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地拉罗司致肝损伤及范可尼综合征 1 例:β 地中海贫血男性患者

Deferasirox-induced liver injury and Fanconi syndrome in a beta-thalassemia major male.

机构信息

Gastroenterology and Hepatology Department, Austin Health, Heidelberg, Victoria, Australia

Gastroenterology and Hepatology Department, Northern Health, Epping, Victoria, Australia.

出版信息

BMJ Case Rep. 2020 Jul 9;13(7):e234542. doi: 10.1136/bcr-2020-234542.

Abstract

A 33-year-old male presenting with subacute abdominal pain was found to have hyperbilirubinaemia, hypokalaemia and hyponatraemia. This was in the setting of transitioning between deferasirox iron chelator formulations, from dispersible tablets to film-coated tablets for ongoing treatment of chronic iron overload secondary to transfusion requirement for beta-thalassemia major. A liver biopsy demonstrated acute cholestasis with patchy confluent hepatocellular necrosis and mild to moderate microvesicular steatosis. Based on the histological, biochemical and clinical findings, the diagnosis of hepatotoxicity and Fanconi-like syndrome was made. The patient improved clinically and biochemically with cessation of the deferasirox film-coated tablets and supportive management. To our knowledge, this is the first case report of hepatotoxicity and Fanconi-like syndrome occurring due to deferasirox film-coated tablets with previous tolerance of dispersible deferasirox tablets. It is important to raise clinical awareness of this potentially severe complication.

摘要

一位 33 岁男性因亚急性腹痛就诊,发现高胆红素血症、低钾血症和低钠血症。这是在他从分散片转换为薄膜包衣片的过程中发生的,目的是继续治疗因重型β地中海贫血需要输血引起的慢性铁过载。肝活检显示急性胆汁淤积,伴有斑片状融合性肝细胞坏死和轻度至中度微泡性脂肪变性。根据组织学、生化和临床发现,诊断为肝毒性和范可尼样综合征。患者在停用地拉罗司薄膜包衣片并接受支持性治疗后临床和生化情况均有所改善。据我们所知,这是首例因先前耐受分散片而发生的地拉罗司薄膜包衣片导致的肝毒性和范可尼样综合征的病例报告。提高对此种潜在严重并发症的临床认识非常重要。

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