Baykan Ali, Akcakus Mustafa, Deniz Kemal
Department of Pediatrics, Division of Cardiology, Erciyes University Faculty of Medicine, Kayseri, Turkey.
J Pediatr Hematol Oncol. 2005 Aug;27(8):430-1. doi: 10.1097/01.mph.0000177427.94164.df.
Hemophagocytic syndrome (HPS) is a fulminant disorder characterized pathologically by multiple-organ infiltration of hemophagocytic histiocytes in the lymphoreticular tissues. The characteristic pathologic feature is reactive histiocytic hyperplasia with leukoerythrophagocytosis in a variety of organs. This disorder occurs most often in patients in whom the immune system is compromised and has been associated with a variety of infectious agents, including viruses, bacteria, mycobacteria, spirochetes, fungi, and parasites. The authors describe a 2.5-month-old girl with rubella-associated HPS, demonstrated by postmortem liver necropsy.
噬血细胞综合征(HPS)是一种暴发性疾病,其病理特征为噬血细胞性组织细胞在淋巴网状组织中的多器官浸润。特征性病理表现为多种器官中出现伴有白细胞红细胞吞噬现象的反应性组织细胞增生。这种疾病最常发生于免疫系统受损的患者,并与多种感染因子有关,包括病毒、细菌、分枝杆菌、螺旋体、真菌和寄生虫。作者描述了一名2.5个月大患风疹相关噬血细胞综合征的女孩,经死后肝脏尸检证实。