Successful management of infection and macrophage activation syndrome patient using low-dose etoposide: A case report.

IF 1.4 Q3 RADIOLOGY, NUCLEAR MEDICINE & MEDICAL IMAGING
Shu-Pei Gao, Xiao-Fang Luo, Mohammadreza Kosari, Wen-Juan Li, Liu Yang, Wei Tu, Ji-Xin Zhong
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引用次数: 0

Abstract

Background: Macrophage activation syndrome (MAS), a sub-type of hemophagocytic lymphohistiocytosis (HLH) secondary to autoimmune rheumatic diseases, is a critical and potentially fatal condition characterized by an excessive inflammatory response. Despite the established efficacy of the HLH-2004 guideline in diagnosing and treating HLH over the years, ongoing discussion persists regarding its application, especially for HLH secondary to complicated conditions, such as autoimmune rheumatic diseases combined with severe infection. Etoposide (VP-16), a topoisomerase II inhibitor that effectively induces DNA damage and subsequent apoptosis in hyperactivated immune cells, has been widely used for the treatment of HLH. However, its suppressive effect on immune system may also cause potential exacerbation of infection in autoimmune rheumatic disease-induced HLH patients complicated with severe infection. Therefore, the use of VP-16 in such cases was inconclusive.

Case summary: In this case study, we propose a potentially effective strategy for managing a patient diagnosed with secondary HLH complicated with systemic lupus erythematosus (SLE) and chronic coronavirus disease 2019 infection. Our approach involves early administration of low-dose VP-16 (100 mg twice a week, 300 mg in total), combined with methylprednisolone, cyclophosphamide, and cyclosporine A. The administration of etoposide effectively led to improvements in various indices of HLH.

Conclusion: Low dose etoposide proves to be an effective approach in alleviating HLH while mitigating the risk of infection.

使用小剂量依托泊苷成功治疗感染和巨噬细胞活化综合征患者:病例报告
背景:巨噬细胞活化综合征(MAS)是继发于自身免疫性风湿病的嗜血细胞淋巴组织细胞增多症(HLH)的一种亚型,是一种以过度炎症反应为特征的危重且可能致命的疾病。尽管多年来 HLH-2004 指南在诊断和治疗 HLH 方面已取得了公认的疗效,但关于其应用的讨论仍在继续,尤其是对于继发于自身免疫性风湿病合并严重感染等复杂情况的 HLH。依托泊苷(VP-16)是一种拓扑异构酶 II 抑制剂,能有效诱导 DNA 损伤,进而导致过度激活的免疫细胞凋亡,已被广泛用于治疗 HLH。然而,VP-16 对免疫系统的抑制作用也可能导致自身免疫性风湿病诱发的 HLH 患者并发严重感染的潜在感染加重。因此,在此类病例中使用 VP-16 尚无定论。病例摘要:在本病例研究中,我们提出了一种可能有效的策略,用于治疗被诊断为继发性 HLH 并发系统性红斑狼疮(SLE)和慢性冠状病毒病 2019 感染的患者。我们的方法包括早期给予小剂量VP-16(100毫克,每周两次,共300毫克),同时联合甲基强的松龙、环磷酰胺和环孢素A:小剂量依托泊苷被证明是缓解 HLH 的有效方法,同时还能降低感染风险。
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来源期刊
World journal of radiology
World journal of radiology RADIOLOGY, NUCLEAR MEDICINE & MEDICAL IMAGING-
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8.00%
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