Hanyue Zhang,* Pan Jiang,* Sibin Mei, Ne Lin, Yanfei Fang, Qian Cao

Department of Gastroenterology, Sir Run Run Shaw Hospital, College of Medicine Zhejiang University, Hangzhou, Zhejiang Province, People’s Republic of China

*These authors contributed equally to this work

Correspondence: Yanfei Fang, Email fyanfei@163.com Qian Cao, Email caoq@zju.edu.cn

Background: Drug reaction with eosinophilia and systemic symptoms (DRESS) syndrome is a severe systemic disorder characterized by fever, rash, and multi-organ involvement, often complicated by drug-induced liver injury. Hemophagocytic lymphohistiocytosis (HLH) and vanishing bile duct syndrome (VBDS) are rare but life-threatening complications that can be triggered by antiepileptic drugs such as carbamazepine. Given the high mortality associated with these conditions, early recognition and timely intervention are crucial for improving patient outcomes.
Case: We report a unique case of an elderly woman who developed DRESS syndrome after using carbamazepine, complicated by both HLH and VBDS. The patient exhibited typical DRESS symptoms, including fever, rash, and eosinophilia, alongside signs of HLH such as hemocytopenia and elevated ferritin levels; along with persistent significant hyperbilirubinemia and coagulation abnormalities.
Results: After six months, liver function showed substantial improvement, with no signs of HLH recurrence. Additionally, our review of HLH cases induced by antiepileptic drugs highlights that the absence of eosinophilia, hemocytopenia, and elevated ferritin levels is key for early HLH identification.
Conclusion: Our findings highlight key diagnostic indicators for early HLH recognition in antiepileptic drug-induced DRESS, especially the absence of eosinophilia. This case represents the first successful management of DRESS syndrome complicated by HLH and VBDS without liver transplantation in the past decade, emphasizing the critical role of early identification and prompt, targeted treatment strategies in optimizing patient outcomes.

Keywords: drug reaction with eosinophilia and systemic symptoms, vanishing bile duct syndrome, hemophagocytic lymphohistiocytosis, immune overactivation