Abstract
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.
Highlights
Sickle-cell intrahepatic cholestasis (SCIC) is a difficult clinical problem
The patient was submitted to aggressive exchange transfusion of packed red blood cells in order to achieve hemoglobin S (HbS) fraction less than 30% and avoid further sickling
Sickle cell intrahepatic cholestasis is an uncommon complication with a possible fatal outcome that may be observed in patients with sickle-cell disease (SCD) [4]
Summary
Sickle-cell intrahepatic cholestasis (SCIC) is a difficult clinical problem. It is a quite rare entity, which could become a fatal complication of sickle cell disease (SCD). Higher death rate may be observed in older patients, with a prior hepatic disease and with homozygous sickle cell anemia [1]. The only effective treatment in most cases is the early onset of exchange transfusion aiming at lowering the hemoglobin S (HbS) fraction, in an attempt to minimize the intrahepatic sickling. In SCD patients, gall-stones is a common medical condition, cholestasis can be attributed to cholelithiasis and treated with endoscopic retrograde. SCIC, treated with multiple manual exchange transfusions and supportive care, who achieved complete recovery of hepatic function after nearly two months of treatment
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