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Late effects after hematopoietic stem cell transplantation in patients with HLH: A Histiocyte Society, PDWP, IEWP, and TCWP EBMT Study 造血干细胞移植对HLH患者的晚期影响:组织细胞学会、PDWP、IEWP和TCWP EBMT研究
IF 11.2 1区 医学 Q1 ALLERGY Pub Date : 2026-02-01 DOI: 10.1016/j.jaci.2025.09.014
Kim Ramme MD, PhD , AnnaCarin Horne MD, PhD , Karin Beutel MD , Jacques-Emmanuel Galimard PhD , Ali Abdallah Alahmari MD , Giorgio Ottaviano MD , Despina Moshous MD, PhD , Savas Kansoy MD , Zohreh Nademi MD, PhD , Maura Faraci MD , Mikael Sundin MD, PhD , Franca Fagioli MD , Michael H. Albert MD , Petr Sedlacek MD , Yves Bertrand MD, PhD , Franco Locatelli MD, PhD , Catherine Paillard MD , Karin Mellgren MD, PhD , Ingo Müller MD , Johann Greil MD , Selim Corbacioglu MD, PhD

Background

Hematopoietic stem cell transplantation (HCT) is the only curative treatment in primary hemophagocytic lymphohistiocytosis (pHLH). However, HCT is associated with a wide range of late effects (LEs).

Objective

We sought to characterize the long-term outcome and LEs following HCT in pHLH.

Methods

A total of 274 children with pHLH from the European Society for Blood and Marrow Transplantation registry who underwent allogeneic HCT between 2004 and 2015 were included. Multivariable logistic regression models were used to evaluate the adjusted impact of baseline variables on central nervous system and hormonal LEs, respectively.

Results

A broad spectrum of LEs was identified, with neurologic (31%) and hormonal (34.8%) complications being the most prevalent. Chemotherapy (HLH-1994/HLH-2004) before HCT was identified as a significant risk factor for endocrinological LEs (P = .03), highlighting a novel aspect not previously reported. The presence of neurologic abnormality at diagnosis was an independent risk factor for neurologic LEs (P < .001) as was incomplete remission status at the time of HCT (P = .04).

Conclusions

HCT has significantly improved survival in patients with pHLH. However, survivors still face significant risks of LEs.
背景:造血干细胞移植是治疗原发性噬血细胞淋巴组织细胞病的唯一有效方法。然而,造血干细胞移植具有广泛的晚期效应。目的探讨造血干细胞移植治疗原发性噬血细胞淋巴组织细胞病的远期疗效和后期效应。方法纳入2004年至2015年间接受同种异体造血干细胞移植的EBMT登记的274例pHLH患儿。采用多变量logistic回归模型分别评估基线变量对中枢神经系统和激素晚期效应的调整影响。结果发现了广谱的晚期效应,其中神经系统并发症(31%)和激素并发症(34.8%)最为普遍。造血干细胞移植前化疗(HLH-94/HLH04)被确定为内分泌晚期效应的重要危险因素(p=0.03),突出了一个以前未报道的新方面。诊断时神经系统异常的存在是神经系统晚期效应的独立危险因素(p<0.001), HCT时不完全缓解状态也是(p=0.04)。结论造血干细胞移植可显著提高原发性噬血细胞淋巴组织细胞增多症患者的生存,但存活者仍面临显著的后期效应风险。
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引用次数: 0
COVID-19 infection raises respiratory type 2 inflammatory disease risk, whereas vaccination is protective COVID-19感染增加了呼吸道2型炎症性疾病的风险,而疫苗接种具有保护作用。
IF 11.2 1区 医学 Q1 ALLERGY Pub Date : 2026-02-01 DOI: 10.1016/j.jaci.2025.07.030
Henning Olbrich MD , Sophie L. Preuß MD , Khalaf Kridin MD, PhD , Gema Hernandez PhD , Diamant Thaçi MD , Ralf J. Ludwig MD , Philip Curman MD, PhD

Background

Coronavirus disease 2019 (COVID-19) infection and vaccination have unclear impacts on type 2 inflammatory diseases. Although viral infections can drive immune dysregulation, the extent to which COVID-19 infection and vaccination affect type 2 inflammatory diseases in various organ systems remains underexplored.

Objective

We sought to assess the risk of new-onset type 2 inflammatory diseases after COVID-19 infection and vaccination.

Methods

We conducted a large-scale retrospective matched cohort study using a US electronic health records database of more than 118 million patients. Three cohorts were defined: individuals with COVID-19 infection (n = 973,794), individuals with COVID-19 vaccination (n = 691,270), and unexposed controls (n = 4,388,409). Propensity score matching balanced demographic and clinical covariates. We calculated hazard ratios (HRs) for incident asthma, allergic rhinitis, chronic rhinosinusitis, atopic dermatitis, and eosinophilic esophagitis over 3-month follow-up.

Results

COVID-19 infection significantly increased the risks of asthma (HR 1.656, 95% CI 1.590-1.725), allergic rhinitis (HR 1.272, 95% CI 1.214-1.333), and chronic rhinosinusitis (HR 1.744, 95% CI 1.671-1.821). Risks for atopic dermatitis or eosinophilic esophagitis remained unchanged. In contrast, vaccination lowered the risks of asthma (HR 0.678, 95% CI 0.636-0.722) and chronic rhinosinusitis (HR 0.799, 95% CI 0.752-0.850). Direct comparison showed a 2- to 3-fold greater risk of respiratory type 2 inflammatory diseases with infection than with vaccination.

Conclusions

COVID-19 infection is associated with a heightened risk of respiratory type 2 inflammatory diseases, whereas vaccination appears protective.
背景covid -19感染和疫苗接种对2型炎症性疾病的影响尚不清楚。尽管病毒感染可导致免疫失调,但COVID-19感染和疫苗接种在多大程度上影响各器官系统的2型炎症性疾病仍未得到充分探讨。目的评估COVID-19感染和疫苗接种后新发2型炎症性疾病的风险。方法:我们在美国电子健康记录数据库中对超过1.18亿患者进行了大规模回顾性匹配队列研究。定义了三个队列:COVID-19感染个体(973,794),COVID-19疫苗接种个体(691,270)和未暴露对照组(4,388,409)。倾向得分匹配平衡人口统计学和临床协变量。我们计算了三个月随访期间哮喘、变应性鼻炎、慢性鼻窦炎、特应性皮炎和嗜酸性粒细胞性食管炎发生的风险比。结果scovid -19感染显著增加哮喘(风险比1.656,95%可信区间1.590 ~ 1.725)、变应性鼻炎(风险比1.272,风险比1.214 ~ 1.333)、慢性鼻窦炎(风险比1.744,风险比1.671 ~ 1.821)的发病风险。特应性皮炎或嗜酸性食管炎的风险保持不变。相比之下,接种疫苗降低了哮喘(0.678,0.636-0.722)和慢性鼻窦炎(0.799,0.752-0.850)的风险。直接比较表明,与接种疫苗相比,感染呼吸道2型炎症性疾病的风险高出两到三倍。结论covid -19感染与呼吸道2型炎症性疾病的风险增加有关,而接种疫苗具有保护作用。临床意义covid -19疫苗接种可减少由2型炎症引起的呼吸道并发症,从而减轻疾病负担。
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引用次数: 0
Post–COVID-19 atopic diseases risk: The need to disentangle variant-specific effects covid -19后的特应性疾病风险:需要理清变异特异性效应。
IF 11.2 1区 医学 Q1 ALLERGY Pub Date : 2026-02-01 DOI: 10.1016/j.jaci.2025.10.015
Mário Morais-Almeida MD , Bernardo Sousa-Pinto MD, PhD , Raquel Baptista-Pestana MD , Jean Bousquet MD, PhD
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引用次数: 0
Validation of a risk predictor score for early polyp recurrence in CRSwNP CRSwNP患者早期息肉复发风险预测评分的验证。
IF 11.2 1区 医学 Q1 ALLERGY Pub Date : 2026-02-01 DOI: 10.1016/j.jaci.2025.09.031
Junqin Bai PhD , Lutfiyya N. Muhammad PhD , Aditi Agarwal MBBS , Julia Huang MS , Caroline P.E. Price , Regan Harmon , Aiko Oka MD, PhD , Masanori Kidoguchi MD, PhD , Joo-Hee Kim MD, PhD , Zhidi Luo MS , Siyuan Dong MS , Chun-Kang Liao MD , Brooke Gleason , Whitney W. Stevens MD, PhD , David B. Conley MD , Kevin C. Welch MD , Stephanie S. Smith MD , Anju T. Peters MD , Robert P. Schleimer PhD , Robert C. Kern MD , Bruce K. Tan MD, MS

Background

Polyp recurrence (PR) can occur in patients with chronic rhinosinusitis with nasal polyps (CRSwNP) following endoscopic sinus surgery (ESS). We previously constructed a PR prediction model incorporating tissue biomarkers, including eosinophil cationic protein (ECP), IL-5, and anti–double-stranded DNA IgG, as well as clinical variables including the pre-ESS modified Lund-Mackay radiographic score and asthma status.

Objective

Our aim was to develop the model into a risk predictor score (RPS), validate it by using an independent set of patients, and test its ability to stratify patients by time to PR.

Methods

Two prospective cohorts of patients with CRSwNP (training and validation), were evaluated for PR for 2 to 5 years after ESS. Baseline demographics, pre-ESS radiographic and endoscopic assessments, and biomarker levels (using the ELISA and Luminex assays) were collected. The RPS was generated by using the prediction model coefficients as weights. Predictive accuracy was evaluated by using receiver operating characteristic analysis. The combined cohort was stratified into low-, intermediate-, and high-risk groups based on RPS tertiles, and Cox regression was used to analyze associations with time to PR.

Results

The model combining ECP level, IL-5 level, level of anti–double-stranded DNA IgG, asthma status, and pre-ESS modified Lund-Mackay radiographic score reliably predicted PR in the validation set (area under the curve = 0.76), which was comparable to the value found for the training set (area under the curve = 0.89) (DeLong P = .19). Cox regression analysis showed significant differences in recurrence time across RPS tertiles. Patients with a high RPS had a median PR time of 38 months, which was significantly shorter than the PR time in the intermediate group (54 months) and that in the group with the lowest RPS (60 months) (both P values < .01).

Conclusions

This study validated the RPS as a predictor for post-ESS PR in an independent cohort of patients with CRSwNP and demonstrated its effectiveness in identifying high-risk patients with significantly shorter times to recurrence.
内镜鼻窦手术(ESS)后慢性鼻窦炎合并鼻息肉(CRSwNP)患者可发生息肉复发(PR)。我们之前构建了一个PR预测模型,包括组织生物标志物,包括ECP、IL-5和抗dsdna IgG,以及临床变量,包括ess前修改的lnd - mackay (MLM)评分和哮喘状态。目的将该模型发展为风险预测评分(RPS),使用一组独立的患者对其进行验证,并测试其按PR时间对患者进行分层的能力。方法对两个前瞻性CRSwNP患者队列(培训和验证)进行评估,评估ess后2至5年的PR。收集基线人口统计学、ess前x线和内窥镜评估以及生物标志物水平(ELISA和Luminex检测)。以预测模型系数为权重生成RPS。采用受试者工作特征(ROC)分析评估预测准确性。根据RPS分位数将合并队列分为低、中、高风险组,Cox回归分析与PR时间的相关性。结果ECP、IL-5、抗dsdna IgG、哮喘状态和ess前MLM相结合的模型可靠地预测了验证集的PR (AUC = 0.76),与训练集(AUC = 0.89)相当(DeLong’s p=0.19)。Cox回归分析显示各RPS分位数的复发时间有显著差异。高rps组患者的中位PR时间为38个月,显著短于低rps组的中位PR时间(54个月)和60个月(p值均<0.01)。本研究在一个独立的CRSwNP队列中验证了RPS作为ess后PR的预测因子,并证明了其在识别复发时间显著缩短的高危患者方面的有效性。
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引用次数: 0
The complex relationship between inborn errors of immunity and autoimmunity 先天免疫错误与自身免疫的复杂关系
IF 11.2 1区 医学 Q1 ALLERGY Pub Date : 2026-02-01 DOI: 10.1016/j.jaci.2025.12.988
Pui Y. Lee MD, PhD , Roshini S. Abraham PhD
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引用次数: 0
News & Notes-AAAAI 新闻和笔记- aaaai
IF 11.2 1区 医学 Q1 ALLERGY Pub Date : 2026-02-01 DOI: 10.1016/S0091-6749(25)02204-3
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引用次数: 0
The evolving landscape of primary hemophagocytic lymphohistiocytosis: Long-term outcomes following hematopoietic cell transplantation 原发性噬血细胞淋巴组织细胞病的演变:造血细胞移植后的长期结果
IF 11.2 1区 医学 Q1 ALLERGY Pub Date : 2026-02-01 DOI: 10.1016/j.jaci.2025.12.990
Rebecca A. Marsh MD , Morton J. Cowan MD
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引用次数: 0
CME Calendar-AAAAI
IF 11.2 1区 医学 Q1 ALLERGY Pub Date : 2026-02-01 DOI: 10.1016/S0091-6749(25)02205-5
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引用次数: 0
Impact of Berotralstat on Healthcare Resource Utilization in Patients with Hereditary Angioedema with Normal C1-Inhibitor 贝洛特司他对c1抑制剂正常的遗传性血管性水肿患者医疗资源利用的影响
IF 11.2 1区 医学 Q1 ALLERGY Pub Date : 2026-02-01 DOI: 10.1016/j.jaci.2025.12.010
Bruce Zuraw MD , Tracy Yee , Francois Laliberte , Colleen Spencer , Sandra Nestler-Parr , Patrick Gillard , Sandra Christiansen MD
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引用次数: 0
Real-World Lanadelumab Effectiveness in Older Adults with HAE: Pooled Analysis From INTEGRATED and ENABLE 真实世界Lanadelumab在老年HAE患者中的有效性:来自INTEGRATED和ENABLE的汇总分析
IF 11.2 1区 医学 Q1 ALLERGY Pub Date : 2026-02-01 DOI: 10.1016/j.jaci.2025.12.017
Mauro Cancian MD, PhD , Laurence Bouillet , Irmgard Andresen MD , Natalie Khutoryansky , Daniel Nova Estepan , Inmaculada Martinez-Saguer
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引用次数: 0
期刊
Journal of Allergy and Clinical Immunology
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