Li Hui, Li Xin, Liao Xiao-Xing, Zhan Hong, Xiong Yan, Hu Chun-Lin, Wei Hong-Yan, Jing Xiao-Li
Hui Li, Xin Li, Xiao-Xing Liao, Hong Zhan, Yan Xiong, Chun-Lin Hu, Hong-Yan Wei, Xiao-Li Jing, Department of Emergency, The First Affiliated Hospital of Sun Yat-Sen University, Guangzhou 510080, Guangdong Province, China.
World J Gastrointest Endosc. 2012 Aug 16;4(8):376-8. doi: 10.4253/wjge.v4.i8.376.
A 28-year-old woman with untreated autoimmune disorder, demonstrated skin rash and fever after taking Amoxicillin-clavulanate and developed progressive jaundice. A bone marrow aspiration indicated an increased number of macrophages with hemophagocytosis and liver biopsy showed pure centrilobular cholestasis with necrosis and some absence of portal bile ducts. Furthermore, a serological test for Epstein-Barr virus was positive. Under treatment by liver dialysis and administration of steroids led to rapidly defervescence and clinical improvement. However, liver enzymes were still markedly elevated with persistent anemia, even after immunosuppressive treatment. The patient is currently waiting for liver transplantation. This is the first description of vanishing bile duct syndrome combined with hemophagocytic lymphohistiocytosis, with underlying causes including infection, drug-induced factors and untreated autoimmune disorder.
一名28岁未治疗的自身免疫性疾病女性，服用阿莫西林克拉维酸后出现皮疹和发热，并逐渐出现黄疸。骨髓穿刺显示巨噬细胞数量增加伴噬血细胞现象，肝脏活检显示单纯性小叶中心性胆汁淤积伴坏死，部分门静脉胆管缺如。此外，爱泼斯坦-巴尔病毒血清学检测呈阳性。经肝透析和类固醇治疗后迅速退热，临床症状改善。然而，即使经过免疫抑制治疗，肝酶仍显著升高，贫血持续存在。该患者目前正在等待肝移植。这是首次描述消失胆管综合征合并噬血细胞性淋巴组织细胞增生症，潜在病因包括感染、药物诱导因素和未治疗的自身免疫性疾病。
