重症监护室治疗嗜血球性淋巴组织细胞增多症:采用侵入性器官支持为时已晚?

IF 2.7 3区 医学 Q2 CRITICAL CARE MEDICINE
SHOCK Pub Date : 2025-06-23 DOI:10.1097/SHK.0000000000002635
Julien Carvelli, Amandine Bichon, Raphael Cauchois, Anderson Loundou, Fouad Bouzana, Audrey Le Saux, Marc Gainnier, Jérémy Bourenne, Frédéric Vély, Hubert Lepidi, Romain Appay, Gilles Kaplanski
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引用次数: 0

摘要

噬血细胞性淋巴组织细胞增多症(HLH)是一种严重的高炎症综合征,可导致多器官功能衰竭导致早期死亡。在继发性HLH中,病因学治疗是一种紧急情况,但有时在获得补充结果(微生物学、组织学)之前是不可能的。用抗炎药物(抗jak、抗细胞因子或依托泊苷)作为“病因治疗的桥梁”是必不可少的。进行这项研究是为了确定开始这种治疗的正确时间。方法:我们对马赛大学医院重症监护病房(ICU)超选择性(HS评分大于215,HLH概率为95%)继发性HLH的成人患者进行回顾性研究。结果:在10年的时间里,我们纳入了23例患者(7名女性,16名男性,49岁(37-59))。HS评分中位数为272(250-294)。14例为感染相关HLH, 4例为淋巴瘤相关HLH, 2例为成人起病斯蒂尔氏病(AOSD)。7例患者死亡(30.4%),均接受有创器官支持(IOS =有创机械通气、去甲肾上腺素和/或肾脏替代治疗)。当将14例IOS患者与其他9例患者进行比较时,我们发现年龄,病因,临床生物学特征和hlh定向治疗方面没有差异。无IOS组的死亡率为0,而IOS组的死亡率为7 (50%)(p = 0.02)。结论:在继发性HLH中,对症抗炎治疗(等待病因治疗)是在器官衰竭发生前阻止巨噬细胞和/或淋巴细胞活化的绝对紧急措施。铁蛋白的急剧增加和血小板的减少可能是生物学上的警告信号。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Treatment of Hemophagocytic Lymphohistiocytosis in the Intensive Care Unit: Too late after resorting to invasive organ supports?

Introduction: Hemophagocytic lymphohistiocytosis (HLH) is a severe hyperinflammatory syndrome that can lead to early death from multiple organ failure. In secondary HLH, etiological treatment is an emergency, but sometimes impossible until complementary results (microbiology, histology) are available. A "bridge to etiological treatment" with anti-inflammatory drugs (anti-JAK, anti-cytokines or etoposide) is then essential. This study was conducted to determine the right time to start such treatment.

Methods: We conducted a retrospective study at the University Hospital of Marseille on ultra-selected (HS score above 215, probability of HLH = 95%) adult patients treated for secondary HLH in the intensive care unit (ICU).

Results: Over a 10-year period, we included 23 patients (7 women, 16 men, 49 years (37-59)). The median HS score was 272 (250-294). 14 patients had infection-related HLH, 4 patients had lymphoma-related HLH and 2 patients had adult-onset Still's disease (AOSD). 7 patients died (30.4%), all of whom received invasive organ support (IOS = invasive mechanical ventilation, noradrenaline and/or renal replacement therapy). When comparing the 14 patients with IOS with the 9 others, we found no differences in terms of age, etiology, clinical-biological characteristics and HLH-directed therapy. The mortality rate was 0 in the group without IOS compared to 7 deaths (50%) in the IOS group (p = 0.02).

Conclusions: In secondary HLH, symptomatic anti-inflammatory treatment (awaiting etiological treatment) is an absolute emergency to stop macrophage and/or lymphocyte activation before organ failure occurs. A drastic increase in ferritin and a decrease in platelets could be biological warning signs.

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来源期刊
SHOCK
SHOCK 医学-外科
CiteScore
6.20
自引率
3.20%
发文量
199
审稿时长
1 months
期刊介绍: SHOCK®: Injury, Inflammation, and Sepsis: Laboratory and Clinical Approaches includes studies of novel therapeutic approaches, such as immunomodulation, gene therapy, nutrition, and others. The mission of the Journal is to foster and promote multidisciplinary studies, both experimental and clinical in nature, that critically examine the etiology, mechanisms and novel therapeutics of shock-related pathophysiological conditions. Its purpose is to excel as a vehicle for timely publication in the areas of basic and clinical studies of shock, trauma, sepsis, inflammation, ischemia, and related pathobiological states, with particular emphasis on the biologic mechanisms that determine the response to such injury. Making such information available will ultimately facilitate improved care of the traumatized or septic individual.
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