Department of Pediatrics, Division of Rheumatology, Washington University School of Medicine, St. Louis, MO, USA.
Department of Pediatrics, Division of Rheumatology, University of Alabama at Birmingham Heersink School of Medicine, Birmingham, AL, USA.
Adv Exp Med Biol. 2024;1448:145-159. doi: 10.1007/978-3-031-59815-9_11.
Natural killer (NK) cells are innate immune lymphocytes that rapidly produce cytokines upon activation and kill target cells. NK cells have been of particular interest in primary hemophagocytic lymphohistiocytosis (pHLH) since all of the genetic defects associated with this disorder cause diminished cytotoxic capacity of NK cells and T lymphocytes, and assays of NK cell killing are used clinically for the diagnosis of HLH. Herein, we review human NK cell biology and the significance of alterations in NK cell function in the diagnosis and pathogenesis of HLH.
自然杀伤 (NK) 细胞是先天免疫淋巴细胞,在激活后迅速产生细胞因子并杀死靶细胞。由于与这种疾病相关的所有遗传缺陷都会导致 NK 细胞和 T 淋巴细胞的细胞毒性能力降低,因此 NK 细胞杀伤的检测临床上用于 HLH 的诊断,因此 NK 细胞在原发性噬血细胞性淋巴组织细胞增多症 (pHLH) 中特别受到关注。本文综述了人类 NK 细胞生物学以及 NK 细胞功能改变在 HLH 的诊断和发病机制中的意义。