Acute and chronic hepatobiliary manifestations of sickle cell disease: A review

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.

Table 2 Chronic hepatobiliary manifestations of sickle cell disease

Chronic hepatobiliary manifestations of SCD	Clinical presentation	Biochemical changes			Management
		Transaminase (AST, ALT) levels	Bilirubin	Alkaline phosphatase
Cholelithiasis	RUQ pain, fever, jaundice	Normal or elevated	Normal or elevated	Normal	Cholecystectomy
Choledocholithiasis	RUQ pain, fever, jaundice, cholangitis	Normal or elevated	Elevated	Elevated	ERCP
Iron overload	Asymptomatic elevated LFTs to frank cirrhosis	Normal or elevated	Normal to mild elevation	Normal	Iron chelation
Viral hepatitis	Viral prodrome, fever, hepatomegaly, jaundice	Acute-elevated	Acute-elevated	Acute - normal to slightly elevated;	Based on AASLD guidelines
		Chronic-normal or elevated	Chronic-normal or elevated	Chronic - mostly normal
Sickle cell cholangiopathy	Obstructive jaundice, itching, cholestatic LFTs	Normal or elevated	Elevated	Elevated	ERCP
					liver transplant

SCD: Sickle cell disease; AST: Aspartate transaminase; ALT: Alanine transaminase; RUQ: Right upper quadrant; ERCP: Endoscopic retrograde cholangiopancreatography; LFTs: Liver function tests; AASLD: American Association for the Study of Liver Diseases.

Table 3 Current evidence of liver transplantation in sickle cell disease

Author	Number of patients	Outcomes
Hurtova et al[46]	6	1, 3, 5, and 10-yr survival rates were 83.3%, 66.7%, 44.4%, and 44.4%, respectively
Mekeel et al[48]	3	Patient and graft survival was 66%
Baichi et al[49]	2	100% mortality in post-transplant period due to multiorgan failure
Emre et al[50]	1	Failure of graft in 5 mo due to SCD crisis
Greenberg et al[51]	1	Successful but follow up only till day 28
Kindscher et al[52]	1	Successful with extrahepatic complications
Lang et al[53]	1	Successful at 6 mo
Ross et al[54]	1	Successful at 22 mo - death due to PE
van den Hazel et al[55]	1	Successful at 5.5 yr
Gilli et al[56]	1	Successful at 2 yr
Berry[57]	1	Death in post-op period