FREQUENCY AND OUTCOME OF HEPATOBILIARY DISORDERS IN ADULT HOMOZYGOUS SICKLE CELL DISEASE PATIENTS

Background: Sickle cell disease (SCD) may affect the hepatobiliary system at different ages in different ways. The liver may be affected by intrasinusoidal sickling, vaso-occlusion, intrahepatic cholestasis, iron overload and viral hepatitis. The biliary tree could be affected by gallstones, biliary sludge, and obstruction. Objective: The aim of this study was to identify the frequency and outcome of hepatobiliary disorders in adult patients with homozygous SCD. Methods: In this prospective screening study we evaluated the demographic, clinical, laboratory, radiologic and histopathologic data concerning hepatic and biliary disorders with emphasis on the related complications and mortality, in 118 adult Saudi patients with homozygous SCD, over three years of follow up, in Yanbu, Saudi Arabia. Results: The mean age of all participants was 27.9 ± 9.0 (18-46) years and 55.9% were males. Hepatobiliary disorders were discovered in 90.7% of patients, including acute conditions like, hepatic crisis (9.3%), acute calcular cholecystitis (7.6%) and acute sickle cell hepatopathy (5.9%), and chronic conditions like, hepatomegaly (61.9%), asymptomatic cholelithiasis (33.9%), hepatic iron overload (31.4%), as well as viral hepatitis C (16.9%) and B (11.0%). Intrasinusoidal sickling and Kupffer cell hyperplasia were consistently seen in all liver biopsies (n=25), denoting a degree of vascular process. There were 11 cholecystectomies (9.3%), seven cases with hepatic dysfunction (5.9%), one mortality (0.8%) due to acute severe sickle hepatopathy, and un otherwise uncomplicated clinical course in most cases. Conclusion: Hepatobiliary disorders are common in adult homozygous SCD patients and often have a benign disease course.