Sabu Siona, Chakrabarty Gayatri, Shahid Fatima
Internal Medicine, Surrey and Sussex Healthcare NHS Trust, Redhill, GBR.
Gastroenterology and Hepatology, Surrey and Sussex Healthcare NHS Trust, Redhill, GBR.
Cureus. 2024 Sep 5;16(9):e68724. doi: 10.7759/cureus.68724. eCollection 2024 Sep.
Vanishing bile duct syndrome (VBDS) is a clinicopathological term coined to describe an acquired liver disease characterised by progressive destruction and disappearance of intrahepatic biliary ducts. We report the case of a 69-year-old female who presented with painless jaundice, fatigue, and weight loss. Initial blood workup revealed hyperbilirubinemia, transaminitis, elevated alkaline phosphatase, and a raised international normalised ratio. Para-aortic lymphadenopathy on computed tomography of the abdomen was biopsied to confirm the diagnosis of Hodgkin's lymphoma. Vanishing bile duct syndrome is a paraneoplastic phenomenon of Hodgkin's lymphoma, a recognised cause of cholestatic jaundice, and our patient's liver biopsy was diagnostic of the same. Despite treatment with steroids, ursodeoxycholic acid, and chemotherapy, our patient passed away. This case report aims to highlight VBDS as a differential diagnosis for painless jaundice in the context of cholestatic liver dysfunction. We believe reporting such cases irrespective of their outcome will help raise awareness of VBDS among clinicians, thus bettering the rate at which it is diagnosed and treated, thereby improving patient outcomes.
消失胆管综合征（VBDS）是一个临床病理学术语，用于描述一种获得性肝病，其特征为肝内胆管进行性破坏和消失。我们报告了一例69岁女性病例，该患者出现无痛性黄疸、疲劳和体重减轻。初步血液检查显示高胆红素血症、转氨酶升高、碱性磷酸酶升高以及国际标准化比值升高。对腹部计算机断层扫描发现的主动脉旁淋巴结病进行活检，以确诊霍奇金淋巴瘤。消失胆管综合征是霍奇金淋巴瘤的一种副肿瘤现象，是胆汁淤积性黄疸的一个公认病因，我们患者的肝脏活检确诊了这一病症。尽管接受了类固醇、熊去氧胆酸和化疗治疗，我们的患者仍去世了。本病例报告旨在强调在胆汁淤积性肝功能障碍背景下，消失胆管综合征是无痛性黄疸的鉴别诊断之一。我们认为，无论结果如何报告此类病例都将有助于提高临床医生对消失胆管综合征的认识，从而提高其诊断和治疗率，进而改善患者预后。
