Successful Management of Sickle Cell Intrahepatic Cholestasis with Combined Use of Exchange Transfusion and Single-Pass Albumin Dialysis: A Case Report  

作　　者：Vassilios Papadopoulos Anna Karagianni Vaia Papageorgiou Maria Topalidou Aristea-Lia Mpellou Panagiotis Patinakis Fotios Girtovitis Despina Pantelidou Anna Kioumi 

机构地区：[1]Blood Center and Blood Transfusion Service, AHEPA University Hospital, Thessaloniki, Greece [2]Hematology Department, Papageorgiou General Hospital, Thessaloniki, Greece [3]Nephrology Department, Papageorgiou General Hospital, Thessaloniki, Greece [4]Second Department of Internal Medicine, Medical School, Aristotle University, Thessaloniki, Greece [5]Thalassemia and Hemoglobinopathies Unit, First Department of Internal Medicine, Medical School, Aristotle University, Thessaloniki, Greece.

出　　处：《Open Journal of Blood Diseases》2013年第1期36-42,共7页血液病期刊（英文）

摘　　要：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.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.

关 键 词：Sickle-Cell INTRAHEPATIC CHOLESTASIS Exchange TRANSFUSION Single-Pass Albumin DIALYSIS (SPAD) HYDROXYUREA 

分 类 号：R5[医药卫生—内科学]