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

作　　者：蔡志鹏[1] 张红[1] 袁敏立[1] 苏菲 Cai Zhipeng;Zhang Hong;Yuan Minli;Su Fei(Eye Hospital,China Academy of Chinese Medical Sciences,Beijing 100040,China)

机构地区：[1]中国中医科学院眼科医院,北京100040

出　　处：《国际眼科纵览》2021年第2期158-164,共7页International Review of Ophthalmology

基　　金：北京中医药薪火传承“3+3”工程。

摘　　要：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.

关 键 词：双侧弥散性葡萄膜黑色素细胞增生症 副肿瘤综合征 葡萄膜疾病/免疫学 

分 类 号：R739.7[医药卫生—肿瘤]