Acute sickle cell hepatopathy: A case report and literature review

Abstract

Sickle cell disease (SCD) is an inherited hemoglobinopathy with protean clinical manifestations. The liver could be affected by various SCD-associated complications of an overlapping nature. The clinical presentations of "sickle cell hepatopathy" range from clinically asymptomatic patients to those with life-threatening complications. Herein we report an SCD patient who presented with right upper quadrant abdominal pain and jaundice, eventually diagnosed as a self-limited form of acute sickle cell hepatopathy with overlapping features of acute hepatic crisis and benign intrahepatic cholestasis. Using this patient as an illustration, we will review the spectrum of hepatobiliary presentations in the SCD population.

Keywords: Acute hepatic crisis; Sickle cell disease; Sickle cell hepatopathy; Sickle cell intrahepatic cholestasis.