Reversal of liver function without exchange transfusion in sickle cell intrahepatic cholestasis

Abstract

Sickle cell intrahepatic cholestasis (SCIC) is a rare but fatal complication of sickle cell disease. It is found mainly in homozygous sickle cell disease. To date, there are no standard diagnostic criteria or well-established therapeutic approaches to this condition. Herein, we report this case of a 48-year-old man with sickle cell anemia and a total bilirubin of 78.5 mg/dL without evidence of extrahepatic biliary obstruction or viral hepatitis. The patient had a hemoglobin S level of 87.9%, acute renal failure, and mild coagulopathy. Despite the disease severity, he refused exchange transfusion (ET) with packed red blood cells. He was transfused with 2 units of blood and treated mainly with supportive measures. His total bilirubin levels trended down to normal days after discharge. Multiple studies have shown a significant decrease in the mortality rate in SCIC after ET. To date, only two reported adult cases have survived SCIC without aggressive treatment. Our case is the third case that demonstrates recovery of severe SCIC without ET.

Figure 1.

Hemoglobin, bilirubin, and hemoglobin S level in relation to hospital course.