Department of Pediatrics (Hematology/Oncology), St. Louis Children's Hospital, Washington University School of Medicine in St. Louis, St. Louis, MO, USA.
Department of Pathology and Immunology, St. Louis Children's Hospital, Washington University School of Medicine in St. Louis , St. Louis, MO, USA.
Methods Mol Biol. 2020;2087:11-29. doi: 10.1007/978-1-0716-0154-9_2.
Primary disorders of neutrophil function result from impairment in neutrophil responses that are critical for host defense. This chapter summarizes inherited disorders of neutrophils that cause defects in neutrophil adhesion, migration, and oxidative killing. These include the leukocyte adhesion deficiencies, actin defects and other disorders of chemotaxis, hyperimmunoglobulin E syndrome, Chédiak-Higashi Syndrome, neutrophil specific granule deficiency, chronic granulomatous disease, and myeloperoxidase deficiency. Diagnostic tests and treatment approaches are also summarized for each neutrophil disorder.
原发性中性粒细胞功能障碍是由宿主防御中关键的中性粒细胞反应受损引起的。本章总结了导致中性粒细胞黏附、迁移和氧化杀伤缺陷的遗传性中性粒细胞疾病。这些疾病包括白细胞黏附缺陷、肌动蛋白缺陷和其他趋化运动障碍、高免疫球蛋白 E 综合征、Chédiak-Higashi 综合征、中性粒细胞特异性颗粒缺乏、慢性肉芽肿病和髓过氧化物酶缺乏。还总结了每种中性粒细胞疾病的诊断测试和治疗方法。
