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 

出　　处：《World Journal of Gastrointestinal Endoscopy》2012年第8期376-378,共3页世界胃肠内镜杂志（英文版）（电子版）

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. 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.

关 键 词：Vanishing bile duct syndrome Hemophagocytic lymphohistiocytosis Epstein-Barr virus Amoxicillin-clavulanate 

分 类 号：R575.7[医药卫生—消化系统]