TITLE:
Successful Management of Sickle Cell Intrahepatic Cholestasis with Combined Use of Exchange Transfusion and Single-Pass Albumin Dialysis: A Case Report
AUTHORS:
Vassilios Papadopoulos, Anna Karagianni, Vaia Papageorgiou, Maria Topalidou, Aristea-Lia Mpellou, Panagiotis Patinakis, Fotios Girtovitis, Despina Pantelidou, Anna Kioumi
KEYWORDS:
Sickle-Cell Intrahepatic Cholestasis; Exchange Transfusion; Single-Pass Albumin Dialysis (SPAD); Hydroxyurea
JOURNAL NAME:
Open Journal of Blood Diseases,
Vol.3 No.1,
March
29,
2013
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.