Li H, Li X, Liao XX, Zhan H, Xiong Y, Hu CL, Wei HY, Jing XL. Drug associated vanishing bile duct syndrome combined with hemophagocytic lymphohistiocytosis. World J Gastrointest Endosc 2012; 4(8): 376-378 [PMID: 22912913 DOI: 10.4253/wjge.v4.i8.376]
Corresponding Author of This Article
Xiao-Li Jing, Professor, Department of Emergency, The First Affiliated Hospital of Sun Yat-Sen University, 58 Zhongshan 2nd Road, Guangzhou 510080, Guangdong Province, China. jing-xl@163.com
Article-Type of This Article
Case Report
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World J Gastrointest Endosc. Aug 16, 2012; 4(8): 376-378 Published online Aug 16, 2012. doi: 10.4253/wjge.v4.i8.376
Drug associated vanishing bile duct syndrome combined with hemophagocytic lymphohistiocytosis
Hui Li, Xin Li, Xiao-Xing Liao, Hong Zhan, Yan Xiong, Chun-Lin Hu, Hong-Yan Wei, Xiao-Li Jing
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
Author contributions: Li H, Hu CL, Wei HY and Jing XL collected the data from the case; Li H and Jing XL wrote the manuscript; Liao XX and Zhan H supervised the project; Li X and Xiong Y edited the manuscript; all authors discussed the results and implications and commented on the manuscript at all stages.
Correspondence to: Xiao-Li Jing, Professor, Department of Emergency, The First Affiliated Hospital of Sun Yat-Sen University, 58 Zhongshan 2nd Road, Guangzhou 510080, Guangdong Province, China. jing-xl@163.com
Received: May 18, 2011 Revised: March 2, 2012 Accepted: August 8, 2012 Published online: August 16, 2012
Abstract
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.
