Pediatric Hepatobiliary Lithiasis in Homozygous Sickle Cell Disease: Laboratory Insights Into Cholestasis and Hemolysis

Abstract

Chronic hemolysis in sickle cell disease (SCD) predisposes patients to hepatobiliary complications, including pigment gallstones. We report a 13-year-old girl with hemoglobin SS SCD who presented with right upper quadrant pain, progressive jaundice, and fever. Laboratory evaluation revealed severe anemia with reticulocytosis, mixed hyperbilirubinemia, elevated lactate dehydrogenase, normal cholestatic enzymes, and marked C-reactive protein elevation. Imaging confirmed microlithiasis without significant bile duct obstruction. Supportive care included transfusion, hydration, antibiotics, and pain control. Elective cholecystectomy was planned after stabilization. This case underscores the diagnostic value of combined hemolysis and cholestasis markers for early recognition of hepatobiliary complications in pediatric patients with SCD.