Drug associated vanishing bile duct syndrome combined with hemophagocytic lymphohistiocytosis

作     者：Hui Li Xin Li Xiao-Xing Liao Hong Zhan et al. Yan Xiong Chun-Lin Hu Hong-Yan Wei Xiao-Li Jing 

作者机构：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. 

出 版 物：《World Journal of Gastrointestinal Endoscopy》 (世界胃肠内镜杂志（英文版）（电子版）)

年 卷 期：2012年第4卷第8期

页      面：376-378页

学科分类：1002[医学-临床医学] 100201[医学-内科学(含：心血管病、血液病、呼吸系病、消化系病、内分泌与代谢病、肾病、风湿病、传染病)] 10[医学] 

主　　题：Vanishing bile duct syndrome Hemophagocytic lymphohistiocytosis Epstein-Barr virus Amoxicillin-clavulanate 

摘      要：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 ?rst description of vanishing bile duct syndrome combined with hemophagocytic lymphohistiocytosis, with underlying causes including infection, drug-induced factors and untreated autoimmune disorder.