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

Abstract

Sickle cell disease (SCD) is a common hemoglobinopathy which can affect multiple organ systems in the body. Within the digestive tract, the hepatobiliary system is most commonly affected in SCD. The manifestations range from benign hyperbilirubinemia to overt liver failure, with the spectrum of acute clinical presentations often referred to as “sickle cell hepatopathy”. This is an umbrella term referring to liver dysfunction and hyperbilirubinemia due to intrahepatic sickling process during SCD crisis leading to ischemia, sequestration and cholestasis. In this review, we detail the pathophysiology, clinical presentation and biochemical features of various acute and chronic hepatobiliary manifestations of SCD and present and evaluate existing evidence with regards to management of this disease process. We also discuss recent advances and controversies such as the role of liver transplantation in sickle cell hepatopathy and highlight important questions in this field which would require further research. Our aim with this review is to help increase the understanding, aid in early diagnosis and improve management of this important disease process.

Diverse manifestations of acute sickle cell hepatopathy in pediatric patients with sickle cell disease: A case series

“…Fulminant liver failure may require liver transplantation, but data on transplant outcomes in SCD are limited. 2 None of the patients presented here were on hydroxyurea or chronic transfusions prior to presentation; both may alter the onset and severity of liver damage in SCD. 20 When primary prevention of sickle hepatopathy is not possible, early diagnosis and aggressive management may limit morbidity and mortality.…”

“…Fresh frozen plasma cryoprecipitate, and/or factor replacement may be required. Fulminant liver failure may require liver transplantation, but data on transplant outcomes in SCD are limited …”

“…Hepatobiliary disease occurs in 10–40% of patients with sickle cell disease (SCD), and is an understudied, morbid SCD complication . Hepatic vaso‐occlusion, hepatic sequestration, and acute intrahepatic cholestasis, collectively termed “sickle hepatopathy” are the major SCD liver diseases . Sickle hepatopathy is caused by ischemic and fibrotic liver damage that is driven by SCD pathophysiology including erythrocyte sickling and adhesion, hypercoagulability, inflammation, and microvascular obstruction .…”

“…1 Hepatic vaso-occlusion, hepatic sequestration, and acute intrahepatic cholestasis, collectively termed "sickle hepatopathy" are the major SCD liver diseases. 2 Sickle hepatopathy is caused by ischemic and fibrotic liver damage that is driven by SCD pathophysiology including erythrocyte sickling and adhesion, hypercoagulability, inflammation, and microvascular obstruction. [3][4][5] Baseline hemolysis in SCD leads to high hepatic demand to degrade bilirubin.…”

Acute Pancreatitis as a Complication of Sickle Cell Anaemia

“…Within the digestive tract, the hepatobiliary system is most commonly affected in SCA. One of these manifestations is cholelithiasis, which is fairly common in patients with homozygous SCA, with an incidence of 26-58% in patients aged between 10-65 [10]. There is still controversy around prophylactic cholecystectomy in asymptomatic SCA patients with known gallstones.…”