胆管消失综合征是一种罕见的获得性疾病,其特征是肝内胆管进行性丢失,导致导管减少和胆汁淤积。它可能与感染有关,缺血,药物不良反应,肿瘤,自身免疫性疾病,和同种异体移植排斥反应.预后是可变的,取决于胆管损伤的病因。我们报告了一例25岁的女性,患有胆汁淤积性肝炎并同时摄入肝毒性物质,比如Garcinia,野外马尾,还有酮洛芬.怀疑药物性肝损伤,药物被迅速撤回,熊去氧胆酸开始进行初步临床和实验室改进,病人出院了。一个月后,她的胆红素水平和小管酶有了新的增加,需要肝活检,显示肝内胆管的显著损失,与胆管消失综合征相容。通过在免疫组织化学上使用细胞角蛋白19证实了这一点。随后有几个链的淋巴结肿大,和相关的减肥。颈部淋巴结的组织学分析显示结节性硬化症亚型经典霍奇金淋巴瘤。在此设置中,胆管消失综合征与霍奇金淋巴瘤和药物性肝损伤重叠有关,导致进行性胆汁淤积,预后较差。病人对化疗的反应很差,需要使用本妥昔单抗vedotin进行生物治疗。对于医生来说,在可疑的胆管消失综合征患者中建立广泛的鉴别诊断至关重要。尤其是排除恶性肿瘤.
Vanishing bile duct syndrome is a rare acquired condition, characterized by progressive loss of intrahepatic bile ducts leading to ductopenia and cholestasis. It can be associated with infections, ischemia, drug adverse reactions, neoplasms, autoimmune disease, and allograft rejection. Prognosis is variable and depends on the etiology of bile duct injury. We report the
case of a 25-year-old female with cholestatic hepatitis and concomitant intakes of hepatotoxic substances, such as
garcinia, field horsetail, and ketoprofen. On suspicion of a drug-induced liver injury, the drugs were promptly withdrawn and ursodeoxycholic acid was started with initial clinical and laboratory improvement, and the patient was discharged from the hospital. One month later, she had a new increase in bilirubin levels and canalicular enzymes, requiring a liver biopsy that showed significant loss of intrahepatic bile ducts, which was compatible with vanishing bile duct syndrome. This was confirmed by using cytokeratin 19 on immunohistochemistry. There was subsequent lymph node enlargement in several chains, and relevant weight loss. Histological analysis of a cervical lymph node revealed nodular sclerosis-subtype classic Hodgkin lymphoma. In this setting, vanishing bile duct syndrome was related to Hodgkin lymphoma and a drug-induced liver injury overlap, leading to progressive cholestasis with a worse prognosis. The patient\'s response to chemotherapy was poor, requiring biological therapy with brentuximab vedotin. It is crucial for physicians to create a broad differential diagnosis in suspected vanishing bile duct syndrome patients, especially to rule out malignancies.