Hepatobiliary manifestations of sickle cell disease

Abstract

The present review updates the miscellaneous hepatobiliary complications of sickle cell disease. Polymerisation of deoxy haemoglobin (Hb) S and vasoocclusion can cause severe liver injury and malfunction. The acute complications or acute clinical syndromes can be attributed to vascular occlusion (acute sickle hepatic crisis, hepatic sequestration and intrahepatic cholestasis). Complications of chronic haemolysis and multiple transfusions are also common: cholelithiasis, hepatic iron overload, viral hepatitis B and C. And finally, there are more rare complications related to the sickle cell state: autoimmune hepatitis, hepatic infarction, hepatic abscess, hepatic biloma, Budd-Chiari syndrome, hyperammonaemia due to zinc deficiency. The clinical presentation, the laboratory findings, abdominal imaging and sometimes liver biopsy can be useful in establishing the correct diagnosis. Hydroxyurea, and blood straight or exchange transfusions for decreasing the level of HbS, are the usual procedures in the treatment of these clinical entities. Early recognition, diagnosis, and prompt treatment are the cornerstone for a favourable outcome of these disorders. Copyright © Hellenic Society of Haematology.