![]() It occurs predominantly in patients with homozygous HbSS disease, and to a lesser extent in patients with hemoglobin SC disease or HbS β-thalassemia. As such, it includes diverse hepatic pathology arising from a variety of insults to the liver that can occur in these patients. “Sickle hepatopathy” is an umbrella term, encompassing the range of liver disease encountered in patients with sickle cell anemia. This included EBT to maintain the HbS percentage at <30 to 40. A protocol was developed with the sickle hematology team for optimal hematologic management in the peri- and postoperative period. A rigorous evaluation excluded significant sickle-related heart, lung, kidney, or brain dysfunction. Regular exchange blood transfusions were instituted with an HbS percentage target of 20% at 1 year) justified assessment at this time. With this regimen, symptoms and acute liver dysfunction resolved, but bilirubin did not return to the preepisode baseline. Treatment included IV fluids, antibiotics, analgesia, and exchange blood transfusion (EBT) with the aim of reducing the HbS fraction to <30% to 40%. The patient was managed as having an acute sickle liver in the context of an acute vaso-occlusive crisis. Cross-sectional imaging demonstrated hepatomegaly, mild intrahepatic bile duct dilation without evidence for bile duct stones, and patency of hepatic vasculature. Liver function tests showed an acute deterioration: bilirubin, 34.4 mg/dL (predominantly unconjugated) alanine transaminase, 341 IU/L alkaline phosphatase, 430 IU/L albumin, 32 g/L. At clinical examination, he was found to be deeply icteric, febrile at 37.8☌, with right upper quadrant tenderness elicited on abdominal palpation. ![]() At age 26 years, the patient presented to the emergency department with a further episode of jaundice and abdominal pain, in the context of a sickle crisis precipitated by viral infection. ![]()
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