LIVER INVOLVEMENT IN WHITE PATIENTS WITH SICKLE-CELL DISEASE

Background: Liver involvement and cholelithiasis are common complicati ons of sickle-cell disease. The incidence of clinically evident hepati c damage reported in the literature for black people varies from 15% t o 30%, while no data are reported for white people. Objective: To eval uate the liver involvement in 40 patients with homozygous sickle cell anemia (the beta(s) beta(s) form of sickle-cell disease) and 102 patie nts with double-heterozygous hemoglobin S and P-thalassemia (65 with t he beta(s) beta(0th) and 37 with the beta(s) beta(+th) form of sickle- cell disease).Setting: The Department of Pediatric Hematology and Onco logy, University of Catania, Catania, Italy. Patients: Outpatients wit h sickle-cell disease. Results: We found that, in our patients, liver disease seems to be clinically irrelevant: only 2 of the 142 patients examined had notable alterations in hepatic function. Cholelithiasis w as found in 42.1% of the subjects with the beta(s) beta(s) form of sic kle-cell disease and in 26.8% of the subjects with the beta(s) beta(th ) form. Age-related analysis revealed a greater incidence of cholelith iasis during the first years of life in the patients with the beta(s) beta(s) form of the disease than in patients with the beta(s) beta(th) form. Conclusion: Our data showed that liver involvement in sickle-ce ll disease is clinically irrelevant, reflecting the fact that the clin ical expression of sickle-cell disease in Sicilian patients is moderat e.