Immunodeficiency: Quantitative and qualitative phagocytic cell defects.

David Mickey,Juanita Valdes Camacho,Adil Khan,David Kaufman
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Abstract

The immune system is divided into two major branches: innate and adaptive. The innate immune system is the body's first line of defense and rapidly responds in a nonspecific manner to various microorganisms, foreign materials, or injuries. Phagocytes, which include macrophages, monocytes, and neutrophils, are innate immune cells that can surround and kill microorganisms, ingest foreign material, and remove dead cells. They also indirectly boost both innate and adaptive immune responses through various activation signals. Phagocytic defects characteristically lead to fungal and bacterial infections of the respiratory tract, lymph nodes, skin, and other organ systems, and they are commonly associated with inflammatory bowel disease. This primer will review high-yield innate defects of phagocytic cells, including defects of respiratory (oxidative) burst, defects of neutrophil migration, cyclic and severe congenital neutropenias and associated disorders, and other phagocyte defect disorders.
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免疫缺陷:吞噬细胞的数量和质量缺陷。
免疫系统分为两大分支:先天性免疫系统和适应性免疫系统。先天性免疫系统是人体的第一道防线,能以非特异性的方式对各种微生物、外来物质或损伤做出快速反应。吞噬细胞(包括巨噬细胞、单核细胞和中性粒细胞)是先天性免疫细胞,可以包围并杀死微生物、摄取异物并清除死亡细胞。它们还能通过各种激活信号间接促进先天性和适应性免疫反应。吞噬细胞缺陷的特点是导致呼吸道、淋巴结、皮肤和其他器官系统的真菌和细菌感染,而且通常与炎症性肠病有关。本初级读本将综述吞噬细胞的高产先天缺陷,包括呼吸(氧化)爆发缺陷、中性粒细胞迁移缺陷、周期性和严重先天性中性粒细胞减少症及相关疾病,以及其他吞噬细胞缺陷疾病。
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