Popko Katarzyna, Górska Elżbieta, Wołowiec Magdalena, Malinowska Iwona
Department of Laboratory Diagnostics and Clinical Immunology of Developmental Age.
Department of Pediatrics, Hematology and Oncology, Medical University of Warsaw, Warsaw, Poland.
J Pediatr Hematol Oncol. 2019 Jul;41(5):e277-e283. doi: 10.1097/MPH.0000000000001514.
Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening disease associated with immune system hyperactivation and the appearance of serious systemic disturbances. The purpose of this study was an assessment of natural killer (NK) cell disturbances in a group of children with clinical signs of HLH. A total of 43 children with HLH and 17 healthy children were enrolled in the study. NK phenotyping, intracellular perforin staining, and cytotoxicity tests were performed by using the flow cytometry method. HLH patients were divided into 6 HLH types: 9% infection-related HLH; 7% malignancy-related HLH; 21% macrophage activating syndrome; 12% familial hemophagocytic lymphohistiocytosis; 2% X-linked lymphoproliferative syndrome; and 49% as HLH of unknown background. A positive correlation was observed between cytotoxicity and NK cells in children with HLH (P=0.01). In all HLH groups, the percentage of NK cells was significantly lower than in the control population. The spontaneous cytotoxicity was significantly lower in HLH patients. The results presented in this study indicate the importance of impaired function and the number of NK cells in the pathogenesis of HLH. Nonetheless, the background of disturbances seems to be different in various cases.
噬血细胞性淋巴组织细胞增生症（HLH）是一种危及生命的疾病，与免疫系统过度激活及严重全身紊乱的出现相关。本研究的目的是评估一组有HLH临床症状的儿童的自然杀伤（NK）细胞紊乱情况。共有43例HLH患儿和17例健康儿童纳入本研究。采用流式细胞术进行NK细胞表型分析、细胞内穿孔素染色及细胞毒性试验。HLH患者分为6种HLH类型：9%为感染相关HLH；7%为恶性肿瘤相关HLH；21%为巨噬细胞活化综合征；12%为家族性噬血细胞性淋巴组织细胞增生症；2%为X连锁淋巴增殖综合征；49%为背景不明的HLH。在HLH患儿中观察到细胞毒性与NK细胞之间存在正相关（P = 0.01）。在所有HLH组中，NK细胞百分比均显著低于对照组人群。HLH患者的自发细胞毒性显著降低。本研究结果表明NK细胞功能受损及数量在HLH发病机制中具有重要意义。尽管如此，不同病例中紊乱的背景似乎有所不同。
