Intrahepatic Cholestasis in Sickle Cell Disease: A Case Report
نویسندگان
چکیده
Intrahepatic cholestasis (SCIC) is an uncommon but potentially fatal complication of sickle cell disease (SCD), with a high death rate, observed mainly in patients with homozygous sickle cell anemia. Herein, we describe a case of severe SCIC treated successfully with aggressive manual exchange transfusion (ET). The patient was admitted with enlarged liver and signs of hepatic failure, such as hyperbilirubinemia and coagulopathy. There was no evidence of viral hepatitis or biliary obstruction. We performed several sessions of ET in order to reduce the percentage of HbS to levels inferior to 30%, which was successfully accomplished. The patient had a complete recovery of hepatic function. This case has shown that ET is an effective treatment of SCIC and should be introduced early on the onset of this severe complication.
منابع مشابه
Successful Orthotopic Liver Transplantation in an Adult Patient with Sickle Cell Disease and Review of the Literature
Sickle cell disease can lead to hepatic complications ranging from acute hepatic crises to chronic liver disease including intrahepatic cholestasis, and iron overload. Although uncommon, intrahepatic cholestasis may be severe and medical treatment of this complication is often ineffective. We report a case of a 37 year-old male patient with sickle cell anemia, who developed liver failure and un...
متن کاملSickle cell intrahepatic cholestasis unresponsive to exchange blood transfusion: a case report
Sickle cell intrahepatic cholestasis (SCIC) is an uncommon but severe complication in sickle cell disease (SCD) patients homozygous for hemoglobin (Hb) S or with Hb S/ thalassemia.1–3 It is clinically characterized by marked conjugated hyperbilirubinemia, right upper quadrant pain, enlarged liver and moderately elevated hepatic enzymes. In more severe cases, coagulopathy and renal dysfunction m...
متن کاملBENIGN EXTREME HYPERBILIRUBINEMIA IN A 9 YEAR-OLD GIRL WITH SICKLE-THALASSEMIA AND THE PROBABLE ROLE OF HBF IN PREDICTING THE OUTCOME
Hepatic dysfunction is a frequent manifestation in patients with sickle cell anemia. It is usually a multifactorial process. A rare benign form of extreme hyperbilirubinemia, presumably due to intrahepatic sickling, may be the cause. We report a 9 year old girl with sickle-thalassemia hemoglo binopathy, presenting with profound jaundice. Sickle cell disease is often mild in the Iranian popu...
متن کاملExchange transfusion for intrahepatic cholestasis due to sickle beta thalassaemia.
Intrahepatic cholestasis is a rare but potentially fatal complication of sickle cell disease [1]. Sickle cell intrahepatic cholestasis (SCIC) is characterised by right upper quadrant pain, hepatomegaly and progressive hyperbilirubinaemia. To date 15 adult patients have been reported in the literature and only six survived [2]. We present a case of haemoglobin S/Beta thalassaemia with intrahepat...
متن کاملSuccessful Outcome of Chronic Intrahepatic Cholestasis in an Adult Patient with Sickle Cell/β+ Thalassemia
Sickle cell/β (+) thalassemia (Hb S/β (+)thal) is considered as a variant form of sickle cell disease. Acute episodes of vasoocclusive pain crisis are characteristic for sickle cell disorders and may be complicated by an acute or chronic life-threatening organ dysfunction. Chronic intrahepatic cholestasis is a rare and severe complication in sickle cell disease, characterized by marked hyperbil...
متن کاملذخیره در منابع من
با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید
عنوان ژورنال:
دوره 2011 شماره
صفحات -
تاریخ انتشار 2011