Gaudel Pramod, Brown Paige, Byrd Ken
Department of Hematology and Oncology, University of Kansas Medical Center, Kansas City, USA.
Internal Medicine, University of Kansas School of Medicine, Kansas City, USA.
Cureus. 2022 Jul 14;14(7):e26842. doi: 10.7759/cureus.26842. eCollection 2022 Jul.
Vanishing bile duct syndrome (VBDS) is an acquired condition characterized by the destruction and loss of intrahepatic bile ducts resulting in cholestasis. VBDS has been described in various conditions including neoplastic and immunologic disorders, infections, hepatic ischemia, and drug toxicity. The diagnosis is confirmed by liver biopsy revealing the loss of interlobular bile ducts in greater than 50% of portal tracts. Prognosis is variable and often unpredictable but appears to be influenced by the etiology of bile duct destruction and overall patient health. VBDS has been described as a rare paraneoplastic process in patients with Hodgkin lymphoma. This case describes a 26-year-old female who presented with a neck mass, jaundice, and pruritus. Initial workup revealed direct hyperbilirubinemia, transaminitis, elevated alkaline phosphatase, and elevated international normalized ratio. She went on to receive a diagnosis of stage II classical Hodgkin lymphoma, nodular sclerosing subtype, and biopsy-proven VBDS. Over the course of chemotherapy, complete metabolic resolution of Hodgkin lymphoma and complete normalization of bilirubin were achieved. She was given gemcitabine and cyclophosphamide as a liver sparing regimen initially with some improvement in liver function tests and a reduction in lymph node volumes. She received six cycles of adriamycin/bleomycin/vinblastine/dacarbazine (ABVD) with complete remission attained after four cycles by positron emission tomography/computed tomography criteria. This report illustrates asafe chemotherapy regimen in the presence of marked liver dysfunction. Workup for VBDS including liver biopsy should be pursued in Hodgkin lymphoma patients with evidence of cholestasis in the absence of extrahepatic bile duct damage or other known etiology of liver injury.
消失胆管综合征（VBDS）是一种后天性疾病，其特征是肝内胆管破坏和丧失，导致胆汁淤积。VBDS已在多种情况下被描述，包括肿瘤和免疫性疾病、感染、肝脏缺血和药物毒性。通过肝活检证实诊断，显示超过50%的门管区小叶间胆管丧失。预后各不相同，通常不可预测，但似乎受胆管破坏的病因和患者整体健康状况影响。VBDS在霍奇金淋巴瘤患者中被描述为一种罕见的副肿瘤过程。本病例描述了一名26岁女性，她出现颈部肿块、黄疸和瘙痒。初步检查发现直接胆红素血症、转氨酶升高、碱性磷酸酶升高和国际标准化比值升高。她随后被诊断为II期经典型霍奇金淋巴瘤，结节硬化亚型，活检证实为VBDS。在化疗过程中，霍奇金淋巴瘤实现了完全代谢缓解，胆红素完全恢复正常。最初给予她吉西他滨和环磷酰胺作为肝脏保护方案，肝功能检查有一些改善，淋巴结体积减小。她接受了六个周期的阿霉素/博来霉素/长春花碱/达卡巴嗪（ABVD）化疗，根据正电子发射断层扫描/计算机断层扫描标准，四个周期后达到完全缓解。本报告说明了在存在明显肝功能障碍的情况下一种安全的化疗方案。对于在没有肝外胆管损伤或其他已知肝损伤病因的情况下有胆汁淤积证据的霍奇金淋巴瘤患者，应进行包括肝活检在内的VBDS检查。
