Successful treatment of acute sickle cell intrahepatic cholestasis with therapeutic plasma exchange
نویسندگان
چکیده
منابع مشابه
Chronic intrahepatic cholestasis in sickle cell disease requiring exchange transfusion.
A 15 year old boy with sickle cell disease developed intrahepatic cholestasis. A course of exchange transfusion successfully corrected the extreme hyperbilirubinaemia over one year. Upon stopping the exchange transfusion programme the hyperbilirubinaemia relapsed but transfusion was effective when reinstituted.
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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 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 کاملReversal of liver function without exchange transfusion in sickle cell intrahepatic cholestasis.
Sickle cell intrahepatic cholestasis (SCIC) is a rare but fatal complication of sickle cell disease. It is found mainly in homozygous sickle cell disease. To date, there are no standard diagnostic criteria or well-established therapeutic approaches to this condition. Herein, we report this case of a 48-year-old man with sickle cell anemia and a total bilirubin of 78.5 mg/dL without evidence of ...
متن کاملLiver transplantation in a patient with acute liver failure due to sickle cell intrahepatic cholestasis.
BACKGROUND Sickle cell intrahepatic cholestasis is a potentially catastrophic complication of sickle cell anemia Once acute liver failure develops, transplantation is the only option. We describe a patient with sickle cell intrahepatic cholestasis who underwent liver transplantation. METHODS Data were obtained from the chart. Serial hemoglobin S levels were monitored, and measures were taken ...
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ژورنال
عنوان ژورنال: eJHaem
سال: 2021
ISSN: 2688-6146,2688-6146
DOI: 10.1002/jha2.150