Decompensated Cirrhosis and Sickle Cell Disease : Case Reports and Review of the Literature

Although its prevalence is unknown, liver involvement by sickle cell disease is not uncommon and encompasses different clinical spectra including non cholestatic and cholestatic disorders. Few data have been provided on chronic sickle cell intrahepatic cholestasis (SCIC) clinical course, although cirrhosis has been reported in sickle cell disease. However, no effective therapeutic approaches have been recognized either to prevent or treat this condition. Here we present two cases of adult sickle cell disease patients with decompensated cirrhosis. Their liver biopsies showed sickle cell thrombi within the hepatic sinusoids. Despite erythroexchange (EEX) transfusions, both patients suffered from major sickle cell disease-related events, suggesting that EEX transfusions may not be enough to impact on advanced liver involvement by sickle cell disease.