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

• Published in: Case reports in hematology Vol. 2014; no. 2014; pp. 1 - 3
• Main Authors: Vlachaki, Euthymia, Boura, Panagiota, Katsinelos, Panagiotis, Vetsiou, Evaggelia, Andreadis, Panagiotis, Neokleous, Nikolaos, Agapidou, Aleka
• Format: Journal Article
• Language: English
• Published: Cairo, Egypt Hindawi Puplishing Corporation 01.01.2014; Hindawi Publishing Corporation; Wiley
• Subjects: Case Report
• ISSN: 2090-6560; 2090-6579
• DOI: 10.1155/2014/213631

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 hyperbilirubinemia and acute hepatic failure with an often fatal course. Despite the fact that patients with Hb S/β+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/β+thal.