SICKLE CELL INTRAHEPATIC CHOLESTASIS WITH CHOLELITHIASIS

Karina Irizarry Department of Pediatrics, University of South Florida College of Medicine, Tampa, Florida, USA

Hans Christof Rossbach Department of Pediatrics, University of South Florida College of Medicine, Tampa, Florida, USA

Joseph R. A. Ignacio Department of Pediatrics, University of South Florida College of Medicine, Tampa, Florida, USA

Michele P. Winesett Department of Pediatrics, University of South Florida College of Medicine, Tampa, Florida, USA

Greg C. Kaiser Department of Pediatrics, University of South Florida College of Medicine, Tampa, Florida, USA

Mudra Kumar Department of Pediatrics, University of South Florida College of Medicine, Tampa, Florida, USA

Enid Gilbert-Barness Department of Pediatrics and Department of Pathology, University of South Florida College of Medicine, Tampa, Florida, USA

Michael J. Wilsey Jr. Department of Pediatrics, University of South Florida College of Medicine, Tampa, Florida, USA

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Abstract

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 life-threatening complications. Diagnosis can be challenging given the overlap in clinical presentation with other conditions affecting the hepatobiliary biliary system in 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.

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SICKLE CELL INTRAHEPATIC CHOLESTASIS WITH CHOLELITHIASIS

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