Gastrointestinal pathology in sickle cell disease

Area of Science:

• Gastroenterology
• Hematology
• Pathology

Background:

• Sickle-cell disease (SCD) is a chronic hemolytic anemia with known gastrointestinal (GI) complications.
• Cholelithiasis and choledocholithiasis are recognized but can occur in any chronic hemolytic anemia.
• Hepatic and biliary complications in SCD require further differentiation from those in chronically transfused populations.

Purpose of the Study:

• To investigate unique gastrointestinal pathological lesions in sickle-cell disease (SCD).
• To differentiate SCD-specific GI manifestations from those common to chronic hemolytic anemia and transfusion therapy.

Main Methods:

• Literature review of gastrointestinal and hepatic manifestations in sickle-cell disease.
• Analysis of reported pathological lesions, including gallstones, pancreatitis, and hepatic dysfunction.
• Evaluation of specific biomarkers and histological findings associated with SCD.

Main Results:

• Bilirubin gallstones and acute pancreatitis are linked to SCD but also occur in other hemolytic anemias or as ischemic events.
• Hepatic lesions in SCD may overlap significantly with those in chronically transfused individuals.
• Specific syndromes like intracanalicular cholestasis and Kupffer cell hyperplasia are noted in SCD, though their pathophysiology can be masked by treatments like exchange transfusion.

Conclusions:

• Most gastrointestinal lesions in SCD are not unique and are shared with heavily transfused patients with chronic hemolytic anemia.
• Further research is needed to clarify the specific role of sickled red blood cells in hepatic dysfunction in SCD.
• Elevated 5'-nucleotidase and altered coagulation inhibitors suggest potential hepatic and biliary involvement in a subgroup of SCD patients.