以双侧视乳头水肿为主要表现的高原红细胞增多症临床分析  

Clinical analysis of high altitude polycythemia manifested as bilateral papilledema

作　　者：杨庆林[1] 李众[1] 王佳伟[1] YANG Qing-lin;LI Zhong;WANG Jia-wei(Department of Neurology,Beijing Tongren Hospital,Capital Medical University,Beijing 100730,China)

机构地区：[1]首都医科大学附属北京同仁医院神经内科,100730

出　　处：《中国现代神经疾病杂志》2019年第2期115-119,共5页Chinese Journal of Contemporary Neurology and Neurosurgery

基　　金：国家重点研发计划精准医学研究重点专项项目(项目编号:2016YFC0904502)~~

摘　　要：目的总结以双侧视乳头水肿为主要表现的高原红细胞增多症的临床特点、治疗和预后。方法回顾分析2007年3月至2017年9月共7例以双侧视乳头水肿为主要表现的高原红细胞增多症患者的临床表现、实验室检查、眼科检查、头部影像学检查、线粒体基因检测以及治疗与转归。结果 7例患者中男性6例、女性1例,平均年龄为(44.43±9.05)岁,高原居住时间为7(4,13)个月,病程为(28.43±10.05)d,发病初期血红蛋白均>210 g/L;均伴不同程度双侧视乳头水肿,视力为1只眼眼前1 m指数、13只眼矫正视力>0.10,视野缺损类型为2只眼生理盲点扩大、4只眼水平下方视野缺损、2只眼中心暗点、6只眼弥漫性视野缺损;7例头部MRI可见额顶叶或侧脑室旁点片状长T_1、长T_2异常信号影;7只眼视神经MRI可见视神经信号增高,呈轻度强化;均予抗血小板和改善微循环治疗,随访6个月视力恢复良好。结论以双侧视乳头水肿为主要表现的高原红细胞增多症易误诊为其他疾病。脱离高原环境、降低血红蛋白含量、辅以抗血小板和改善微循环治疗可以促进视力恢复。Objective To investigate the clinical features, treatment and prognosis of high altitude polycythemia mainly manifested as bilateral papilledema. Methods Clinical data of 7 patients with high altitude polycythemia mainly manifested as bilateral papilledema in our hospital from March 2007 to September 2017 were retrospectively analyzed, including clinical manifestations, laboratory results,ophthalmologic examinations, head imaging features, mitochondrial gene detection, treatment and prognosis.Results There were 6 male patients and one female patient, with mean age(44.43 ± 9.05) years old, highaltitude residence 7(4, 13) months, and duration(28.43 ± 10.05) d. All patients were detected with high concentrations of hemoglobin(> 210 g/L), and different degrees of bilateral papilledema after onset. One eye had severe vision impairment [best corrected visual acuity(CVA) was finger counting], and the CVA of other 13 eyes were > 0.10. Visual field defects included enlargement of psychological blind spot(2 eyes),lower altitudinal visual field defect(4 eyes), central scotoma(2 eyes) and diffuse visual field defect(6 eyes).Brain MRI of 7 cases showed dot-like and patchy long T1 and long T2 signals in frontal lobe, parietal lobeand paraventricular region. Optic nerve MRI showed hyperintense signal and slight enhancement in 7 eyes and no abnormality in other 7 eyes. All patients were treated by antiplatelet therapy and microcirculation improvement. Best CVA of all patients were remarkably improved within follow up of 6 months.Conclusions High altitude polycythemia mainly manifested as bilateral papilledema was easily misdiagnosed as other diseases. Moving away from plateau, decreasing hemoglobin, anti-platelet therapy and microcirculation improvement may play an important role in the favorable prognosis.

关 键 词：红细胞增多症 视乳头水肿 高海拔 

分 类 号：R555.1[医药卫生—血液循环系统疾病]