北京大学学报(医学版) ›› 2017, Vol. 49 ›› Issue (6): 985-989. doi: 10.3969/j.issn.1671-167X.2017.06.009

• 论著 • 上一篇    下一篇

23例POEMS综合征临床特点分析

胡萍,罗樱樱,吴静,高蕾莉△   

  1. (北京大学人民医院内分泌科,北京100044)
  • 出版日期:2017-12-18 发布日期:2017-12-18
  • 通讯作者: 高蕾莉 E-mail:plum_jj@sina.com

Analysis of clinical features of 23 patients with POEMS syndrome

HU Ping, LUO Ying-ying, WU Jing, GAO Lei-li△   

  1. (Department of Endocrinology, Peking University People’s Hospital, Beijing 100044, China)
  • Online:2017-12-18 Published:2017-12-18
  • Contact: GAO Lei-li E-mail:plum_jj@sina.com

摘要: 目的:了解POEMS综合征患者合并的内分泌异常的特点,为在合并内分泌系统异常的患者中识别出POEMS综合征的高危人群提供依据。方法:以“POEMS综合征”为词检索北京大学人民医院病案数据库,共纳入23例患者。使用Epidata 3.0软件进行数据录入,SPSS 19.0统计软件进行数据分析。结果:入组患者平均年龄47岁,男女比例为1.88 ∶1,首发症状以多发神经病变(56.5%)最为多见,从首发症状至确诊的中位时间为30个月。就诊于神经内科的患者例数最多,诊断的中位时间最短。患者各项临床表现的发生率分别为多发神经病变100.0%、器官肿大82.6%、内分泌异常95.7%、浆细胞克隆性增生52.2%、皮肤改变91.3%,临床体征有视乳头水肿45.5%、血管外负荷增加43.5%、血小板升高43.5%、Castleman病17.4%。在内分泌系统异常中,泌乳素水平异常的发生率最高,为60.0%,其次分别为甲状腺轴及肾上腺轴功能异常。在内分泌系统受累的患者中,2个靶腺受累的发生率最高,占36.5%。结论:内分泌系统异常是POEMS综合征常见的临床表现,对于甲状腺、肾上腺、性腺、甲状旁腺功能异常者以及泌乳素水平或糖耐量异常者,尤其是累及2个或2个以上的内分泌系统功能异常者,应提高警惕,关注多发神经病变的症状和体征,减少POEMS综合征的误诊、漏诊,同时,对POEMS综合征患者需全面评估各个内分泌系统的异常,以使患者得以及时的诊断和治疗。

关键词:  , POEMS综合征, 内分泌系统疾病, 诊断, 鉴别

Abstract: Objective: To describe the endocrine abnormalities in patients with POEMS syndrome in order to identify more patients with POEMS syndrome among those who have endocrine dysfunctions. Methods: We searched the inpatient medical record system of Peking University People’s Hospital with “POEMS syndrome”. Finally the data from 23 patients were analyzed. Epidata 3.0 was used for data entering and SPSS 19.0 was used for statistical analysis. Results: The median age of all the 23 patients was 47 years. The ratio of male to female was 1.88 ∶1. Polyneuropathy was the most common initial symptom which accounted for 56.5% in the 23 patients. The median duration from the initial symptoms to diagnosis as POEMS syndrome was 30 months. Among all the departments, the number of confirmed cases was highest in the department of neurology. The median duration from the onset of initial symptoms (6 months) to the diagnosis made in neurology department was also significantly shorter than in other departments. Among all the 23 patients, 100.0% of them had polyneuropathy, 82.6% had organomegaly, 95.7% had endocrinopathy, 52.2% were M protein positive, 91.3% had skin changes, 45.5% had papilledema, 43.5% had extravascular volume overload, 43.5% had platelet elevation and 17.4% had Castleman disease. Among all the patients with endocrinopathy, the incidence of hyperprolactinemia was 60.0% which was the highest one followed by thyroid dysfunction and adrenal dysfunction. Among the patients who had endocrine system dysfunctions, most of them had two target endocrine glands involved (36.5%). Conclusion: Endocrinopathy is an important component of POEMS syndrome and it is of great importance to pay more attention to POEMS syndrome in patients with thyroid dysfunction, adrenal dysfunction, gonadal dysfunction, parathyroid dysfunction, hyperprolactinemia and glucose intolerance, especially in patients with two or more target gland dysfunctions. Symptoms and signs of polyneuropathy should be assessed carefully to reduce misdiagnosis of POEMS syndrome. Evaluation of the endocrine system should also be done in patients diagnosed with POEMS syndrome in order to treat the patients properly.

Key words: POEMS syndrome, Endocrine System Diseases, Diagnosis, differential

中图分类号: 

  •  
[1] 赵业, 刁小莉, 熊焰. 细胞转移技术在微量细胞液病理诊断中的应用[J]. 北京大学学报(医学版), 2026, 58(1): 208-213.
[2] 王月, 梁宇红. 繁茂型牙骨质-骨结构不良1例[J]. 北京大学学报(医学版), 2026, 58(1): 220-224.
[3] 池彦廷, 蒋鸿杰, 陈艳, 徐志秀, 李斌斌. 直接免疫荧光在口腔黏膜寻常型天疱疮诊断中的价值: 基于多指标联合分析的回顾性研究[J]. 北京大学学报(医学版), 2026, 58(1): 68-73.
[4] 梁景原, 张霞, 姚海红. 误诊为系统性硬化症的POEMS综合征1例[J]. 北京大学学报(医学版), 2025, 57(6): 1184-1187.
[5] 顾静妍, 李欣艺, 赵金霞, 穆荣. 误诊为类风湿关节炎、痛风的糖尿病致Charcot关节病1例[J]. 北京大学学报(医学版), 2025, 57(6): 1193-1197.
[6] 肖晓笛, 夏有辰, 柳剑英, 付鹏. 左侧胸锁乳突肌间血管内乳头状内皮增生1例[J]. 北京大学学报(医学版), 2025, 57(5): 1002-1004.
[7] 孙翔宇, 袁超, 周芯竹, 刁婧, 郑树国. 唾液微生态在口腔及全身疾病早期防治中的应用[J]. 北京大学学报(医学版), 2025, 57(5): 859-863.
[8] 陈钊, 邱永康, 康磊. 经典型Sweet综合征 18F-FDG PET/CT多脏器异常显像1例[J]. 北京大学学报(医学版), 2025, 57(2): 403-407.
[9] 方媛媛, 徐帆, 雷杰, 张昊, 张文宇, 孙宇, 吴宏新, 傅开元, 毛伟玉. 基于颞下颌关节紊乱病诊断标准的临床自动诊断系统的建立及验证[J]. 北京大学学报(医学版), 2025, 57(1): 192-201.
[10] 车佳璐, 刘子臣, 李琨, 张晨, 车南颖. 全自动EasyNAT核酸快速检测系统检测石蜡包埋组织诊断结核病的临床价值[J]. 北京大学学报(医学版), 2024, 56(6): 1047-1051.
[11] 陈心心, 唐哲, 乔艳春, 荣文笙. 北京市密云区4岁儿童患龋状况及其与龋活跃性检测的相关性[J]. 北京大学学报(医学版), 2024, 56(5): 833-838.
[12] 钟华, 李原, 徐丽玲, 白明欣, 苏茵. 18F-FDG PET/CT在风湿免疫病中的应用[J]. 北京大学学报(医学版), 2024, 56(5): 853-859.
[13] 李正芳,罗采南,武丽君,吴雪,孟新艳,陈晓梅,石亚妹,钟岩. 抗氨基甲酰化蛋白抗体在诊断类风湿关节炎中的应用价值[J]. 北京大学学报(医学版), 2024, 56(4): 729-734.
[14] 姚海红,杨帆,唐素玫,张霞,何菁,贾园. 系统性红斑狼疮及成人Still病合并巨噬细胞活化综合征的临床特点及诊断指标[J]. 北京大学学报(医学版), 2023, 55(6): 966-974.
[15] 熊焰,李鑫,梁丽,李东,鄢丽敏,李雪迎,邸吉廷,李挺. 甲状腺粗针穿刺活检病理诊断的准确性评估[J]. 北京大学学报(医学版), 2023, 55(2): 234-242.
Viewed
Full text


Abstract

Cited

  Shared   
  Discussed   
No Suggested Reading articles found!