Sickle cell intrahepatic cholestasis with cholelithiasis

Sickle cell intrahepatic cholestasis (SCIC) is a rare complication seen in sickle cell patients who present with sudden onset of RUQ pain, progressive hepatomegaly, mild elevation of transaminases, coagulopathy, and extreme hyperbilirubinemia. Early recognition of this entity is essential to avoid l life threatening complications. Diagnosis can be challenging given the overlap in clinical presentation. with other conditions affecting the hepatobiliary bilary systemin sickle cell anemia such as hepatitis, cholecystitis, and hepatic crisis. Treatment is currently limited, to exchange transfusion. The authors present two patients with SCIC and cholelithiasis; the clinical picture of one is complicated by choledocholithiasis.