Gastrointestinal pathology in sickle cell disease

The literature was reviewed to investigate the existence of unique gastrointestinal (GI) pathological lesions in sickle-cell disease (SCD). Chole- and choledocholithiasis have long been recognized, but bilirubin gallstones can occur in any chronic hemolytic anemia. Acute pancreatitis has been reported as a possible ischemic consequence of sickling. It is unclear if the hepatic lesions of SCD differ from those of any chronically transfused population. Hepatic failure has been associated with massive sickling and hyperviscous bile ("sludge") has been linked to SCD. Elevated 5'-nucleotidase in the presence of elevated aminotransferase may suggest both hepatic and biliary tree involvement in a subgroup of patients with SCD. Low levels of the hepatically produced coagulation inhibitors, Protein S and Protein C, have been identified in SCD, but their precise relation to thrombosis in this instance remains unclear. Finally, a syndrome of intracanalicular cholestasis, sinusoidal dilation. Kupffer cell hyperplasia, and erythrophagocytosis has been linked to SCD. It has been suggested that the use of exchange transfusion prior to liver biopsy in this group of pediatric SCD patients may mask the pathophysiological role of sickled red blood cells in hepatic dysfunction. With the exception of some of the situations cited, it is concluded that most GI lesions in SCD are common to a heavily transfused population with chronic hemolytic anemia.