The effectiveness of etoposide-containing chemotherapy regimens in the initial treatment of Hemophagocytic Syndrome in pregnancy.

IF 1.6 4区 医学 Q3 HEMATOLOGY
Hematology Pub Date : 2025-12-01 Epub Date: 2025-08-06 DOI:10.1080/16078454.2025.2538307
Jie Wang, Na Wei, Jingshi Wang, Lin Wu, Hongli Hu, Zhao Wang
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引用次数: 0

Abstract

Objective: To summarize the clinical characteristics, treatment outcomes, and prognosis of pregnant patients with hemophagocytic lymphohistiocytosis (HLH), and to evaluate the efficacy of chemotherapy regimens containing etoposide (VP16) in these patients and their impact on survival outcomes.

Methods: A retrospective analysis was performed on 41 pregnant HLH patients admitted to our hospital between April 2015 and March 2024, focusing on clinical features, treatment strategies, and prognostic factors.

Results: Among the 41 cases, 29 developed HLH during pregnancy and 12 postpartum. Etiologies included malignancy-related HLH (5 cases), Epstein-Barr virus (EBV)-related HLH (5 cases), rheumatologic disease-related HLH (2 cases), and pregnancy-related HLH (29 cases, defined as no comorbid HLH-related etiologies except pregnancy). In pregnancy-related HLH patients presenting during gestation, the 2-week overall response rate (ORR) was significantly higher in those receiving VP16-containing induction chemotherapy compared to glucocorticoids-only induction therapy (P = 0.011). Multivariate analysis identified the interval from symptom onset to VP16 initiation as an independent prognostic factor for pregnancy-related HLH (P = 0.025). Survival analysis revealed improved survival in patients who received VP16 within 7 weeks of onset versus those treated after 7 weeks (P = 0.044).

Discussion and conclusion: HLH in pregnancy progresses rapidly. Early administration of VP16-containing chemotherapy significantly improves ORR and survival outcomes, particularly in pregnancy-related HLH.

含依托泊苷化疗方案在妊娠期噬血细胞综合征初始治疗中的有效性。
目的:总结孕妇嗜血球性淋巴组织细胞病(HLH)的临床特点、治疗结果及预后,评价含依托泊苷(VP16)化疗方案对HLH患者的疗效及对生存结局的影响。方法:回顾性分析2015年4月~ 2024年3月我院收治的41例妊娠HLH患者的临床特点、治疗策略及预后因素。结果:41例患者中,29例发生于妊娠期,12例发生于产后。病因包括恶性肿瘤相关HLH(5例)、eb病毒相关HLH(5例)、风湿病相关HLH(2例)和妊娠相关HLH(29例,定义为除妊娠外无合并症的HLH相关病因)。在妊娠期间出现的妊娠相关HLH患者中,接受含vp16诱导化疗的患者2周总缓解率(ORR)明显高于仅接受糖皮质激素诱导治疗的患者(P = 0.011)。多因素分析发现,从症状出现到VP16启动的时间间隔是妊娠相关HLH的独立预后因素(P = 0.025)。生存分析显示,与7周后治疗的患者相比,在发病7周内接受VP16治疗的患者生存率更高(P = 0.044)。讨论与结论:妊娠期HLH进展迅速。早期给予含有vp16的化疗可显著改善ORR和生存结果,特别是在妊娠相关的HLH中。
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来源期刊
Hematology
Hematology 医学-血液学
CiteScore
2.60
自引率
5.30%
发文量
140
审稿时长
3 months
期刊介绍: Hematology is an international journal publishing original and review articles in the field of general hematology, including oncology, pathology, biology, clinical research and epidemiology. Of the fixed sections, annotations are accepted on any general or scientific field: technical annotations covering current laboratory practice in general hematology, blood transfusion and clinical trials, and current clinical practice reviews the consensus driven areas of care and management.
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