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.

Keywords: Sickle cell disease, Hepatopathy, Hepatobiliary, Intrahepatic cholestasis, Hepatic sequestration, Sickle cell hepatic crisis, Sickle cell cholangiopathy, Liver transplant, Iron overload

Core tip: This review: (1) identifies the pathophysiology, common clinical and biochemical features of a spectrum of hepatobiliary manifestations in sickle cell disease; (2) presents the current evidence of role of liver transplant in end stage liver disease due to sickle cell hepatopathy; and (3) identifies important areas of future research to explore unanswered questions regarding sickle cell hepatopathy.