Sickle-cell intrahepatic cholestasis (SCIC) is an uncommon complication of sickle-cell disease (SCD), which can be life-threating if left untreated. We present the case of a 28-year-old man with SCD, who presented wit...Sickle-cell intrahepatic cholestasis (SCIC) is an uncommon complication of sickle-cell disease (SCD), which can be life-threating if left untreated. We present the case of a 28-year-old man with SCD, who presented with jaundice and abdominal pain, one month after hydroxyurea discontinuation. Laboratory investigation revealed solely increased serum bilirubin, mainly conjugated, and imaging studies revealed choledocholithiasis. The patient unterwent an endoscopic sphincterectomy, but his jaundice deteriorated. Sickle-cell intrahepatic cholestasis was suspected and he received aggressive exchange transfusion therapy in combination with hydroxyurea. The jaundice had no signs of improvement;in fact total bilirubin raised up to 1053.7 μmol/L (61.62 mg/dl). Subsequently, the patient was treated with single-pass albumin dialysis (SPAD), in order to remove the excess bilirubin and protect mainly the brain and liver cells from its toxic effects. His laboratory values started to improve after one-and-a-half months of treatment. For the next two months, exchange transfusions were continued and bilirubin gradually returned to baseline values. The successful response appeared after the combined use of exchange transfusion and SPAD, which is being reported for the first time.展开更多
文摘Sickle-cell intrahepatic cholestasis (SCIC) is an uncommon complication of sickle-cell disease (SCD), which can be life-threating if left untreated. We present the case of a 28-year-old man with SCD, who presented with jaundice and abdominal pain, one month after hydroxyurea discontinuation. Laboratory investigation revealed solely increased serum bilirubin, mainly conjugated, and imaging studies revealed choledocholithiasis. The patient unterwent an endoscopic sphincterectomy, but his jaundice deteriorated. Sickle-cell intrahepatic cholestasis was suspected and he received aggressive exchange transfusion therapy in combination with hydroxyurea. The jaundice had no signs of improvement;in fact total bilirubin raised up to 1053.7 μmol/L (61.62 mg/dl). Subsequently, the patient was treated with single-pass albumin dialysis (SPAD), in order to remove the excess bilirubin and protect mainly the brain and liver cells from its toxic effects. His laboratory values started to improve after one-and-a-half months of treatment. For the next two months, exchange transfusions were continued and bilirubin gradually returned to baseline values. The successful response appeared after the combined use of exchange transfusion and SPAD, which is being reported for the first time.
