title: Rare but Lethal Hepatopathy-Sickle Cell Intrahepatic Cholestasis and Management Strategies.
abstract: BACKGROUND
  Sickle cell disease can affect the liver by way of the disease process, including sickling in hepatic sinusoids, as well as its treatment, including repeated blood transfusions leading to hemosiderosis and hepatitis. Sickle cell intrahepatic cholestasis (SCIC) is an extreme variant of sickle cell hepatopathy, and is associated with high fatality.
  
  
  METHODS
  We present the case of a 31-year-old man with past medical history of sickle cell disease and cholecystectomy who was admitted with uncomplicated vaso occlusive crisis and during the hospital stay developed fever, upper abdominal pain, and jaundice. There was an accelerated rise in total bilirubin to 50 mg/dL, direct bilirubin 38 mg/dL, and Cr 3.0 mg/dL. Hb was 6.4 g/dL, reticulocyte count 16%, ALT 40 IU/L, AST 155 IU/L, ALP 320 IU/L, and LDH 475 IU/L. Hepatitis panel was negative and MRCP showed normal caliber of the common bile duct, with no obstruction. Exchange transfusion of 9 units of packed red blood cells led to great improvement in his condition.
  
  
  CONCLUSIONS
  SCIC, unlike the other sickle cell hepatopathies, requires urgent and vigorous exchange transfusion. Renal impairment in SCIC has not been well studied but usually is reversible with the hepatic impairment, as in this case. Unresolved renal impairment requires dialysis and is associated with poor outcome. There is limited data on use of hydroxyurea to prevent SCIC, and liver transplant is associated with high mortality. A timely diagnosis of SCIC and appropriate management is life-saving.
authors.affiliation: Department of Internal Medicine, Monmouth Medical Center, Long Branch, NJ, USA.
authors.forename: Aamir
authors.initials: A
authors.lastname: Malik
authors.affiliation: Department of Internal Medicine, Monmouth Medical Center, Long Branch, NJ, USA.
authors.forename: Chandni
authors.initials: C
authors.lastname: Merchant
authors.affiliation: Department of Internal Medicine, Monmouth Medical Center, Long Branch, NJ, USA.
authors.forename: Mana
authors.initials: M
authors.lastname: Rao
authors.affiliation: Department of Internal Medicine, Monmouth Medical Center, Long Branch, NJ, USA.
authors.forename: Rosemary P
authors.initials: RP
authors.lastname: Fiore
mesh_terms: D000328:Adult; D000755:Anemia, Sickle Cell / Q000150:complications* / Q000175:diagnosis / Q000628:therapy; D049448:Cholangiopancreatography, Magnetic Resonance; D002780:Cholestasis, Intrahepatic / Q000175:diagnosis / Q000209:etiology* / Q000628:therapy; D019468:Disease Management; D005078:Exchange Transfusion, Whole Blood / Q000379:methods*; D006801:Humans; D008099:Liver / Q000473:pathology*; D008297:Male