Cholelithiasis in patients with major sickle hemoglobinopathies

Area of Science:

• Hematology
• Gastroenterology
• Medical Diagnostics

Background:

• Cholelithiasis is a known complication in homozygous sickle cell disease (Hb SS).
• The prevalence of gallstones in patients with heterozygous sickle hemoglobinopathies remains largely uncharacterized.
• Sickle hemoglobinopathies encompass a spectrum of genetic conditions affecting hemoglobin.

Purpose of the Study:

• To investigate the frequency of cholelithiasis in patients with various major sickle hemoglobinopathies.
• To assess the association between gallstone formation and different sickle cell genotypes.
• To determine the overall risk of biliary tract disease in this patient population.

Main Methods:

• Sonographic examinations were conducted on 65 unselected patients diagnosed with major sickle hemoglobinopathies.
• Patients were categorized based on their specific sickle hemoglobinopathy diagnosis (Hb SS, Hb SC, Hb S-beta-thalassemia).
• Data on gallstone presence and patient age were collected and analyzed.

Main Results:

• Cholelithiasis was detected in 26% of Hb SS patients (11/42), 20% of Hb SC patients (3/15), and 12.5% of Hb S-beta-thalassemia patients (1/8).
• A positive correlation was observed between the presence of gallstones and increasing patient age.
• These findings highlight a significant prevalence of gallstones across different sickle hemoglobinopathy types.

Conclusions:

• All patients with major sickle hemoglobinopathies are susceptible to developing gallstones.
• Early detection and monitoring for biliary tract complications are crucial in managing sickle cell disease.
• Heterozygous sickle hemoglobinopathies also carry a notable risk for cholelithiasis, necessitating further clinical attention.