Sickle Cell Hepatopathy

The term ‘Sickle Cell Hepatopathy’ encompasses a range of hepatic dysfunction arising from a wide variety of insults to the liver in patients with sickle cell disease(SCD). It occurs predominantly in patients with homozygous sickle cell anemia, and to a lesser extent in patients with sickle cell trait, HbSC disease and HbSb Thalassemia.The liver can be affected by a number of complications due to the disease itself and its treatment. The direct affection of liver in sickle cell disease is predominantly due to vascular occlusion by sickled RBCs with acute ischemia, sequestration, and cholestasis. The risk of viral hepatitis B and C and iron overload due to multiple blood transfusions and chronic hemolysis leading to the development of pigment stones, with consequent cholecystitis and choledocholithiasis contribute to the development of liver disease. Reversible hepatic toxicity may be seen with androgenic steroids used in the past as a therapy for SCD with severe anaemia. In some cases cardiac failure may lead to hepatocellular damage in SCD.