Open AccessIntrahepatic Cholestasis in a Sickle Cell Patient Unresponsive to Exchange Blood Transfusion
Author(s) -
Michelle Likhtshteyn,
Sadat Iqbal,
Samy I. McFarlane,
Savanna Thor
Publication year - 2020
Publication title -
american journal of medical case reports
Language(s) - English
Resource type - Journals
eISSN - 2374-216X
pISSN - 2374-2151
DOI - 10.12691/ajmcr-7-4-4
Subject(s) - medicine , exchange transfusion , cholestasis , complication , disease , coagulopathy , population , jaundice , sickle cell anemia , transfusion therapy , pediatrics , acute chest syndrome , priapism , hemoglobinopathy , intensive care medicine , surgery , blood transfusion , environmental health
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.