Department of Pediatrics, Oncology and Hematology, Medical University of Lodz, Lodz, Poland.
Laboratory of Epigenetics, Institute of Medical Biology, Polish Academy of Sciences, Lodz, Poland.
Front Immunol. 2023 Jun 7;14:1128581. doi: 10.3389/fimmu.2023.1128581. eCollection 2023.
Congenital defects of neutrophil number or function are associated with a severe infectious phenotype that may require intensive medical attention and interventions to be controlled. While the infectious complications in inherited neutrophil disorders are easily understood much less clear and explained are autoimmune and autoinflammatory phenomena. We survey the clinical burden of autoimmunity/autoinflammation in this setting, search for common patterns, discuss potential mechanisms and emerging treatments.
先天性中性粒细胞数量或功能缺陷与严重的感染表型相关，可能需要密切的医疗关注和干预来进行控制。虽然遗传性中性粒细胞疾病的感染并发症很容易理解，但自身免疫和自身炎症现象则不那么清楚和易于解释。我们在这种情况下调查了自身免疫/自身炎症的临床负担，寻找共同的模式，讨论潜在的机制和新兴的治疗方法。
