Sickle cell intrahepatic cholestasis unresponsive to exchange blood transfusion: a case report

Sickle cell intrahepatic cholestasis (SCIC) is an uncommon but severe complication in sickle cell disease (SCD) patients homozygous for hemoglobin (Hb) S or with Hb S/ thalassemia.1–3 It is clinically characterized by marked conjugated hyperbilirubinemia, right upper quadrant pain, enlarged liver and moderately elevated hepatic enzymes. In more severe cases, coagulopathy and renal dysfunction may be observed and the condition occasionally progresses to liver failure. SCIC is most commonly described in its acute or recurrent forms but it eventually becomes chronic. The pathogenesis of SCIC involves sickling within the hepatic sinusoids leading to vascular stasis, hypoxia and ballooning of the hepatocytes, resulting in intracanalicular cholesthasis.1,4 Treatment is based on exchange blood transfusions (EBT) and aims to reduce Hb S blood levels and consequently the sickling process. Although several case reports show reversal of SCIC with prompt EBT,1,3,5 we describe a case of a non-responsive patient who perished despite treatment.