Copyright ©The Author(s) 2017. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Gastrointest Pathophysiol. Aug 15, 2017; 8(3): 108-116
Published online Aug 15, 2017. doi: 10.4291/wjgp.v8.i3.108
Acute and chronic hepatobiliary manifestations of sickle cell disease: A review
Rushikesh Shah, Cesar Taborda, Saurabh Chawla
Rushikesh Shah, Saurabh Chawla, Division of Digestive Diseases, Department of Internal Medicine, Emory University School of Medicine, Atlanta, GA 30322, United States
Rushikesh Shah, Cesar Taborda, Saurabh Chawla, Department on Medicine, Emory University School of Medicine, Atlanta, GA 30322, United States
Saurabh Chawla, Grady Memorial Hospital, Atlanta, GA 30303, United States
Author contributions: Shah R and Taborda C contributed significantly to literature review and manuscript preparation; Shah R and Chawla S contributed significantly to conception, interpretation and revision.
Conflict-of-interest statement: This review is contributed by all authors as a voluntary work. Authors have no commercial, financial, political, religious or any other conflicts of interest for contributing to this review.
Open-Access: This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See:
Correspondence to: Saurabh Chawla, MD, Assistant Professor of Medicine, Division of Digestive Diseases, Department of Internal Medicine, Emory University School of Medicine, Faculty Office Building, 49 Jesse Hill Jr. Drive, Suite 431, Atlanta, GA 30322, United States.
Telephone: +1-140-47781684 Fax: +1-140-47781681
Received: March 21, 2017
Peer-review started: March 23, 2017
First decision: May 19, 2017
Revised: June 2, 2017
Accepted: July 21, 2017
Article in press: July 24, 2017
Published online: August 15, 2017

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.