Title : Sickle cell liver disease: A multidisciplinary hemato-hepatology approach
Abstract:
Sickle cell disease (SCD) is a common inherited hematological disorder in Saudi Arabia, and hepatic involvement is an important but frequently underestimated component of its multisystem burden. This presentation reviews the spectrum of sickle cell liver disease (SCLD), highlights clinically important acute and chronic manifestations, and proposes a multidisciplinary hemato-hepatology model to improve recognition and management. Published data demonstrate that chronic liver disease is not uncommon in SCD: an autopsy series of 141 adults identified cirrhosis in 11.3%, while a prospective National Institutes of Health cohort of 247 patients reported liver fibrosis in 4.5% and 9% mortality during follow-up. In that cohort, ferritin levels above 1,000 µg/L were associated with poorer survival, and patients with elevated ferritin who underwent liver biopsy had substantial hepatic iron deposition, supporting the importance of assessing transfusional iron burden and progressive liver injury. The acute hepatic syndromes of SCD include acute sickle hepatic crisis/sinusoidal obstruction, hepatic sequestration, and sickle cell intrahepatic cholestasis. Severe vaso-occlusion may progress to massive hepatic injury and acute liver failure. Sickle cell cholangiopathy is another important manifestation, in which recurrent vaso-occlusion and ischemic injury to the biliary circulation may lead to bile duct dilatation, ischemic cholangitis, and intrahepatic stones, sometimes in the absence of gallbladder stones. Because these presentations may overlap with other causes of jaundice and liver dysfunction, coordinated assessment by hematology and hepatology is essential. A dedicated Sickle Cell-Liver Disease Clinic can provide comprehensive evaluation, streamlined referral pathways, regular interdisciplinary review, and patient-centered treatment planning while also supporting research, education, and international collaboration. Establishing a robust hemato-hepatology program may facilitate earlier diagnosis, more individualized management, and greater awareness of liver complications across the SCD care pathway.
