Hicks John, Flaitz Catherine M
Surgical and Ultrastructural Pathology, Texas Children's Hospital, Houston 77030-2313, USA.
Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 2005 Aug;100(2 Suppl):S42-66. doi: 10.1016/j.tripleo.2005.06.016.
Langerhans cell histiocytosis (LCH) commonly involves the oral and maxillofacial region, and comes to the attention of dental practitioners when a patient presents with orofacial pain and a bony or soft tissue lesion. This is a relatively rare entity, which has made it difficult to investigate the clinical, biologic, and molecular aspects of the disease. Treatment protocols are not well defined, particularly in adults. During the past decade, the Histiocyte Society has formulated various LCH categories, based on risk stratification, and treatment protocols for the pediatric population. Adult trials are currently available through the Histiocyte Society. Although there has been considerable controversy, the neoplastic nature of LCH has been established by demonstrating clonality. LCH symptoms and the development and persistence of LCH lesions have been ascribed to a "chemokine/cytokine storm" due to autocrine and paracrine mechanisms. Discovery of biologic, cytogenetic, and molecular abnormalities in LCH have already affected treatment by providing novel therapeutic targets.
朗格汉斯细胞组织细胞增多症（LCH）常累及口腔颌面部，当患者出现口面部疼痛以及骨或软组织病变时，会引起牙科医生的注意。这是一种相对罕见的疾病，使得对该疾病的临床、生物学和分子方面进行研究变得困难。治疗方案尚未明确界定，尤其是在成人患者中。在过去十年中，组织细胞协会根据风险分层制定了各种LCH分类以及针对儿童群体的治疗方案。目前可通过组织细胞协会获取成人试验信息。尽管存在相当大的争议，但通过证明克隆性已确定LCH的肿瘤性质。由于自分泌和旁分泌机制，LCH症状以及LCH病变的发生和持续存在归因于“趋化因子/细胞因子风暴”。LCH中生物学、细胞遗传学和分子异常的发现已经通过提供新的治疗靶点影响了治疗。
