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。

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.