Exchange transfusion for sickle cell intrahepatic cholestasis.

Only one of eight previously described patients with sickle cell intrahepatic cholestasis (SCIH) survived. Death usually resulted from liver failure and/or a hemorrhagic diathesis. This report deals with the successful treatment of SCIH by partial blood-plasma exchange transfusion. This treatment led to regression of hepatic encephalopathy, impending bleeding diathesis, and noticeable clinical improvement withn 48 hours. Within six days, the serum bilirubin level decreased from 146 to 16 mg/dL. Partial blood-plasma exchange transfusion may be valuable for the treatment of SCIH.