Chinese General Practice ›› 2016, Vol. 19 ›› Issue (35): 4385-4387.DOI: 10.3969/j.issn.1007-9572.2016.35.019

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Clinical Symptoms of POEMS Syndrome:17 Cases Report and Literature Review

  

  1. Department of Endocrinology,the First Affiliated Hospital of Nanchang University,Nanchang 330006,China Corresponding author:XU Ji-xiong,Department of Endocrinology,the First Affiliated Hospital of Nanchang University,Nanchang 330006,China;E-mail:xujixiong@163.com
  • Published:2016-12-15 Online:2026-01-28

POEMS综合征17例临床症状分析并文献复习

  

  1. 330006江西省南昌市,南昌大学附属第一医院内分泌科 通信作者:徐积兄,330006江西省南昌市,南昌大学附属第一医院内分泌科;E-mail:xujixiong@163.com

Abstract: Objective To analyze the clinical features of POEMS syndrome for improving early diagnostic rate of POEMS syndrome and to reduce misdiagnosis.Methods 17 patients with POEMS syndrome admitted to the First Affiliated Hospital of Nanchang University from 2008 to 2015 were selected and their clinical features were analyzed,and related literatures were also reviewed.Results The initial symptoms of 10 patients were numbness,weakness and pain,and 7 patients were bloating.16 patients had polyneuropathy,15 patients electromyography showed slowed motor nerve conduction,normal proximal amplitude and obviously lowered distal amplitude;increased protein could be found through lumbar puncture examination of 5 patients.14 patients had swelled organs,and swelled lymphadenopathy biopsy presented chronic inflammatory reaction or Castleman’ s disease of plasmocyte types.14 patients had endocrine abnormality with manifestation of gonad axis,thyroid axis,hypothalamic pituit-aryadvenal axis and glucose metabolism.4 patients had positive M-protein;12 patients underwent bone marrow aspiration,and the proportion of plasmocyte in bone marrow smears increased with 4 cases.16 patients had skin changes,manifested by skin blackening and redness,tough skin hardening,and pale fingernails.16 patients had increased water-loading with peripheral edema,pleural effusion,ascites and hydropericardium.3 patients had sclerotic bone lesion.ConclusionPOEMS syndrome has complicated clinical features with high misdiagnosis rate.Clinical doctors should raise awareness of the disease and perform careful examinations for suspected patients so as to avoid misdiagnosis.

Key words: POEMS syndrome, Signs and symptoms, Diagnosis

摘要: 目的分析POEMS综合征的临床特征,提高POEMS综合征早期诊断率,减少误诊。方法 选取2008—2015年南昌大学附属第一医院治疗的POEMS综合征患者17例,分析其临床特征,并复习相关文献。结果 10例患者首发症状为肢体麻木、乏力、疼痛,7例患者首发症状为腹胀。16例患者出现多发性神经病变,15例患者肌电图检查表现为多发性运动神经传导速度减慢,近端波幅正常,远端波幅明显降低;5例患者行腰椎穿刺检查发现脑脊液蛋白水平增高。14例患者出现脏器肿大,肿大淋巴结活检示慢性炎性反应或浆细胞型Castleman’s病。14例患者有内分泌异常,表现为性腺轴、甲状腺轴、肾上腺轴和糖代谢异常。血清M蛋白阳性4例;12例患者行骨髓穿刺,其中骨髓细胞涂片浆细胞比例增加4例。16例患者皮肤改变,表现为皮肤发黑、发红,皮肤粗糙变硬,手掌、指甲苍白。16例患者水负荷增多,表现为外周水肿、胸腔积液、腹腔积液、心包积液。3例患者有硬化性骨改变。结论 POEMS综合征临床表现复杂,误诊率高,临床医师应加深认识,对疑诊患者进行细致检查以避免误诊。

关键词: POEMS综合征, 体征和症状, 诊断