Intrahepatic Cholestasis in Sickle Cell Disease: a Case Report

Overview

Journal Anemia

Publisher Wiley

Date 2011 Apr 15

PMID 21490769

Citations 14

Authors

Denise Menezes Brunetta

Ana Cristina Silva-Pinto

Maria do Carmo Favarin de Macedo

Sarah Cristina Bassi

Joao Victor Piccolo Feliciano

Fernanda Borges Ribeiro

Benedito de Pina Almeida Prado Jr

Gil Cunha de Santis

Ivan de Lucena Angulo

Dimas Tadeu Covas

Affiliations

Soon will be listed here.

Abstract

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.

Citing Articles

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Acute Liver Failure With Liver Enzymes >5,000 in Sickle Cell Disease.

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Fulminant dengue hepatitis in sickle cell disease: Recovery against the odds.

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