title: Liver transplantation in sickle cell anemia: a case of acute sickle cell intrahepatic cholestasis and a case of sclerosing cholangitis.
abstract: Very few cases of liver transplantation in patients with sickle cell disease have been reported in peer-reviewed literature. We reviewed the medical records of two patients with sickle cell disease that received liver transplantation at our institution. The first patient was a 27-year-old female who presented with encephalopathy and cholestatic jaundice with a Hemoglobin S (HbS) level of 69.6%. She was diagnosed with acute sickle cell intrahepatic cholestasis. The second patient was a 26-year-old female with sclerosing cholangitis who presented with encephalopathy, bleeding, and cholestatic jaundice. Her HbS level was normal. Both patients underwent liver transplantation successfully but died in the postoperative period from multiorgan failure. We report a rare case of liver transplantation for acute sickle cell intrahepatic cholestasis and a novel case of transplantation in a patient with sickle cell disease and sclerosing cholangitis. Liver transplantation did not lead to a successful outcome in either case.
authors.affiliation: Department of Medicine, Digestive and Liver Diseases Unit, University of Rochester, Strong Memorial Hospital, 601 Elmwood Avenue, Box 646, Rochester, NY 14642, USA. Matthew_Baichi@urmc.rochester.edu
authors.forename: Matthew M
authors.initials: MM
authors.lastname: Baichi
authors.forename: Razi M
authors.initials: RM
authors.lastname: Arifuddin
authors.forename: Parvez S
authors.initials: PS
authors.lastname: Mantry
authors.forename: Adel
authors.initials: A
authors.lastname: Bozorgzadeh
authors.forename: Charlotte
authors.initials: C
authors.lastname: Ryan
mesh_terms: D000328:Adult; D000755:Anemia, Sickle Cell / Q000150:complications* / Q000473:pathology; D015209:Cholangitis, Sclerosing / Q000473:pathology / Q000601:surgery*; D002780:Cholestasis, Intrahepatic / Q000473:pathology / Q000601:surgery*; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D016031:Liver Transplantation; D009102:Multiple Organ Failure / Q000209:etiology