American Journal of Medical Case Reports
ISSN (Print): 2374-2151 ISSN (Online): 2374-216X Website: http://www.sciepub.com/journal/ajmcr Editor-in-chief: Apply for this position
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American Journal of Medical Case Reports. 2019, 7(4), 67-70
DOI: 10.12691/ajmcr-7-4-4
Open AccessCase Report

Intrahepatic Cholestasis in a Sickle Cell Patient Unresponsive to Exchange Blood Transfusion

Michelle Likhtshteyn1, Sadat Iqbal1, Samy I. McFarlane1, and Savanna Thor2

1SUNY Downstate Medical Center, Brooklyn, NY, USA

2Department of Gastroenterology and Hepatology, SUNY Downstate Medical Center, Brooklyn, NY, USA

Pub. Date: April 25, 2019

Cite this paper:
Michelle Likhtshteyn, Sadat Iqbal, Samy I. McFarlane and Savanna Thor. Intrahepatic Cholestasis in a Sickle Cell Patient Unresponsive to Exchange Blood Transfusion. American Journal of Medical Case Reports. 2019; 7(4):67-70. doi: 10.12691/ajmcr-7-4-4

Abstract

With the advent of hydroxyurea, the sickle cell population has been enjoying a prolonged life span as compared to the pre-hydroxyurea era. Traditionally, acute complications of sickle cell disease includes acute chest syndrome, MI and stroke. In this report we present a case of an elderly man with sickle cell disease who presented with intrahepatic cholestasis (SCIC); a rather rare and fatal complication of sickle cell hemoglobinopathy. The patient presented with jaundice and elevated bilirubin up to 53, his hospital course was complicated by coagulopathy and encephalopathy, and expired on day 43 of presentation after failing multiple therapeutic interventions including exchange transfusion. In this report, we will provide literature review and discuss the underlying pathophysiologic mechanisms of intrahepatic cholestasis in the sickle cell population highlighting the need for immediate recognition and institution of therapy for this fatal complication of sickle cell disease, particularly in elderly populations with low metabolic reserve.

Keywords:
liver transplantation exchange transfusion sickle cell cholestasis intrahepatic

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