

I am an author on this paper

Click your name to claim this paper and add it to your profile.

Reviews

Primary Rating

4.5

Not enough ratings

Secondary Ratings

Novelty

Significance

Scientific rigor

Rate this paper

The Liver in Sickle Cell Disease

Florence Lacaille, Slimane Allali, Mariane de Montalembert

Summary: Liver involvement is common in children with sickle cell disease, with complications such as cholelithiasis and acute hepatic crisis. Exchange transfusion is the emergency treatment, while chronic cholangiopathy and iron overload should also be monitored and addressed. Collaboration between hepatologists and specialists of sickle cell disease is essential for the management of these complications.

JOURNAL OF PEDIATRIC GASTROENTEROLOGY AND NUTRITION (2021)