Department of Pediatric Hematology and Oncology, University Hospital Ghent, Ghent, Belgium.
Eur Cytokine Netw. 2011 Sep;22(3):148-53. doi: 10.1684/ecn.2011.0290.
Langerhans cell histiocytosis (LCH) is a rare disorder characterized by an abnormal accumulation and/or proliferation of cells with a Langerhans cell phenotype. Although no clear cause of LCH has been identified, it has been postulated that LCH might be the consequence of an immune dysregulation, causing Langerhans cells to migrate to and accumulate at various sites. Production of cytokines and chemokines is a central feature of immune regulation. Cytokines are abundantly present within LCH lesions. We review here the potential role of cytokines and chemokines in the pathogenesis of LCH. The type, distribution, and number of different cytokines released within lesions can provide clues to the possible aetiology of LCH and, ultimately, might offer therapeutic possibilities using recombinant cytokines or antagonists for this disorder.
朗格汉斯细胞组织细胞增生症（LCH）是一种罕见的疾病，其特征是异常积聚和/或增殖具有朗格汉斯细胞表型的细胞。虽然尚未确定 LCH 的明确病因，但有人推测 LCH 可能是免疫失调的结果，导致朗格汉斯细胞迁移并积聚在各种部位。细胞因子和趋化因子的产生是免疫调节的一个核心特征。细胞因子在 LCH 病变中大量存在。在这里，我们回顾了细胞因子和趋化因子在 LCH 发病机制中的潜在作用。病变中释放的不同细胞因子的类型、分布和数量可以为 LCH 的可能病因提供线索，并最终可能为使用重组细胞因子或针对这种疾病的拮抗剂提供治疗可能性。
