Abstract

Liver involvement in sickle cell disease is multifactorial and is caused by either the vaso-occlusive phenomena that characterize the disease as well as hemolysis and transfusions or exchange transfusions that these patients often require and result in secondary liver hemochromatosis or transmission of infectious agents such as hepatitis B and C viruses. The acute and chronic liver complications of the disease are summarized under the term “sickle hepatopathy”. The liver may be involved in sickle cell crisis with acute liver crisis, acute hepatic sequestration or acute intrahepatic cholestasis. Other acute manifestations associated with the disease are viral hepatitis, liver abscesses and occlusion of large vessels of the liver. Chronic liver involvement is mainly related to secondary hemochromatosis and chronic viral hepatitis that often lead to liver cirrhosis and liver failure if not treated, but also related with the increased formation of gallstones as well as chronic sickle cholangiopathy after repeated episodes of ischemic injury to the biliary tree.