hypereosinophilia

嗜酸性粒细胞增多症
  • 文章类型: Journal Article
    Dupilumab已被批准通过抑制白细胞介素(IL)-4和IL-13信号传导来治疗患有2型炎症的严重哮喘。然而,dupilumab诱导的嗜酸性粒细胞增多(HE)已有报道,不应忽视.这项研究的目的是探讨dupilumab在中国重症哮喘患者中的疗效。HE是否影响其功效,以及他可能的危险因素。
    纳入2019年至2022年在广州医科大学附属第一医院接受dupilumab治疗至少12个月的20例重症哮喘患者。我们比较了dupilumab治疗前以及治疗后4个月和12个月的临床数据和实验室检查结果。根据dupilumab治疗是否触发HE定义为血液嗜酸性粒细胞计数(BEC)≥1.5×109细胞/L,将患者分为非HE组和HE组.
    患者显示哮喘控制测试(ACT)评分显着增加,恶化次数的减少,服用口服皮质类固醇(OCS)的患者比例和剂量下降,使用dupilumab治疗4个月和12个月后,肺功能参数FEV1/FVC(%)和FEV1(预测的%)显着改善。对于2型炎症生物标志物,呼出一氧化氮(FeNO)的分数浓度水平,痰嗜酸性粒细胞计数百分比(SEC%)和总免疫球蛋白E(TIgE)显著下降,而BEC在治疗4个月后较高,但12个月后恢复到基线水平。8例患者(40%)在dupilumab后出现无症状HE,HE组和非HE组之间的疗效没有显着差异。最早的BEC升高出现在治疗后1个月。但是大多数在6个月后下降了,在治疗12个月左右基本恢复到基线水平。此外,我们进一步发现,当患者FeNO≥60ppb时,食物过敏原阳性和联合嗜酸性中耳炎(EOM),治疗4个月和12个月后,他们的BEC显著高于对照组.
    这项研究表明dupilumab对中国重度哮喘患者有效,一些患者无症状,自我限制的他,这并不影响其疗效。此外,FeNO≥60ppb,食物过敏原阳性,与EOM的合并症可能是发展为HE的危险因素。
    Dupilumab has been approved for the treatment of severe asthma with type 2 inflammation by inhibiting interleukin (IL)-4 and IL-13 signaling. However, dupilumab-induced hypereosinophilia (HE) has been reported and should not be ignored. The aim of this study was to investigate the efficacy of dupilumab in Chinese patients with severe asthma, whether HE affects its efficacy, and the possible risk factors for HE.
    20 patients with severe asthma who received dupilumab treatment for at least 12 months in the First Affiliated Hospital of Guangzhou Medical University from 2019 to 2022 were included. We compared clinical data and laboratory tests results before dupilumab treatment and at 4 and 12 months after treatment. Based on whether dupilumab treatment triggers HE defined as blood eosinophil count (BEC) ≥ 1.5 × 109 cells/L, the patients were allocated into non-HE and HE groups.
    The patients showed a significant increase in asthma control test (ACT) scores, a decrease in the number of exacerbations, a decrease in the proportion of patients taking an oral corticosteroid (OCS) and in the dose, and a significant improvement in the pulmonary function parameters FEV1/FVC (%) and FEV1 (% predicted) after 4 and 12 months of treatment with dupilumab. For type 2 inflammatory biomarkers, the levels of fractional concentration of exhaled nitric oxide (FeNO), sputum eosinophil count percentage (SEC%) and total immunoglobulin E (TIgE) decreased significantly, whereas BEC were higher after 4 months of treatment, but returned to baseline levels after 12 months. 8 patients (40%) developed asymptomatic HE after dupilumab, and the efficacy was not significantly different between the HE and non-HE groups. The earliest BEC elevation appeared at 1 month after treatment, but most of them declined after 6 months, and basically returned to the baseline level around 12 months of treatment. In addition, we further found that when patients had FeNO ≥ 60 ppb, food allergens positive and combined eosinophilic otitis media (EOM), their BEC increased significantly more than that of the control group after 4 months as well as 12 months of treatment.
    This study demonstrated that dupilumab was efficacious in Chinese patients with severe asthma, and some patients developed asymptomatic, self-limited HE, which did not affect its efficacy. Additionally, FeNO ≥60 ppb, food allergens positive, and co-morbidities with EOM may be the risk factors for developing HE.
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  • 文章类型: Journal Article
    Introduction: Hypereosinophilic syndrome (HES) and immunoglobulin light-chain amyloidosis (ALA) are two, rare haematological disorders associated with cardiac alterations. Aim: The goal of the present study was a comparative assessment of left ventricular (LV) deformational parameters in HES and ALA patients using three-dimensional speckle-tracking echocardiography (3DSTE). Method: In the present study, results of 10 HES patients (mean age: 60.9 ± 14.7 years) and 19 ALA patients (mean age: 63.4 ± 7.8 years, 13 males) were analysed. The control group contained 13 age- and gender-matched healthy adults (mean age: 59.2 ± 4.3 years, 5 males). All patients underwent a complete two-dimensional Doppler echocardiography followed by 3DSTE. Results: All basal segmental LV strains were significantly reduced in ALA patients as compared to the control group. Global and mean segmental LV longitudinal strain (LS) values of ALA patients proved to be significantly decreased as compared to those of the healthy control group. During comparison of HES patients and healthy controls, significant difference could be detected in global LV-LS, while segmental basal LV-LS was also significantly reduced in HES patients. Basal LV radial and 3D strains showed significant differences when parameters of HES and ALA patient groups were compared. Conclusion: 3DSTE is a feasible tool for the detailed assessment of LV deformation in HES and ALA patients. Significant LV deformational abnormalities could be detected in both groups. In the case of ALA, these abnormalities are more prominent. Orv Hetil. 2020; 161(5): 169-176.
    Absztrakt: Bevezetés: A hypereosinophilia-szindróma (HES) és az immunglobulinkönnyűlánc-amyloidosis (ALA) két ritka hematológiai betegség, melyek cardialis eltérésekkel járnak együtt. Célkitűzés: A jelen vizsgálat célja a HES- és ALA-betegek bal kamrai (BK-i) deformációs paramétereinek összehasonlító vizsgálata volt háromdimenziós speckle-tracking echokardiográfia (3DSTE) segítségével. Módszer: A vizsgálatok során 10 HES-beteg (átlagos életkor: 60,9 ± 14,7 év) és 19 ALA-ban szenvedő páciens (átlagos életkor: 63,4 ± 7,8 év, 13 férfi) eredményeit elemeztük. Kontrollcsoportként 13, korban és nemben egyeztetett, egészséges felnőtt szolgált (átlagos életkor: 59,2 ± 4,3 év, 5 férfi). Valamennyi esetben teljes körű kétdimenziós Doppler-echokardiográfiás vizsgálat készült 3DSTE-vel kiegészítve. Eredmények: A kontrollcsoporthoz képest az ALA-betegcsoportban mért valamennyi basalis szegmentális BK-i strain szignifikánsan alacsonyabbnak mutatkozott. Az ALA-betegek globális és átlagolt szegmentális BK-i longitudinális strain (LS) értékei az egészséges kontrollcsoporthoz hasonlítva szignifikánsan alacsonyabbnak bizonyultak. A HES-betegcsoport és az egészséges kontrollok összehasonlítása során szignifikáns különbséget tapasztaltunk a globális BK-LS tekintetében, míg a szegmentális basalis BK-LS is szignifikánsan alacsonyabbnak bizonyult a HES-betegekben. A HES- és az ALA-betegcsoport értékeit összehasonlítva a basalis BK-i radiális és 3D strain mutatott szignifikáns eltérést. Következtetések: A 3DSTE alkalmas módszer a HES- és az ALA-betegcsoportban a BK-i deformációs mechanika részletes vizsgálatára. Mindkét betegcsoportban jelentős deformációs eltérések tapasztalhatók, ALA fennállása esetén az eltérések kifejezettebbek. Orv Hetil. 2020; 161(5): 169–176.
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