An Hypothesis Langerhans cell histiocytosis: The failure of the immune system to switch from an innate to an adaptive mode
An Hypothesis Langerhans cell histiocytosis: The failure of the immune system to switch from an innate to an adaptive mode
复制标题
朗格汉斯细胞组织细胞增多症的假设：免疫系统未能从先天模式切换到适应性模式
作者：
C. Nezelof;F. Basset
正如最近发表的社论，朗格汉斯细胞组织细胞增多症 (LCH) 正在遭受关于其发病机制的身份危机 [1]。虽然所有人都同意朗格汉斯细胞 (LC) 的增殖和激活是该疾病的一致特征，但这种细胞反应是否代表反应性过程或肿瘤过程的问题仍然悬而未决 [2]。最近证实的嗜酸性肉芽肿骨染色体异常[3]、遗传失衡[4]、某些（LC）增殖的克隆特征[5]以及某些类型的抗有丝分裂药物的临床有效性为肿瘤理论提供了支持。然而，我们认为 LCH 是一种肿瘤性疾病的概念作为一个统一的假设，基于以下论点是值得商榷的。
As recently editorialized, Langerhans cell histiocytosis (LCH) is suffering from an identity crisis [1] regarding its pathogenesis. While all agree that the proliferation and activation of Langerhans cells (LCs) are consistent features of the disease, the issue remains unsettled whether this cellular response represents a reactive or neoplastic process [2]. The recent demonstration of chromosomal abnormalities in eosinophilic granuloma bone [3], genetic imbalances [4] the clonal character of certain (LCs) proliferations [5], and the clinical effectiveness of some types of antimitotic drugs have provided support for the neoplastic theory. However, we suggest that the concept that LCH is a neoplastic condition as a unifying hypothesis, is open to question on the basis of the following arguments.