摘要
噬血细胞淋巴组织细胞增生症(hemophagocytic lymphohistiocytosis,HLH)是儿科临床上少见但病情凶险、病死率很高的疾病,分家族性、继发性和肿瘤相关性3种类型。其病因复杂,机制尚未完全阐明。有证据表明,该病的发生与编码免疫应答功能相关的蛋白基因缺陷有关,其基本发病机制是在免疫缺陷的基础上,伴有感染、肿瘤及坏死组织细胞等抗原的诱发下,导致细胞因子的大量释放,进而激活单核巨噬细胞发生吞噬现象和多脏器损伤。HLH-2004诊疗指南是当前诊治HLH的主要方法,细胞因子的快速测定有助于HLH的早期诊断。
Hemophagocytic lymphohistiocytosis （HLH） is a rare but aggressive disease in pediatric clinical practice with a very high mortality. It is currently divided into three major categories including familial, secondary and malignancy-associated HLH with complicated and incomplete etiological basis. There are evidences to show that the primary underlying mechanism of this syndrome is due to the defect of the genes and the proteins involved in the immune responses and triggered by some causative factors such as infective pathogens, malignancies and damaged tissue, and cell debris as antigens, which results in a vast amount of production of eytokines. Over activation of the macrophages results in phagocytosis and damages of the organs and tissues. Guideline of HLH-2004 proposed by the International Society of Histiocyte is currently the practical and effective model of both diagnosis and treatment. Rapid determination of cytokines signigicantly helps early diagnosis.
出处
《临床儿科杂志》
CAS
CSCD
北大核心
2009年第8期705-708,共4页
Journal of Clinical Pediatrics
关键词
噬血细胞综合征
发病机制
细胞因子
早期诊断
治疗
hemophagocytic hemolymphohistiocytosis
pathogenesis
cytokines
early diagnosis
treatment
