title: Case of fatal sickle cell intrahepatic cholestasis despite use of exchange transfusion in an African-American patient.
abstract: Sickle cell intrahepatic cholestasis (SCIC) is a rare complication of sickle cell anemia, characterized by marked hyperbilirubinemia and acute hepatic failure with an often fatal course. However, the few reported adult cases that were treated with exchange transfusion had a favorable outcome. We herein describe a 48-year-old African-American man with hemoglobin S/B thalassemia and previously treated hepatitis C with compensated cirrhosis, who presented with a total bilirubin of 59.7 mg/dL and direct bilirubin of 43.6 mg/dL in the absence of choledocholithiasis. Despite an exchange transfusion and aggressive packed red blood cell transfusions, which successfully decreased the hemoglobin S levels to <15%, he perished from progressive hepatic and renal failure. Autopsy demonstrated extensive intrahepatocellular and intracanalicular cholestasis in a background of cirrhosis. Our case suggests that poor prognostic factors for adult SCIC patients treated with exchange transfusion may include older age and underlying hepatic disease.
authors.affiliation: Department of Medicine, Division of Hemotology/Oncology, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, MA 02215, USA.
authors.forename: Daniel B
authors.initials: DB
authors.lastname: Costa
authors.forename: Rebecca A
authors.initials: RA
authors.lastname: Miksad
authors.forename: Michael S
authors.initials: MS
authors.lastname: Buff
authors.forename: Yihong
authors.initials: Y
authors.lastname: Wang
authors.forename: Bruce J
authors.initials: BJ
authors.lastname: Dezube
mesh_terms: D001741:Black or African American; D000755:Anemia, Sickle Cell / Q000150:complications* / Q000453:epidemiology*; D002780:Cholestasis, Intrahepatic / Q000209:etiology*; D015897:Comorbidity; D017707:Erythrocyte Transfusion; D005078:Exchange Transfusion, Whole Blood; D017809:Fatal Outcome; D005355:Fibrosis / Q000453:epidemiology*; D006526:Hepatitis C / Q000453:epidemiology*; D006801:Humans; D008099:Liver / Q000473:pathology; D017093:Liver Failure / Q000209:etiology; D008297:Male; D008875:Middle Aged; D051437:Renal Insufficiency / Q000209:etiology; D017086:beta-Thalassemia / Q000453:epidemiology*