Sickle cell intrahepatic cholestasis: approach to a difficult problem.

Abstract

Sickle cell intrahepatic cholestasis is a rare but potentially fatal complication of sickle cell disease. Its characteristic features include hepatomegaly, extreme total hyperbilirubinemia, coagulopathy, and acute liver failure. Although the pathophysiology is uncertain, most reports in the medical literature indicate that the prognosis is grim. The only effective therapy that has been reported in this setting is exchange transfusion. We describe two hemoglobin SS patients with sickle cell intrahepatic cholestasis. We conclude that exchange transfusion and supportive care aimed at correction of coagulopathy, stabilization of the acute liver disease, and perhaps most important, avoidance of surgical intervention are the keys to a successful outcome.