Department of Pediatrics and Department of Pathology & Immunology, Washington University School of Medicine in St. Louis, St. Louis, MO.
Blood. 2019 May 16;133(20):2130-2139. doi: 10.1182/blood-2018-11-844563. Epub 2019 Mar 21.
Primary immunodeficiencies affecting the function of neutrophils and other phagocytic leukocytes are notable for an increased susceptibility to bacterial and fungal infections as a result of impaired leukocyte recruitment, ingestion, and/or killing of microbes. The underlying molecular defects can also impact other innate immune responses to infectious and inflammatory stimuli, leading to inflammatory and autoimmune complications that are not always directly related to infection. This review will provide an update on congenital disorders affecting neutrophil function in which a combination of host defense and inflammatory complications are prominent, including nicotinamide dinucleotide phosphate oxidase defects in chronic granulomatous disease and β2 integrin defects in leukocyte adhesion deficiency.
原发性免疫缺陷影响中性粒细胞和其他吞噬白细胞的功能,由于白细胞募集、吞噬和/或杀死微生物的功能受损,导致其易患细菌和真菌感染。潜在的分子缺陷也会影响其他对感染和炎症刺激的固有免疫反应,导致炎症和自身免疫并发症,这些并发症并不总是与感染直接相关。本综述将提供有关影响中性粒细胞功能的先天性疾病的最新信息,其中宿主防御和炎症并发症突出,包括慢性肉芽肿病中的烟酰胺腺嘌呤二核苷酸磷酸氧化酶缺陷和白细胞黏附缺陷中的β2 整合素缺陷。