Drug associated vanishing bile duct syndrome combined with hemophagocytic lymphohistiocytosis.
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.
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.
Hui
H
Li
Xin
X
Li
Xiao-Xing
XX
Liao
Hong
H
Zhan
Yan
Y
Xiong
Chun-Lin
CL
Hu
Hong-Yan
HY
Wei
Xiao-Li
XL
Jing