Successful treatment of acute sickle cell intrahepatic cholestasis with therapeutic plasma exchange

Acute sickle cell intrahepatic cholestasis (SCIC) is a potentially life-threatening complication of sickle cell disease (SCD) characterised by extreme hyperbilirubinaemia in the absence of significant obstruction by gallstones, and is caused by extensive sickling within the hepatic sinusoids leading to ischaemia and damage of hepatocytes with ballooning and intracanalicular stenosis and cholestasis. The aim was primarily to remove bilirubin from the circulation but also circulating pro-inflammatory cytokines and free Hb in an attempt to dampen the systemic inflammatory response and halt the sickling process. Biochemical response to therapeutic plasma exchange SCD is associated with a systemic inflammatory state at least partly due to ongoing limited bone marrow infarction and release of fat in the venous circulation with generation of pro-inflammatory cytokines under the action of secretory phospholipase A2 even at steady state, a situation accentuated during crises [ 4].