双侧弥散性葡萄膜黑色素细胞增生症的研究现状

Progress of bilateral diffuse uveal melanocytic proliferation

双侧弥散性葡萄膜黑色素细胞增生症(bilateral diffuse uveal melanocytic proliferation,BDUMP)是一种罕见的以双眼弥漫性葡萄膜增厚和良性黑色素细胞增生为主要特征的副肿瘤综合征。其发病机制尚不明确,好发于中老年人,可引起双眼视力逐渐丧失,眼底表现为多发性类圆形斑片状病灶,病灶周围伴橘红色多边形色素性沉着,呈现出长颈鹿斑样改变。多模式影像学技术和实验室免疫学检测可协助诊断。其预后较差,血浆置换疗法联合针对原发性恶性肿瘤的综合治疗是最有效的治疗手段。

Bilateral diffuse uveal melanocytic proliferation(BDUMP)is a rare paraneoplastic ocular syndrome characterised by diffuse uveal thickening and proliferation of benign melanocytes which leads to progressive loss of vison bilaterally.The pathogenesis of BDUMP is unclear.The typical clinical findings is a giraffe-like pattern with multiple scattered oval or round subretinal greyish patchy lesions separated by polygonal orange pigmentation.Multimodal imaging and immunologic investigations facilitate early diagnosis of BDUMP.Plasmapheresis with simultaneous treatment of the underlying malignancy is the most effective treatment.