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Sickle cell hepatopathy

Sickle cell hepatopathy encompasses a range of hepatic pathology arising from a wide variety of insults to the liver in patients with sickle cell disease. It occurs predominantly in patients with homozygous sickle cell anemia, and to a lesser extent in patients with sickle cell trait, Hb SC disease and Hb S b thalassemia. The hepatic disease may primarily be caused by the sickling process, but more commonly arises as a consequence of the multiple transfusions that these patients require in their lifetime. The term multitransfusion hepatopathy may therefore be more appropriate for these latter patients. The direct manifestations of sickle cell disease in the liver relate predominantly to vascular occlusion with acute ischemia, sequestration, and cholestasis, although chronic cholestatic syndromes have also been described. The main hepatic complications of multiple transfusions include acute and chronic infection with hepatitis B and C and iron overload. A further potential consequence of the chronic hemolysis is the development of pigment stones, with consequent cholecystitis and acute and chronic biliary obstruction from choledocholithiasis. Unfortunately, much of the literature on sickle cell hepatopathy lacks depth, because there are few sizeable or controlled studies, and most studies provide only incomplete information regarding coexisting chronic infection with hepatitis B or C, hepatic iron deposition, and chronic biliary obstruction from choledocholithiasis. It is therefore difficult to evaluate the contributing effects of these various insults in the final pathologic and clinical picture in these patients. As will be discussed subsequently, sickle cell anemia may lead to several unique liver syndromes (Table 1).