Abid Suddle, Consultant Hepatologist at the Institute of Liver Studies, Kings College Hospital, UK
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. Liver disease may be caused primarily by the sickling process, but also commonly occurs as a consequence of the multiple transfusions that these patients require during their lifetime. The clinical spectrum of disease ranges from mild liver function test abnormalities in asymptomatic patients, to dramatic clinical crises with marked hyperbilirubinemia and liver failure. Patients commonly present with one of several acute liver syndromes. Chronic liver disease is also common. Medical management of Sickle Cell Hepatopathy is limited and few treatments have a robust evidence base. World-wide experience of liver transplantation for Sickle Cell Hepatopathy is accruing slowly, many questions remain unanswered. The generally poor results of transplantation reported in the early clinical experience have been improved upon to some degree, reasons for this probably include better patient selection and haematologic management in the peri-transplant period. The role of transplantation for this condition is not as yet fully defined