Red Blood Cell Exchange Followed by Plasma Exchange in Patients with Intrahepatic Cholestasis due to Sickle Cell Disease

Abstract

Intrahepatic cholestasis, a rare complication of sickle cell anemia, is characterized by marked hyperbilirubinemia, acute hepatic failure, and an often fatal course. In this report, we present patients with homozygous hemoglobin S and one patient with sickle-beta thalassemia disease who have intrahepatic cholestasis. Despite automated red blood cell exchange transfusion, which successfully decreased the hemoglobin S level to less than 30% of total hemoglobin level in the peripheral blood, basic biochemical laboratory examination revealed signs of cholestasis with a serum bilirubin level of up to 50 mg/dL in the first case, 30 mg/dL in the second, and 10 mg/dL in the third. The patients underwent plasma exchange, which improved their clinical condition except one. These cases suggest that plasma exchange may have a role in improving the clinical condition of patients with sickle cell disease complicated with intrahepatic cholestasis (SCIC) that fails to respond to automated red blood cell exchange therapy