CD18的临床表现及表达指导1型白细胞粘附缺乏症的诊断:墨西哥经验

IF 2.5 4区 医学 Q3 ALLERGY
Elma Isela Fuentes-Lara, Gabriel Emmanuel Arce-Estrada, Abner Bojalil-Cabildo, Marco Antonio Yamazaki-Nakashimada, Sara Elva Espinosa-Padilla, Luisa Berenise Gamez-Gonzalez, Rosa Mar�a Nideshda Ramirez-Uribe, Omar Josue Saucedo-Ramirez, Laura Berron-Ruiza
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引用次数: 0

摘要

背景:白细胞粘附缺陷1型(LAD-1)是一种以白细胞运输缺陷为特征的先天性免疫错误。方法:对临床怀疑为lad1的患者进行转诊。进行全血细胞计数和流式细胞术分析,以确定CD18、CD11b的表达和淋巴细胞群体表型,并完成统计分析。结果:我们报告了6例诊断为lad1的墨西哥患者的临床表现和免疫学结果。诊断基于典型的临床表现,结合白细胞增多的实验室证明,以及白细胞上CD18及其相关分子CD11a, CD11b和CD11c的明显减少或几乎缺失。我们发现了其他国家未描述的非典型表现,如早发性自身免疫或某些微生物引起的感染。结论:lad1患者可能表现不典型,流式细胞术是诊断lad1不可缺少的工具。我们提出了拉丁美洲国家的第一份lad1患者报告。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Clinical manifestations and expression of CD18 to guide the diagnosis of leukocyte adhesion deficiency type 1: Mexico experience
Background: Leukocyte adhesion deficiency type 1 (LAD-1) is an inborn error of immunity characterized by a defect in leukocyte trafficking. Methods: Patients with clinical suspicion of LAD-1 were referred to our institution. Complete blood count and flow cytometric analysis, to identify the expression of CD18, CD11b, and the lymphocyte population phenotyping, were performed, and statistical analysis was completed. Results: We report clinical manifestations and immunological findings of six Mexican patients diagnosed with LAD-1. The diagnosis was based on typical clinical presentation, combined with laboratory demonstration of leukocytosis, and significant reduction or near absence of CD18 and its associated molecules CD11a, CD11b, and CD11c on leukocytes. We found atypical manifestations, not described in other countries, such as early-onset autoimmunity or infections caused by certain microorganisms. Conclusions: Patients with LAD-1 may present with atypical manifestations, making flow cytometry an indispensable tool to confirm the diagnosis. We present the first report of LAD-1 patients in a Latin American country.
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来源期刊
CiteScore
3.70
自引率
0.00%
发文量
131
审稿时长
6-12 weeks
期刊介绍: Founded in 1972 by Professor A. Oehling, Allergologia et Immunopathologia is a forum for those working in the field of pediatric asthma, allergy and immunology. Manuscripts related to clinical, epidemiological and experimental allergy and immunopathology related to childhood will be considered for publication. Allergologia et Immunopathologia is the official journal of the Spanish Society of Pediatric Allergy and Clinical Immunology (SEICAP) and also of the Latin American Society of Immunodeficiencies (LASID). It has and independent international Editorial Committee which submits received papers for peer-reviewing by international experts. The journal accepts original and review articles from all over the world, together with consensus statements from the aforementioned societies. Occasionally, the opinion of an expert on a burning topic is published in the "Point of View" section. Letters to the Editor on previously published papers are welcomed. Allergologia et Immunopathologia publishes 6 issues per year and is included in the major databases such as Pubmed, Scopus, Web of Knowledge, etc.
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