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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. Mini ReviewSickle cell disease (SCD) is one of the most common monogenic disorders worldwide. The replacement of glutamic acid by valine at position 6 of the β globin molecule leads to the polymerization of hemoglobin S (HbS) in conditions of hypoxemia, with consequent intravascular hemolysis and vaso-occlusive effects. The disease affects a variety of organs of the human body, with the liver being one of the organs most frequently affected, both acutely and in chronic. The acute and chronic liver complications due to sickle cell disease are briefly described as "sickle hepatopathy" [1]. Sickle hepatopathy is primarily and predominantly associated with the pathophysiology of the disease itself, namely vaso-occlusive phenomena and hemolysis as a consequence of the sickling process, and secondary with the transfusion or exchange transfusion therapy that are often required in these patients.