Vlachaki Efthymia, Andreadis Panagiotis, Neokleous Nikolaos, Agapidou Aleka, Vetsiou Evaggelia, Katsinelos Panagiotis, Boura Panagiota
Adults Thalassemia Unit, Second Medical Clinic, Aristotle University of Thessaloniki, Ippokration Hospital, Konstantinoupoleos 49, 54642 Thessaloniki, Greece.
Department of Gastroenterology, Second Medical Clinic, Aristotle University of Thessaloniki, Ippokration Hospital, Konstantinoupoleos 49, 54642 Thessaloniki, Greece.
Case Rep Hematol. 2014;2014:213631. doi: 10.1155/2014/213631. Epub 2014 Feb 9.
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.
镰状细胞/β（+）地中海贫血（Hb S/β（+）thal）被认为是镰状细胞病的一种变异形式。血管闭塞性疼痛危象的急性发作是镰状细胞疾病的特征，可能并发急性或慢性危及生命的器官功能障碍。慢性肝内胆汁淤积是镰状细胞病中一种罕见且严重的并发症，其特征为显著的高胆红素血症和急性肝衰竭，通常病程致命。尽管Hb S/β（+）thal患者通常病情较轻，但在此我们描述了一例成年Hb S/β（+）thal患者发生慢性肝内胆汁淤积且预后良好的有趣病例。
