Laboratory parameters identify familial haemophagocytic lymphohistiocytosis from other forms of paediatric haemophagocytosis
Laboratory parameters identify familial haemophagocytic lymphohistiocytosis from other forms of paediatric haemophagocytosis
复制标题
DOI：
10.1111/bjh.13461
复制
发表时间：
2015-08-01
影响因子：
6.5
通讯作者：
Heike, Toshio
中科院分区：
文献类型：
--
作者：
Yasumi, Takahiro;Hori, Masayuki;Heike, Toshio
嗜血球性淋巴组织细胞增多症（HLH）是一种危及生命的免疫失调综合征，根据潜在的病因分为原发性和继发性。这两组推荐的治疗策略有很大不同；然而，人们认为不可能用传统的实验室测试来预测HLH的潜在原因。最近的研究表明，血清中可溶性白细胞介素-2受体（sIL2R）和铁蛋白水平可用于区分某些类型的HLH。本研究报告了常见实验室参数的组合，如外周血白细胞群中总淋巴细胞的百分比，血清乳酸脱氢酶水平和sIL2R/铁蛋白比值，可用于识别家族性嗜血淋巴组织细胞增多症患者，并可用于鉴别疾病早期儿科HLH的潜在病因。这些发现表明，HLH的发病机制在先天免疫和适应性免疫方面存在很大差异，这取决于病因，并可能为揭示该综合征背后复杂的病理生理学提供新的途径。
Haemophagocytic lymphohistiocytosis (HLH) is a life-threatening syndrome of immune dysregulation and is classified as primary or secondary according to the underlying aetiology. The treatment strategies recommended for these two groups differ substantially; however, it is thought to be impossible to predict the underlying causes of HLH using conventional laboratory tests. Recent studies show that serum levels of soluble interleukin-2 receptor (sIL2R) and ferritin are useful for differentiating some forms of HLH. The present study reports that combinations of common laboratory parameters, such as the percentage of total lymphocytes within the peripheral blood leucocyte population, serum levels of lactate dehydrogenase and the sIL2R/ferritin ratio, are useful for identifying patients with familial haemophagocytic lymphohistiocytosis and for differentiating the underlying aetiology of paediatric HLH during the early course of the disease. These findings suggest that the pathogenesis of HLH differs greatly in terms of innate and adaptive immunity depending on the aetiology and may provide a new approach to unravelling the complex pathophysiology underlying this syndrome.