title: Liver transplantation in a patient with acute liver failure due to sickle cell intrahepatic cholestasis.
abstract: BACKGROUND
  Sickle cell intrahepatic cholestasis is a potentially catastrophic 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 prevent sickle cell crisis.
  
  
  RESULTS
  Although the allograft functioned well initially, the patient developed veno-occlusive disease and required repeat transplantation at 5 months after transplant. Histologic examination of the explant revealed occlusion of the terminal hepatic venules due to fibrosis and packed red cells. Repeat transplant was complicated by thrombosis of the intrahepatic portion of the hepatic artery, and sepsis. The patient died of sepsis after a third transplant.
  
  
  CONCLUSIONS
  Liver transplantation for sickle cell disease involving the liver may carry a high risk of graft loss due to vascular problems. Repeat transplantation may not be feasible if disease recurs.
authors.affiliation: The Recanati/Miller Transplantation Institute, Mount Sinai Hospital of Mount Sinai-New York University Health, New York 10029-6574, USA. sukru_emre@smtplink.mssm.edu
authors.forename: S
authors.initials: S
authors.lastname: Emre
authors.forename: K
authors.initials: K
authors.lastname: Kitibayashi
authors.forename: M E
authors.initials: ME
authors.lastname: Schwartz
authors.forename: J
authors.initials: J
authors.lastname: Ahn
authors.forename: A
authors.initials: A
authors.lastname: Birnbaum
authors.forename: S N
authors.initials: SN
authors.lastname: Thung
authors.forename: C M
authors.initials: CM
authors.lastname: Miller
mesh_terms: D000755:Anemia, Sickle Cell / Q000150:complications*; D001706:Biopsy; D002648:Child; D002780:Cholestasis, Intrahepatic / Q000150:complications*; D006801:Humans; D008099:Liver / Q000473:pathology; D017093:Liver Failure / Q000209:etiology / Q000601:surgery*; D016031:Liver Transplantation / Q000473:pathology; D008297:Male