Pathognomonic serum cytokine profiles identify life-threatening langerhans cell histiocytosis
Pathognomonic serum cytokine profiles identify life-threatening langerhans cell histiocytosis
复制标题
特征性血清细胞因子谱可识别危及生命的朗格汉斯细胞组织细胞增多症
DOI：
10.1111/bjh.13970
复制
发表时间：
2017
期刊：
影响因子：
--
通讯作者：
Yachie A
中科院分区：
文献类型：
--
作者：
Ikawa Y;Nishimura R;Araki R;Noguchi K;Muraoka M;Fukuda M;Fujiki T;Kuroda R;Mase S;Maeba H;Nomura K;Yachie A
朗格汉斯细胞组织细胞增生症(LCH)是一种罕见的表皮抗原提呈细胞增生性疾病，如树突状细胞(DC)(Badian-Very等，2010)。这种疾病表现出广泛的临床行为，从温和的自限形式到与高死亡风险相关的侵略性形式。为了改善潜在致命性LCH病例的预后，对发生危及生命的LCH相关吞血细胞综合征(LCH-HPS)的重症患者的识别至关重要(Favara等人，2002年)。基于疾病定位对LCH患者进行分层已被证明在确定预后和计划治疗方面很有用，例如肿瘤涉及单个器官系统(SS)、多器官系统(MS)还是危险器官(RO)，如骨髓、肺、肝和脾(Gadner等人，2008)。一些研究表明，有RO受累或发烧的MS-LCH病例被认为有可能发展为致命性并发症，如LCH-HPS(Favara等人，2002年)。然而，根据我们的病例，即使在发热或C反应蛋白(CRP)水平升高的MS-LCH病例中，疾病活动性也不同。因此，除了通过疾病定位进行分层外，为了明确地识别致命患者，还需要一种新的评估方法。
Langerhans cell histiocytosis (LCH) is a rare proliferative disorder of epidermal antigen-presenting cells, such as dendritic cells (DCs)(Badalian-Very et al, 2010). The disease shows a broad spectrum of clinical behaviours, from mild, self-limiting forms to aggressive forms associated with a high risk of mortality. To improve outcomes for potentially lethal LCH cases, the identification of severe patients developing life-threatening LCH-associated haemophagocytic syndrome (LCH-HPS) is crucially important (Favara et al, 2002).Stratifying LCH patients based on disease localization has proven useful in determining prognosis and planning therapy, such as whether the tumour involves a single organ system (SS), multiple organ systems (MS) or risk organs (RO), such as bone marrow, lung, liver and spleen (Gadner et al, 2008). Some studies have indicated that MS-LCH cases with RO involvement or fever are recognized as having the potential to develop lethal complications, such as LCH-HPS (Favara et al, 2002). However, according to our cases, disease activity varies even in MS-LCH cases with fever or elevated levels of C-reactive protein (CRP). Therefore, to definitively identify lethal patients, a novel evaluation methodology is warranted in addition to stratification by disease localization.