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常见可变免疫缺陷患者多形核中性粒细胞的功能缺陷

Defective functions of polymorphonuclear neutrophils in patients with common variable immunodeficiency.

作者信息

Casulli Sarah, Coignard-Biehler Hélène, Amazzough Karima, Shoai-Tehrani Michka, Bayry Jagadeesh, Mahlaoui Nizar, Elbim Carole, Kaveri Srini V

机构信息

UMR-S CR7, Sorbonne University, UPMC University Paris 06, 75005, Paris, France.

出版信息

Immunol Res. 2014 Oct;60(1):69-76. doi: 10.1007/s12026-014-8555-7.

Abstract

Common variable immunodeficiency (CVID) is a heterogeneous antibody deficiency condition with alterations in T cell regulation and function, dendritic and B-cell compartment and represents the most frequent cause of symptomatic primary immunodeficiency. We addressed whether CVID is associated with abnormalities in the polymorphonuclear neutrophil (PMN) compartment, an important component of innate immunity and plays a key role in host defenses against invading microorganisms. We used flow cytometry to examine PMN phenotypic and functional abnormalities in CVID patients, using whole-blood conditions in order to avoid artifacts due to isolation procedures. We demonstrated that PMN from CVID patients displays, at resting state, a decreased expression of CD15, CD11b and CD16b, which might be related to an abnormality in neutrophil maturation. In addition, these neutrophils exhibit a decrease in degranulation, phagocytosis and reactive oxygen species production, as well as an increased death by apoptosis. These PMN abnormalities observed in CVID patients could result in an increased risk for recurrent bacterial infections.

摘要

普通变异型免疫缺陷(CVID)是一种异质性抗体缺陷疾病,伴有T细胞调节和功能、树突状细胞和B细胞区室的改变,是有症状的原发性免疫缺陷的最常见原因。我们探讨了CVID是否与多形核中性粒细胞(PMN)区室的异常有关,PMN是固有免疫的重要组成部分,在宿主抵御入侵微生物的防御中起关键作用。我们使用流式细胞术,在全血条件下检测CVID患者的PMN表型和功能异常,以避免因分离程序导致的假象。我们证明,CVID患者的PMN在静息状态下,CD15、CD11b和CD16b的表达降低,这可能与中性粒细胞成熟异常有关。此外,这些中性粒细胞的脱颗粒、吞噬作用和活性氧生成减少,凋亡导致的死亡增加。在CVID患者中观察到的这些PMN异常可能导致复发性细菌感染的风险增加。

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