Liver transplantation in a patient with acute liver failure due to sickle cell intrahepatic cholestasis

Background. Sickle cell intrahepatic cholestasis is a potentially catastrop hic complication of sickle cell anemia. Once acute liver failure develops, transplantation is the only option. We describe a patient with sickle cell intrahepatic cholestasis who underwent liver transplantation. Methods. Data were obtained from the chart. Serial hemoglobin S levels were monitored, and measures were taken to maintain hemoglobin S <20% to preven t sickle cell crisis. Results. Although the allograft functioned well initially, the patient deve loped veno-occlusive disease and required repeat transplantation at 5 month s after transplant. Histologic examination of the explant revealed occlusio n of the terminal hepatic venules due to fibrosis and packed red cells. Rep eat transplant was complicated by thrombosis of the intrahepatic portion of the hepatic artery, and sepsis. The patient died of sepsis after a third t ransplant. Conclusion. Liver transplantation for sickle cell disease involving the liv er may carry a high risk of graft loss due to vascular problems. Repeat tra nsplantation may not be feasible if disease recurs.