Successful Outcome of Chronic Intrahepatic Cholestasis in an Adult Patient with Sickle Cellβ+ Thalassemia

Abstract EN

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.