Ikeda Munehiro, Nakanishi Yuki, Niwa Ryotaro, Sakamoto Soichiro, Yoshioka Takuto, Kanda Naoki
Department of Gastroenterology and Hepatology, Kyoto University Graduate School of Medicine, 54 Kawahara-cho, Syogoin, Sakyo-ku, Kyoto, 606-8507, Japan.
Department of Hematology, Yodogawa Christian Hospital, 1-7-50 Shibashima, Higashiyodogawa-ku, Osaka, 533-0024, Japan.
Clin J Gastroenterol. 2025 Aug;18(4):700-705. doi: 10.1007/s12328-025-02142-x. Epub 2025 Jun 10.
Vanishing bile duct syndrome (VBDS) is a rare but life-threatening cause of cholestatic liver dysfunction characterized by progressive bile duct loss. While associated with various triggers, its occurrence after hematopoietic stem cell transplantation is uncommon and diagnostically challenging, especially when mimicking hepatitis B virus (HBV) reactivation.
We report a 71-year-old woman who developed VBDS following umbilical cord blood transplantation for myelodysplastic syndrome. Five years post-transplant, she presented with jaundice and elevated HBV-DNA levels, initially raising suspicion for HBV reactivation. Despite antiviral therapy, her liver function deteriorated. Imaging revealed widespread lymphadenopathy, and biopsies confirmed classical Hodgkin lymphoma-type post-transplant lymphoproliferative disorder (PTLD). Liver biopsy demonstrated marked bile duct loss, consistent with VBDS. Despite initiation of chemotherapy, hepatic dysfunction necessitated dose reduction, and the patient succumbed 93 days after treatment initiation.
This is the first reported case of VBDS associated with PTLD. The clinical course underscores the importance of distinguishing VBDS from HBV reactivation in transplant recipients presenting with cholestatic liver dysfunction. Liver biopsy remains essential for accurate diagnosis. Early recognition is crucial, as progressive liver injury may compromise treatment and worsen outcomes. This case expands the differential diagnosis of post-transplant liver failure and highlights the potential for PTLD to trigger VBDS.
消失性胆管综合征（VBDS）是一种罕见但危及生命的胆汁淤积性肝功能障碍病因，其特征为胆管进行性丧失。虽然与多种诱因相关，但其在造血干细胞移植后发生并不常见，且诊断具有挑战性，尤其是当它模仿乙型肝炎病毒（HBV）再激活时。
我们报告一名71岁女性，她在接受脐带血移植治疗骨髓增生异常综合征后发生了VBDS。移植后五年，她出现黄疸和HBV-DNA水平升高，最初怀疑是HBV再激活。尽管进行了抗病毒治疗，但其肝功能仍恶化。影像学检查显示广泛淋巴结病，活检证实为经典霍奇金淋巴瘤型移植后淋巴细胞增生性疾病（PTLD）。肝活检显示明显的胆管丧失，符合VBDS。尽管开始了化疗，但肝功能障碍需要减少剂量，患者在开始治疗93天后死亡。
这是首例报告的与PTLD相关的VBDS病例。该临床过程强调了在出现胆汁淤积性肝功能障碍的移植受者中区分VBDS与HBV再激活的重要性。肝活检对于准确诊断仍然至关重要。早期识别至关重要，因为进行性肝损伤可能会影响治疗并恶化预后。该病例扩展了移植后肝衰竭的鉴别诊断，并突出了PTLD引发VBDS的可能性。
