Progress in understanding the pathogenesis of Langerhans cell histiocytosis: back to Histiocytosis X?
Progress in understanding the pathogenesis of Langerhans cell histiocytosis: back to Histiocytosis X?
复制标题
DOI：
10.1111/bjh.13247
复制
发表时间：
2015-04
影响因子：
6.5
通讯作者：
Allen CE
中科院分区：
文献类型：
--
作者：
Berres ML;Merad M;Allen CE
朗格汉斯细胞组织细胞增生症（LCH）是最常见的组织细胞疾病，其特征是CD 1A +/CD 207+单核吞噬细胞在肉芽肿性病变内积聚，几乎可以影响所有器官系统。历史上，LCH被认为是由转化或病理激活的表皮树突状细胞（称为朗格汉斯细胞）引起的。然而，新的证据支持一个模型，其中LCH发生的结果是一个误导的分化程序的髓样树突状细胞前体。遗传、分子和功能数据表明，在髓样分化的关键阶段，ERK信号通路的激活是LCH病理学的基本和普遍驱动因素。基于这些发现，我们建议将LCH重新定义为炎性髓样瘤。对LCH发病机制的进一步了解将提供机会，通过改善风险分层、靶向治疗和基于特定分子特征和病理性骨髓细胞起源的治疗反应评估来优化和个性化治疗。
Langerhans cell histiocytosis (LCH), the most common histiocytic disorder, is characterized by the accumulation of CD1A+/CD207+ mononuclear phagocytes within granulomatous lesions that can affect nearly all organ systems. Historically, LCH has been presumed to arise from transformed or pathologically activated epidermal dendritic cells called Langerhans cells. However, new evidence supports a model in which LCH occurs as a consequence of a misguided differentiation programme of myeloid dendritic cell precursors. Genetic, molecular and functional data implicate activation of the ERK signalling pathway at critical stages in myeloid differentiation as an essential and universal driver of LCH pathology. Based on these findings, we propose that LCH should be re-defined as an inflammatory myeloid neoplasia. Increased understanding of LCH pathogenesis will provide opportunities to optimize and personalize therapy through improved risk-stratification, targeted therapy and assessment of therapy response based on specific molecular features and origin of the pathological myeloid cells.
登录
查看更多内容
DOI：
10.4049/jimmunol.0902336
发表时间：
2010-04-15
期刊：
Journal of immunology (Baltimore, Md. : 1950)
影响因子：
--
作者：
Allen CE;Li L;Peters TL;Leung HC;Yu A;Man TK;Gurusiddappa S;Phillips MT;Hicks MJ;Gaikwad A;Merad M;McClain KL
通讯作者：
McClain KL
DOI：
10.1084/jem.20130977
发表时间：
2014-04-07
期刊：
The Journal of experimental medicine
影响因子：
--
作者：
Berres ML;Lim KP;Peters T;Price J;Takizawa H;Salmon H;Idoyaga J;Ruzo A;Lupo PJ;Hicks MJ;Shih A;Simko SJ;Abhyankar H;Chakraborty R;Leboeuf M;Beltrão M;Lira SA;Heym KM;Bigley V;Collin M;Manz MG;McClain K;Merad M;Allen CE
通讯作者：
Allen CE
影响因子：
20.3
作者：
Badalian-Very, Gayane;Vergilio, Jo-Anne;Rollins, Barrett J.
通讯作者：
Rollins, Barrett J.
影响因子：
20.3
作者：
Gadner, Helmut;Minkov, Milen;Ladisch, Stephan
通讯作者：
Ladisch, Stephan
影响因子：
3.2
作者：
Abraham, Allistair;Alsultan, Abdulrahman;Campbell, Patrick K.
通讯作者：
Campbell, Patrick K.