The literature was reviewed to investigate the existence of unique gas
trointestinal (GI) pathological lesions in sickle-cell disease (SCD).
Chole- and choledocholithiasis have long been recognized, but bilirubi
n gallstones can occur in any chronic hemolytic anemia. Acute pancreat
itis 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 lin
ked to SCD. Elevated 5'-nucleotidase in the presence of elevated amino
transferase 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 rem
ains unclear. Finally, a syndrome of intracanalicular cholestasis, sin
usoidal dilation, Kupffer cell hyperplasia, and erythrophagocytosis ha
s been linked to SCD. It has been suggested that the use of exchange t
ransfusion prior to liver biopsy in this group of pediatric SCD patien
ts may mask the pathophysiological role of sickled red blood cells in
hepatic dysfunction. With the exception of some of the situations cite
d, it is concluded that most GI lesions in SCD are common to a heavily
transfused population with chronic hemolytic anemia.