淋巴管对骨代谢的影响——从戈勒姆综合征的研究中理解淋巴管的新机能

Effect of lymphatic vessels on Bone metabolism——understanding the new function of lymphatics from the study of Gorham-Stout Syndrome

淋巴管是淋巴系统的重要组成部分,担负着维持组织液和血液平衡,免疫调节等重要机能.最近的研究表明淋巴管在骨代谢中发挥着重要作用.戈勒姆综合征(Gorham-Stout Syndrome,GSS)又被称为骨溶解症,是一种伴随着骨内淋巴管(或血管)和纤维组织增生,受累骨渐进性被溶解,吸收,消失为特征的极为罕见的慢性疾病,其发病机制至今尚不清楚.戈勒姆综合征诊断主要依据影像学和病理学检查.组织切片检测显示早期多见骨质内淋巴管瘤样或血管瘤的非肿瘤组织增生,晚期主要表现为髓腔内纤维组织增生.近年来随着新的淋巴内皮细胞标记物的发现,戈勒姆综合征患者受累骨中被证实主要是淋巴管而不是血管.近年来对该病的研究进一步加深了对淋巴管和骨代谢中功能的理解,为戈勒姆综合征治疗提供基础和靶点.本文对该病的病理和组织学特征、诊断治疗等进行了回顾,着重对最近关于淋巴管在戈勒姆综合征中作用机制的研究进展进行概述.

Lymphatic vessels are important parts of the lymphatic system,which take on important responsibility of maintaining balance of tissue fluid and blood and regulating humoral immune,and so on.Recently it has been shown that lymphatic vessels play an important role in bone metabolism.Gorham Stout syndrome(GSS),also known as vanishing bone disease,is a rare chronic disease characterized by progressive dissolution,absorption,and disappearance of the affected bone,along with the proliferation of lymphatic vessels(or blood vessels)and fibrous tissue in the bone.However,the pathogenesis of GSS is still unclear.The diagnosis of GSS was mainly based on imaging and pathological examination.The examination of tissue section showed that the hyperplasia of osseous lymphangioma or hemangioma-like non-tumor tissue was common in the early stage of GSS,and in intramedullary fibrous tissue hyperplasia was main manifestation in the late stage.With the discovery of noval markers of lymphatic endothelial cell(LEC),lymphatics rather than blood vessels has been determined in the samples of GSS patients.In recent studies on GSS,it was found that lymphatics play an important role in bone metabolism,which not only gets deep understanding the function of lymphatics in human body,but also provides the clues and targets for GSS treatment.Besides reviewing the pathological and histological features,diagnosis and treatment for GSS,this article emphatically surmmarires the recent research progress of the mechanism of lymphatics in GSS.