Table 1.

Acute hepatobiliary manifestations of sickle cell disease

Acute manifestations of SCD	Clinical presentation	Biochemical changes			Management
		Transaminase (AST, ALT) levels	Bilirubin	Alkaline phosphatase
Acute sickle cell hepatic crisis	Fever, acute onset RUQ pain, jaundice and tender hepatomegaly	Normal to 3 × upper normal	Upto 15 mg/dL, mainly conjugated	Normal to slight elevation	Supportive with treatment of SCD crisis
Acute Hepatic sequestration	Acute onset RUQ pain, hepatomegaly and anemia	Normal	Upto 24 mg/dL, mainly conjugated	Can go upto 650 IU/L	Supportive with blood or exchange transfusion
Acute intrahepatic cholestasis	Fever, RUQ pain rapidly progressing to acute liver failure	Elevated usually > 1000	Elevated in 100 s, mostly conjugated	Normal or elevated > 1000 IU/L	Supportive, exchange transfusion, correction of coagulopathy? Liver transplant

SCD: Sickle cell disease; AST: Aspartate transaminase; ALT: Alanine transaminase; RUQ: Right upper quadrant.