朗格汉斯细胞组织细胞增生症神经变性病研究进展

Research progress in neurodegenerative langerhans cell histiocytosis

朗格汉斯细胞组织细胞增生症(LCH)是一种罕见的以CD1a+CD207+树突状细胞浸润为特征的髓源性肿瘤疾病, 由于受累器官的部位和多少的不同临床表现差异很大。神经变性病(ND)是LCH中枢神经系统受累的表现之一。LCH-ND发病机制不清, 临床上主要以神经功能学障碍和进行性发展的影像学改变为特征。目前LCH-ND主要的治疗包括静脉注射免疫球蛋白、化疗及靶向治疗等。早期治疗和积极干预可能是延迟LCH-ND进展、稳定中枢神经系统功能和提高生活质量的关键。现就LCH-ND的发病机制、临床表现、诊断、治疗及临床评估作简要综述。

Langerhans cell histiocytosis(LCH)is a rare myeloid tumor disease,which is characterized by CD1a+CD207+dendritic cell proliferation.The clinical manifestations of LCH vary greatly due to the different locations and different involved organs.Among them,neurodegenerative disease(ND)is one of the manifestations of central nervous system involvement in LCH.The pathogenesis of LCH-ND is unclear and it is mainly characterized by neurological disorders and progressive imaging changes.Due to its unclear etiologies and long progress of LCH-ND,the treatment of LCH-ND remains very challenging.Presently,the main modalities of treatment include intravenous immunoglobulin,chemotherapy and targeted therapy.Early treatment and timely intervention may be the key to halt the progression of LCH-ND,to stabilize the central nervous system function and to improve the quality of life.The pathogenesis,clinical manifestations,diagnosis,treatment and clinical evaluation of LCH-ND are briefly reviewed.