Zhang Hanyue, Jiang Pan, Mei Sibin, Lin Ne, Fang Yanfei, Cao Qian
Department of Gastroenterology, Sir Run Run Shaw Hospital, College of Medicine Zhejiang University, Hangzhou, Zhejiang Province, People's Republic of China.
J Asthma Allergy. 2025 Apr 29;18:655-664. doi: 10.2147/JAA.S505666. eCollection 2025.
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.
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.
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.
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.
药物超敏反应伴嗜酸性粒细胞增多和全身症状（DRESS）综合征是一种严重的全身性疾病，其特征为发热、皮疹和多器官受累，常并发药物性肝损伤。噬血细胞性淋巴组织细胞增生症（HLH）和消失性胆管综合征（VBDS）是罕见但危及生命的并发症，可由卡马西平等抗癫痫药物引发。鉴于这些病症的高死亡率，早期识别和及时干预对于改善患者预后至关重要。
我们报告了一例独特的老年女性病例，该患者在使用卡马西平后发生DRESS综合征，并并发HLH和VBDS。患者表现出典型的DRESS症状，包括发热、皮疹和嗜酸性粒细胞增多，同时伴有HLH的体征，如血细胞减少和铁蛋白水平升高；以及持续性显著高胆红素血症和凝血异常。
六个月后，肝功能显示出显著改善，无HLH复发迹象。此外，我们对抗癫痫药物诱发的HLH病例的回顾强调，不存在嗜酸性粒细胞增多、血细胞减少和铁蛋白水平升高是早期识别HLH的关键。
我们的研究结果突出了抗癫痫药物诱发的DRESS中早期识别HLH的关键诊断指标，尤其是不存在嗜酸性粒细胞增多。该病例代表了过去十年中首例成功治疗并发HLH和VBDS的DRESS综合征且未进行肝移植的病例，强调了早期识别以及及时、有针对性的治疗策略在优化患者预后方面的关键作用。
