Meng Guangqiang, Feng Saran, Wang Yan
Department of Hematology, The First Affiliated Hospital of Shandong First Medical University and Shandong Provincial Qianfoshan Hospital, Jinan, China.
Front Pharmacol. 2025 Jun 11;16:1599873. doi: 10.3389/fphar.2025.1599873. eCollection 2025.
Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening hyper-inflammatory clinical syndrome characterized by a storm of inflammatory factors. In the treatment of HLH, it is critical to provide active and effective treatment immediately and hamper the inflammatory cytokine storm in a timely manner to improve patient symptoms. Currently, the first-line treatment for HLH is still based on etoposide and glucocorticoids. Unfortunately, the treatment effect of HLH remains insufficient, the mortality rate of patients remains high, and the prognosis remains poor. Therefore, effective salvage treatments are urgently needed to alleviate relapsed and refractory HLH. More than 10 years have passed since the liposomal doxorubicin combined with etoposide and methylprednisolone (DEP) regimen was first reported as a salvage treatment for HLH. In more than 10 years of clinical practice, many studies have reported the effectiveness and safety of the DEP regimen for the treatment of HLH. The DEP regimen not only demonstrated a good salvage treatment effect in relapsed refractory HLH but also revealed an optimal therapeutic effect in first-line induction treatment of HLH.
噬血细胞性淋巴组织细胞增生症（HLH）是一种危及生命的高炎症临床综合征，其特征为炎症因子风暴。在HLH的治疗中，立即提供积极有效的治疗并及时遏制炎症细胞因子风暴以改善患者症状至关重要。目前，HLH的一线治疗仍以依托泊苷和糖皮质激素为基础。不幸的是，HLH的治疗效果仍然不足，患者死亡率仍然很高，预后仍然很差。因此，迫切需要有效的挽救治疗来缓解复发难治性HLH。自脂质体阿霉素联合依托泊苷和甲泼尼龙（DEP）方案首次作为HLH的挽救治疗方法被报道以来，已经过去了10多年。在10多年的临床实践中，许多研究报告了DEP方案治疗HLH的有效性和安全性。DEP方案不仅在复发难治性HLH中显示出良好的挽救治疗效果，而且在HLH的一线诱导治疗中也显示出最佳治疗效果。
