溶骨症发病机制的研究进展

Research Progress in Pathogenesis of Osteolytic Disease

溶骨症又称为Gorham综合征,是一种病因不明,主要侵犯骨骼罕见的综合征,主要临床表现为慢性自发性骨溶解,并可因此引发一系列临床症状。病理改变主要为脉管异常增殖和骨溶解两方面。前者可因非瘤性毛细血管和淋巴管异常增殖所致,后者系破骨细胞数目增多和活性增强或成骨细胞退行性改变。两者之间可能存在某种内在联系而导致互相促进。迄今为止,国内外报道共200余例,但大多数报道针对该病的诊断及临床疗效,而缺乏对该病发病机制的陈述。

Osteolytic disease,also known as Gorham syndrome,is a rare syndrome of unknown etiology that mainly invades bones.The main clinical manifestation is chronic spontaneous osteolysis,which can lead to a series of clinical symptoms.Pathological changes are mainly abnormal vascular proliferation and osteolysis.The former can be caused by abnormal proliferation of the capillaries and lymphatic vessels;the latter is due to the increased number and activity of osteoclasts or degenerative changes of osteoblasts.There may be an intrinsic link between the two,which leads to mutual promotion.Up to now,more than 200 cases have been reported in domestic and foreign literature,however,most of them were aimed at the diagnosis and clinical efficacy of the disease,while the statements about the pathogenesis are still in lack.