Leukocyte Disorders

白细胞紊乱
  • 文章类型: Journal Article
    慢性自发性荨麻疹(CSU)是一种令人不安的皮肤病,通常严重损害生活质量。炎症的血液学标志物如嗜中性粒细胞-淋巴细胞和血小板-淋巴细胞可用于评估炎性皮肤病。它们在荨麻疹中的有用性未知。中性粒细胞对淋巴细胞,血小板到淋巴细胞,和血清总IgE在荨麻疹患者进行了研究:急性自发性荨麻疹(ASU)与CSU,儿童与CSU成人相比,以及轻度至中度与重度CSU患者。这项回顾性队列研究包括2005年至2020年诊断为荨麻疹的所有年龄段的患者以及诊断后30天内的血细胞计数。合并症影响血细胞的患者(感染,手术,恶性肿瘤)被排除在外。在ASU与CSU以及轻度至中度CSU与重度CSU(通过使用全身药物或住院来定义)的患者中,评估了中性粒细胞对淋巴细胞和血小板对淋巴细胞的影响。共有13,541例荨麻疹患者被纳入研究。CSU患者(n=5,021)有较高的中性粒细胞对淋巴细胞和血小板对淋巴细胞,以及与ASU患者(n=8,520)相比的血清IgE水平。成人的中性粒细胞对淋巴细胞和血小板对淋巴细胞的比例高于儿童。与轻度至中度受影响的患者(n=4,968)相比,严重受影响的患者(n=53)的中性粒细胞对淋巴细胞和血小板对淋巴细胞的比例更高。中性粒细胞对淋巴细胞和血小板对淋巴细胞较高的患者患CSU而不是ASU和严重荨麻疹的几率更高,而轻度至中度。总之,中性粒细胞-淋巴细胞和血小板-淋巴细胞是简单且可用的标志物,可用于预测和评估重度和慢性荨麻疹.
    Chronic spontaneous urticaria (CSU) is a disturbing skin condition often severely detrimental to quality of life. Haematological markers of inflammation such as neutrophil-to-lymphocyte and platelet-to-lymphocyte may be used in the assessment of inflammatory skin diseases. Their usefulness in urticaria is unknown. Neutrophil- to-lymphocyte, platelet-to-lymphocyte, and total serum IgE were investigated in urticaria patients: acute spontaneous urticaria (ASU) versus CSU, children versus adults with CSU, and patients with mild-to-moderate versus severe CSU. This retrospective cohort study included patients of all ages diagnosed with urticaria between 2005 and 2020 and blood counts within 30 days of diagnosis. Patients with comorbidities influencing blood cells (infection, surgery, malignancy) were excluded. Neutrophil-to-lymphocyte and platelet-to-lymphocyte were evaluated in patients with ASU vs CSU and mild-to-moderate CSU vs severe CSU (defined by the use of systemic medications or hospitalizations). A total of 13,541 urticaria patients were included in the study. CSU patients (n = 5,021) had higher neutrophil-to-lymphocyte and platelet-to-lymphocyte, as well as serum IgE levels compared with ASU patients (n = 8,520). Adults had higher neutrophil-to-lymphocyte and platelet-to-lymphocyte than children. Severely affected patients (n = 53) had higher neutrophil-to-lymphocyte and platelet-to-lymphocyte compared with mild-to-moderately affected patients (n = 4,968). Patients with higher neutrophil-to-lymphocyte and platelet-to-lymphocyte had higher odds of having CSU rather than ASU and severe urticaria rather mild-to-moderate. In conclusion, neutrophil-to-lymphocyte and platelet-to-lymphocyte are simple and available markers that can be used to predict and assess severe and chronic urticaria.
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  • 文章类型: Editorial
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  • 文章类型: Case Reports
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  • 文章类型: Review
    背景:嗜酸细胞性膀胱炎(EC)是临床上罕见的特异性透壁性炎症性疾病。目前,其病因不明,其临床表现多样,其辅助检查缺乏特异性,临床上容易漏诊或误诊。
    方法:1例72岁男性下尿路梗阻伴血尿患者,经B超和尿CT检查诊断为良性前列腺增生伴出血。在接受导管插入治疗后,抗感染和止血,他接受了经尿道前列腺电切术的选择性治疗,但是他在手术中看到膀胱右后壁上有一个图案肿块。考虑到膀胱肿瘤,他切除了病灶,并给予吡柔比星膀胱灌注。然而,术后病理结果为EC。
    方法:EC的诊断只能依靠病理检查,通过在多点活检的同时尽可能多地获取肌肉组织,可以提高活检的准确性和阳性率。
    方法:经尿道切除病灶后口服泼尼松和西替利嗪,并定期给予坦索罗辛和非那雄胺治疗良性前列腺增生。
    结果:随访半年无复发及排尿异常,上尿路功能正常。
    结论:EC的临床表现不典型,实验室检查和影像学检查没有特异性,手术前很难明确诊断。诊断取决于病理检查。经尿道病灶电切术在彻底清除病灶的同时,可明显提高活检阳性率,联合药物治疗可在短时间内取得满意效果。术后积极的随访对明确病情复发、防止上尿路功能受损非常重要。
    BACKGROUND: Eosinophilic cystitis (EC) is a rare and specific transmural inflammatory disease in clinic. At present, its etiology is unknown, its clinical manifestations are diverse, and its auxiliary examination lacks specificity, so it is easy to be missed or misdiagnosed in clinical practice.
    METHODS: A 72-year-old male patient with symptoms of lower urinary tract obstruction accompanied by hematuria was diagnosed with benign prostatic hyperplasia with bleeding by B-ultrasound and urinary CT examination. After being treated with catheterization, anti-infection and hemostasis, he was selectively treated with transurethral resection of prostate, but he saw a pattern mass on the right back wall of the bladder during the operation. Considering bladder tumor, he removed the lesion and gave pirarubicin for bladder perfusion. However, the postoperative pathological result was EC.
    METHODS: The diagnosis of EC can only rely on pathological examination, and the accurate and positive rate of biopsy can be improved by obtaining muscle tissue as much as possible at the same time of multi-point biopsy.
    METHODS: Prednisone and cetirizine were given orally after transurethral resection of lesions, and tamsulosin and finasteride were given regularly to treat benign prostatic hyperplasia.
    RESULTS: No recurrence and abnormal urination were found during the follow-up for half a year, and the upper urinary tract function was normal.
    CONCLUSIONS: The clinical manifestations of EC are atypical, the laboratory examination and imaging examination are not specific, and it is difficult to make a definite diagnosis before operation. The diagnosis depends on pathological examination. Transurethral resection of the lesion can obviously improve the positive rate of biopsy while completely removing the lesion, and the combined drug treatment can achieve satisfactory results in a short period of time. Active follow-up after operation is very important to identify the recurrence of the disease and prevent the upper urinary tract function from being damaged.
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  • 文章类型: Multicenter Study
    背景:白细胞介素-5(IL-5)抑制剂代表了嗜酸性肉芽肿性多血管炎(EGPA)的新疗法。这项研究评估了IL-5受体抑制剂benralizumab在欧洲EGPA患者队列中的有效性和安全性。
    方法:这项回顾性队列研究包括来自欧洲EGPA研究组的28个欧洲转诊中心的EGPA患者,这些患者来自六个国家(意大利,法国,英国,俄罗斯,西班牙,和瑞士),他们在2019年1月1日至2022年9月30日之间接受了贝那利珠单抗作为任何治疗方案。我们评估了完全反应率,定义为无疾病活动(伯明翰血管炎活动评分[BVAS]为0)和泼尼松剂量高达4mg/天,与部分反应相反,定义为BVAS为0,泼尼松剂量大于4mg/天。活动性疾病表现,肺功能,糖皮质激素剂量的变化,在12个月的随访中还评估了安全性结局.
    结果:纳入121例复发难治性EGPA患者(64[53%]女性和57[47%]男性;开始benralizumab治疗时的中位年龄54·1岁[IQR44·2-62·2])。15例报告完全缓解(12.4%,在第3,25个月时,121例患者的95%CI7·1-19·6(28·7%,19·5-39·4)在第6个月的87例患者中,32例(46·4%,34·3-58·8)在12个月时有69名患者;在另外43名患者中观察到部分反应(35·5%,27·0-44·8)患者在3个月,23(26·4%,17·6-37·0),第6个月和第13个月(18·8%,10·4-30·1)在12个月。BVAS从基线时的3·0(IQR2·0-8·0)降至第3个月和第6个月时的0·0(0·0-2·0),并在第12个月时降至0·0(0·0-1·0)。有全身表现的患者比例,活动性周围神经疾病,耳朵,鼻子,和喉咙受累,肺部受累减少,随着肺功能测试的改善。六名患者在完全反应后复发。口服泼尼松(或等效剂量)从基线时的10·0mg/天(5·0-12·5)减少到第3个月时的5·0mg/天(3·6-8·5)(p<0·01),在第6个月达到5·0毫克/天(2·5-6·3),在第12个月达到2·5毫克/天(0·0-5·0)(p<0·0001)。121例患者中有19例(16%)出现不良事件,16例(13%)停用贝那利珠单抗。
    结论:这些数据表明,贝那利珠单抗在实际临床实践中可能是EGPA的有效治疗方法。需要进一步的临床试验来确认贝那利珠单抗在具有较高基线疾病活动性的患者中的疗效。
    背景:无。
    Interleukin-5 (IL-5) inhibitors represent novel therapies for eosinophilic granulomatosis with polyangiitis (EGPA). This study assessed the effectiveness and safety of the IL-5 receptor inhibitor benralizumab in a European cohort of patients with EGPA.
    This retrospective cohort study included patients with EGPA from 28 European referral centres of the European EGPA Study Group across six countries (Italy, France, UK, Russia, Spain, and Switzerland) who received benralizumab as any line of treatment between Jan 1, 2019, and Sep 30, 2022. We assessed the rates of complete response, defined as no disease activity (Birmingham Vasculitis Activity Score [BVAS] of 0) and a prednisone dose of up to 4 mg/day, in contrast to partial response, defined as a BVAS of 0 and a prednisone dose greater than 4 mg/day. Active disease manifestations, pulmonary function, variation in glucocorticoid dose, and safety outcomes were also assessed over a 12-month follow-up.
    121 patients with relapsing-refractory EGPA treated with benralizumab at the dose approved for eosinophilic asthma were included (64 [53%] women and 57 [47%] men; median age at the time of beginning benralizumab treatment 54·1 years [IQR 44·2-62·2]). Complete response was reported in 15 (12·4%, 95% CI 7·1-19·6) of 121 patients at month 3, 25 (28·7%, 19·5-39·4) of 87 patients at month 6, and 32 (46·4%, 34·3-58·8) of 69 patients at month 12; partial response was observed in an additional 43 (35·5%, 27·0-44·8) patients at month 3, 23 (26·4%, 17·6-37·0) at month 6, and 13 (18·8%, 10·4-30·1) at month 12. BVAS dropped from 3·0 (IQR 2·0-8·0) at baseline to 0·0 (0·0-2·0) at months 3 and 6, and to 0·0 (0·0-1·0) at month 12. The proportion of patients with systemic manifestations, active peripheral neurological disease, ear, nose, and throat involvement, and pulmonary involvement decreased, with an improvement in lung function tests. Six patients relapsed after having a complete response. The oral prednisone (or equivalent) dose decreased from 10·0 mg/day (5·0-12·5) at baseline to 5·0 mg/day (3·6-8·5) at month 3 (p<0·01), to 5·0 mg/day (2·5-6·3) at month 6, and to 2·5 mg/day (0·0-5·0) at month 12 (p<0·0001). 19 (16%) of 121 patients had adverse events and 16 (13%) discontinued benralizumab.
    These data suggest that benralizumab could be an effective treatment for EGPA in real-life clinical practice. Further clinical trials are required to confirm the efficacy of benralizumab in patients with a higher baseline disease activity.
    None.
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  • 文章类型: Journal Article
    嗜酸性粒细胞相关疾病代表一组具有高度异质性的临床表现和从轻度到危急的症状的病理状况。通常用糖皮质激素治疗全身性和局部形式的疾病。针对白细胞介素5途径的新型生物疗法的批准可以帮助减少在嗜酸性粒细胞疾病中使用全身性糖皮质激素(SGC),并降低SGC相关不良反应(AE)的风险。在这篇文章中,来自不同医学专业的专家小组审查了在两种全身性嗜酸性粒细胞疾病中使用SGC的最新证据:嗜酸性肉芽肿伴多发性血管炎(EGPA)和嗜酸性粒细胞增多综合征(HES);在两种单器官(呼吸道)嗜酸性粒细胞疾病中:慢性鼻鼻窦炎伴鼻息肉(CRSwNP)和重度哮喘伴嗜酸性粒细胞表型(EP-SA)并将其与他们在临床实践中的经验进行了对比。使用标称分组技术,他们就与SGC的剂量和逐渐减少有关的关键方面以及作为SGC保护剂的生物制剂的启动达成共识。早期使用生物制剂治疗有助于预防与SGC中长期使用相关的不良事件。
    Eosinophil-related diseases represent a group of pathologic conditions with highly heterogeneous clinical presentation and symptoms ranging from mild to critical. Both systemic and localized forms of disease are typically treated with glucocorticoids. The approval of novel biologic therapies targeting the interleukin-5 pathway can help reduce the use of systemic glucocorticoids (SGC) in eosinophilic diseases and reduce the risk of SGC-related adverse effects (AEs). In this article, a panel of experts from different medical specialties reviewed current evidence on the use of SGC in two systemic eosinophilic diseases: Eosinophilic Granulomatosis with PolyAngiitis (EGPA) and HyperEosinophilic Syndrome (HES); and in two single-organ (respiratory) eosinophilic diseases: Chronic RhinoSinusitis with Nasal Polyps (CRSwNP) and Severe Asthma with Eosinophil Phenotype (SA-EP), and contrasted it with their experience in clinical practice. Using nominal group technique, they reached consensus on key aspects related to the dose and tapering of SGC as well as on the initiation of biologics as SGC-sparing agents. Early treatment with biologics could help prevent AEs associated with medium and long-term use of SGC.
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  • 文章类型: Journal Article
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    文章类型: Journal Article
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  • 文章类型: Journal Article
    肠道微生物群在影响各种健康结果中起着关键作用,包括免疫介导的疾病。多血管炎肉芽肿病(GPA)就是这样一种情况,其与肠道微生物群的潜在关联仍未充分开发。
    使用双样本孟德尔随机化方法,我们调查了肠道菌群和GPA之间的因果关系。我们的数据来自多个队列和联盟,包括MiBioGen财团.我们的研究设计纳入了免疫性状对肠道微生物群和GPA之间关系的直接关联和调解作用。
    我们的分析显示,1门之间存在显著关联,1科9属微生物群分类群和GPA。此外,我们确定了几种介导肠道微生物群对GPA影响的免疫细胞性状。例如,Defluviitaleaceae和DefluviitaleaceaeUCG011通过粒细胞中的CD11c影响GPA。中介效应比例进一步阐明了肠道微生物群暴露之间的复杂动态,免疫标记,以及它们对GPA的综合影响。
    我们的发现强调了肠道微生物群之间的复杂关系,免疫标记,和GPA。所确定的关联和中介效应为针对肠道微生物群管理GPA的潜在治疗途径提供了有价值的见解。
    The gut microbiota plays a pivotal role in influencing various health outcomes, including immune-mediated conditions. Granulomatosis with Polyangiitis (GPA) is one such condition, and its potential associations with gut microbiota remain underexplored.
    Using a two-sample Mendelian randomization approach, we investigated the causal links between gut microbiota and GPA. We sourced our data from multiple cohorts and consortiums, including the MiBioGen consortium. Our study design incorporated both direct associations and mediation effects of immune traits on the relationship between gut microbiota and GPA.
    Our analysis revealed significant associations between 1 phylum, 1 family 9 genus microbiota taxa and GPA. Furthermore, we identified several immune cell traits that mediated the effects of gut microbiota on GPA. For instance, the family Defluviitaleaceae and genus Defluviitaleaceae UCG011 influenced GPA through CD11c in granulocytes. The mediation effect proportions further elucidated the complex dynamics between gut microbiota exposures, immune markers, and their combined influence on GPA.
    Our findings underscore the intricate relationship between gut microbiota, immune markers, and GPA. The identified associations and mediation effects provide valuable insights into the potential therapeutic avenues targeting gut microbiota to manage GPA.
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