Table 2.

Management recommendations in acute sickle hepatopathy

Clinical scenarioClinical/investigative dataManagement recommendations
Gallstones May be asymptomatic
Right upper quadrant pain
Cholangitis 
Cholecystectomy for symptomatic gallstones
ERCP with duct clearance for bile duct stones 
Acute sickle hepatic crisis Vaso-occlusive crisis
RUQ pain/jaundice/leukocytosis
Bilirubin <15 mg/dL
ALT rarely >300 IU/L 
Supportive
Consider exchange blood transfusion 
Sickle cell intrahepatic cholestasis Vaso-occlusive crisis
RUQ pain, leukocytosis, fever, striking jaundice
Very high bilirubin
ALT can be in 1000s
Coagulopathy
Renal failure 
Full supportive management
Exchange blood transfusion 
Hepatic sequestration Enlarging liver
RUQ pain
Anemia
Reticulocytosis 
Supportive
Transfusion 
Clinical scenarioClinical/investigative dataManagement recommendations
Gallstones May be asymptomatic
Right upper quadrant pain
Cholangitis 
Cholecystectomy for symptomatic gallstones
ERCP with duct clearance for bile duct stones 
Acute sickle hepatic crisis Vaso-occlusive crisis
RUQ pain/jaundice/leukocytosis
Bilirubin <15 mg/dL
ALT rarely >300 IU/L 
Supportive
Consider exchange blood transfusion 
Sickle cell intrahepatic cholestasis Vaso-occlusive crisis
RUQ pain, leukocytosis, fever, striking jaundice
Very high bilirubin
ALT can be in 1000s
Coagulopathy
Renal failure 
Full supportive management
Exchange blood transfusion 
Hepatic sequestration Enlarging liver
RUQ pain
Anemia
Reticulocytosis 
Supportive
Transfusion 

Adapted from Tavabie and Suddle with permission.

ALT, alanine aminotransferase; RUQ, right upper quadrant.

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