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17岁男童,因腹痛、腹泻伴嗜酸性粒细胞增多3年,加重3 d入院。3年前患儿因进食“老酸奶”后出现腹痛、腹泻,彩超示大量腹腔积液,血常规、骨髓细胞形态学检查、腹水组织学检查可见大量嗜酸性粒细胞;3 d前因腹痛、腹泻再次入院,胃肠镜检查胃角见嗜酸性粒细胞浸润,确诊为嗜酸细胞性胃肠疾病(嗜酸细胞性胃肠炎),给予糖皮质激素及饮食规避治疗后好转,随访1年未反复。对于因腹痛、腹泻等消化道症状就诊的患儿,如伴外周血嗜酸性粒细胞增多,需考虑嗜酸细胞性胃肠疾病的可能,内镜活检胃肠道组织中见嗜酸性粒细胞浸润且计数异常为诊断的关键。 [引用格式:中国当代儿科杂志,2021,23(11):1169-1173]  相似文献   
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<正>嗜酸性筋膜炎(eosinophilic fasciitis,EF)是一种罕见的硬皮病样疾病,由Shulman~[1]在1974年首次报道,以对称性的四肢肿痛、皮肤硬化、外周血嗜酸性粒细胞增多、高丙种球蛋白血症、血沉加快等为主要临床特征。EF在各年龄段均可发病,平均发病年龄多集中在40~50岁之间~[2],男女发病率基本一致~[3]。目前尚无国际诊断标准,EF的诊断多建立在典型的临  相似文献   
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Monogenic diseases of the immune system, also known as inborn errors of immunity (IEIs), are caused by single-gene mutations and result in immune deficiency and dysregulation. More than 400 monogenic diseases have been described to date, and this number is rapidly expanding. The increasing availability of next-generation sequencing is now facilitating the diagnosis of IEIs. It is known that IEIs can predispose a person to not only infectious diseases but also cancer and immune disorders, such as inflammatory, autoimmune, and atopic diseases. IEIs with eosinophilia and atopic diseases can occur in several disorders. IEIs with eosinophilia have provided insights into human immunity and the pathogenesis of allergic diseases. Eosinophilia is not a rare finding in clinical practice, and it often poses problems in terms of etiologic research and differential diagnoses. Secondary eosinophilia is the most common form. The main underlying conditions are infectious diseases such as parasitic infections, allergic disorders, drug reactions, and of course IEIs. In clinical settings, the recognition of IEIs in the context of an allergic phenotype with eosinophilia is critical for prompt diagnosis and appropriate treatment aimed at modulating pathophysiological mechanisms and improving clinical symptoms.  相似文献   
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Eosinophilia in the peripheral blood is classified as primary (clonal) hematologic neoplasms or secondary (nonclonal) disorders, associated with hematologic or nonhematologic disorders. This review focuses on the categories of hematolymphoid neoplasms recognized by the 2008 World Health Organization Classification of Tumours and Haematopoietic and Lymphoid Tissues that are characteristically associated with eosinophilia. We provide a systematic approach to the diagnosis of these neoplastic proliferations via morphologic, immunophenotypic, and molecular-based methodologies, and provide the clinical settings in which these hematolymphoid neoplasms occur. We discuss recommendations that eosinophilia working groups have published addressing some of the limitations of the current classification scheme.  相似文献   
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AIM: To validate methods for determining mast cell density, extracellular major basic protein content, and presence of fibrosis in esophageal eosinophilia.METHODS: Twenty specimens with > 20 eosinophils/high-power field (hpf) classified as high eosinophil density (HE) and 20 specimens with < 5 eosinophils/hpf classified as low esophageal density (LE) were identified. All 40 specimens underwent immunohistochemical staining and trichrome staining. Mast cell density, extracellular major basic protein (MBP) density, and presence of subepithelial fibrosis were assessed in a standardized manner. All specimens were evaluated by two separate observers and by a single observer on two separate occasions to evaluate reproducibility of the methods.RESULTS: A strong inter-observer correlation was noted for both peak and mean mast cell counts (r = 0.725, P < 0.0001 and r = 0.823, P < 0.0001). A strong intra-observer correlation also was noted for both peak and mean mast cell counts (r = 0.752, P < 0.0001 and r = 0.878, P < 0.0001). A very strong inter-observer correlation was noted for both peak (τ = 0.867, P < 0.0001) and mean extracellular MBP densities (r = 0.925, P < 0.0001). A very strong intra-observer correlation was noted for both peak (τ = 0.875; P < 0.0001) and mean extracellular MBP densities (r = 0.956, P < 0.0001). Excellent inter-rater reliability was found for fibrosis (κ = 0.887). Mast cell and MBP densities, as well as presence of fibrosis, were significantly increased in HE vs LE. The HE group had significantly higher intraepithelial mast cell peak (29.35 ± 21.61 vs 12.45 ± 8.26, P = 0.002) and mean (19.84 ± 15.81 vs 6.35 ± 4.5, P = 0.001) densities than the LE group. The HE group had significantly higher peak extracellular MBP (2.35 ± 0.67 vs 0.45 ± 0.61, P < 0.001) and mean extracellular MBP (1.95 ± 0.76 vs 0.20 ± 0.29, P < 0.0001) densities than the LE group. Seventy-three percent of patients with HE (11/15) had fibrosis, whereas only 10% of patients with LE (1/10) had fibrosis (P < 0.01). MBP performed the best in predicting classification of HE vs LE, with mean MBP demonstrating 100% sensitivity and 95% specificity at the optimal cut point.CONCLUSION: This study provides methodology and proof-of-concept for future evaluation of these biomarkers for differentiating esophageal eosinophilic diseases such as reflux esophagitis and eosinophilic esophagitis.  相似文献   
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宋明月  刘沁华  丁刚  吴燕明 《安徽医药》2022,26(6):1193-1196
目的探讨伴嗜酸粒细胞( EOS)增多的外周 T细胞淋巴瘤( PTCL)的发病机制、临床特点及治疗措施。方法分析安徽医科大学附属巢湖医院 2018年 7月收治的 1例及国内外近 5年文献报道确诊的 14例伴 EOS增多的 PTCL病人的临床资料,探讨该病的发病机制、临床特点及治疗和预后。结果 15例病人中高龄者多见, Ann Arbor分期晚,结外有多处组织侵犯,大多数国际预后指数( IPI)评分位于中高危组。除 1例失访外,化疗组( n=12)总生存时间[ 10.00(6.50,32.13)个月]与对症治疗组(n=2)总生存时间(分别为 11.00个月, 14.00个月)相比,差异无统计学意义( P>0.05);一线方案化疗组( n=10)总生存时间[10.00(7.50,26.37)个月]与二线方案化疗组( n=2)总生存时间(分别为 2.50个月, 50.00个月)相比,差异无统计学意义( P>  相似文献   
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洪舟  潘龙飞  杜英俊 《海南医学》2014,(19):2924-2926
目的探讨限制型心肌病患者心内膜心肌活检的组织学病理特点以及相关心功能变化。方法对我院急诊科2010年1月至2013年10月收治的40例限制型心肌病患者(心肌病组)进行心内膜心肌活检,并做实验室检查、右心导管以及心肌病理检查,对其结果进行分析,并与40例正常对照组比较。结果心肌病组的B型利钠肽(BNP)、肌酸激酶同工酶(CK-MB)和肌钙蛋白I(TnI)明显高于对照组(P均〈0.05);心肌病组的右心房压、右心室收缩压、右心室舒张末压和肺动脉楔压明显高于对照组[右心房压:(18.7±4.5)mmHg vs(10.2±1.4)mmHg;右心室收缩压:(46.5±5.6)mmHg vs(34.7±3.7)mmHg;右心室舒张末压:(16.3±4.6)mmHg vs(8.6±2.6)mmHg;肺动脉楔压:(28.6±5.3)mmHg vs(14.2±3.8)mmHg,P均〈0.05];20例诊断为心肌淀粉样变性,6例诊断为嗜酸性粒细胞增多综合征累及心肌,14例心内膜心肌活检提示存在心肌病变。结论淀粉样变性、嗜酸性粒细胞浸润和特发性心肌病是限制型心肌病的主要病因。心内膜心肌活检对于限制型心肌病的病因诊断意义重大。  相似文献   
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