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Case Reports in Hematology
Volume 2014, Article ID 213631, 3 pages
http://dx.doi.org/10.1155/2014/213631
Case Report

Successful Outcome of Chronic Intrahepatic Cholestasis in an Adult Patient with Sickle Cell/β+ Thalassemia

1Adults Thalassemia Unit, Second Medical Clinic, Aristotle University of Thessaloniki, Ippokration Hospital, Konstantinoupoleos 49, 54642 Thessaloniki, Greece
2Department of Gastroenterology, Second Medical Clinic, Aristotle University of Thessaloniki, Ippokration Hospital, Konstantinoupoleos 49, 54642 Thessaloniki, Greece

Received 1 December 2013; Accepted 30 December 2013; Published 9 February 2014

Academic Editors: U. Dasgupta, K. Konstantopoulos, F. Kutlar, and M.-C. Kyrtsonis

Copyright © 2014 Efthymia Vlachaki et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Sickle cell/ thalassemia (Hb S/ 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 hyperbilirubinemia and acute hepatic failure with an often fatal course. Despite the fact that patients with Hb S/ thal usually have a mild type of disease, herein we describe an interesting case of chronic intrahepatic cholestasis with successful outcome in an adult patient with Hb S/ thal.