Kannourakis G, Abbas A
L.A.R.C.H. Cancer Research Unit, Department of Haematology and Oncology, Royal Children's Hospital, Parkville, Victoria, Australia.
Br J Cancer Suppl. 1994 Sep;23:S37-40.
Langerhans cell histiocytosis (LCH) is characterised by an accumulation of cells ('LCH cells') with the same phenotypic features as normal Langerhans cells found in skin and other organs. The pathogenesis of LCH is unknown but there is increasing evidence to implicate the involvement of lymphokines and proinflammatory cytokines in the tissue damage seen in this disorder. Apart from histiocytes, the lesions contain giant cells, macrophages, neutrophils, eosinophils, lymphocytes, plasma cells and occasional mast cells that are the hallmark of an inflammatory process. The role of cytokines in the recruitment of haemopoietic cells within inflammatory lesions has only recently been recognised. In this article, we review the possible role of cytokines in the pathogenesis of LCH, and provide an overview of the methods currently used to detect and quantitate them. An appreciation of the type, distribution and amount of different cytokines released within lesions can provide clues to the possible aetiology of LCH. Using immunoassays, in situ hybridisation and RT-PCR, increased amounts of IL-1, IL-3, IL-4, IL-8, GM-CSF, TNF alpha, TGF beta and LIF have been demonstrated in LCH lesions. Lymphocytes constitutively produce GM-CSF and IL-3 and, to a lesser degree, IL-1, IL-4 and LIF whilst histiocytes produce TNF alpha, IL-1 beta and GM-CSF.
朗格汉斯细胞组织细胞增多症（LCH）的特征是出现一群细胞（“LCH细胞”），这些细胞具有与皮肤和其他器官中正常朗格汉斯细胞相同的表型特征。LCH的发病机制尚不清楚，但越来越多的证据表明，淋巴因子和促炎细胞因子参与了该疾病中所见的组织损伤。除组织细胞外，病变还包含巨细胞、巨噬细胞、中性粒细胞、嗜酸性粒细胞、淋巴细胞、浆细胞以及偶尔出现的肥大细胞，这些都是炎症过程的标志。细胞因子在炎症病变中造血细胞募集方面的作用直到最近才被认识到。在本文中，我们综述了细胞因子在LCH发病机制中的可能作用，并概述了目前用于检测和定量细胞因子的方法。了解病变中释放的不同细胞因子的类型、分布和数量可为LCH的可能病因提供线索。通过免疫测定、原位杂交和逆转录聚合酶链反应，已证实在LCH病变中白细胞介素-1（IL-1）、IL-3、IL-4、IL-8、粒细胞-巨噬细胞集落刺激因子（GM-CSF）、肿瘤坏死因子α（TNFα）、转化生长因子β（TGFβ）和白血病抑制因子（LIF）的含量增加。淋巴细胞持续产生GM-CSF和IL-3，在较小程度上产生IL-1、IL-4和LIF，而组织细胞产生TNFα、IL-1β和GM-CSF。
