Laparoscopic cholecystectomy in adult patients with beta-thalassemia or sickle cell disease

Excerpt

Sickle cell disease is a hereditary hemolytic anemia in which the sickle hemoglobin (HbS) substitutes for the normal adult hemoglobin (HbA). Beta-thalassemia is another hereditary hemolytic anemia in which the reduction of beta chain synthesis produces a marked decrease in normal HbA, with a compensatory increase in fetal hemoglobin (HbF). It is well-known that cholelithiasis, which is due to pigmented stones from increased hemoglobinolysis and bilirubin release, is more common in patients with sickle cell disease or thalassemia than in the general population; it occurs at younger age and is often symptomatic, requiring cholecystectomy. In addition, the modern appropriate management of patients with homozygous beta-thalassemia dealing mainly with secondary hemosiderosis and its consequences has increased survival time, prolonging life. Therefore, more patients may suffer from pigmented gallstone disease [5]. …