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伴有淋巴结窦血管转化的POEMS综合征:一例报告

POEMS syndrome with vascular transformation of the lymph node sinuses: A case report.

作者信息

Wang Xiaoqing, Yu Xiaowen, Zhu Desheng, Zhang Sheqing, Zhou Xiajun, Liu Mingyuan, Guan Yangtai

机构信息

Department of Neurology, Changhai Hospital, Second Military Medical University, Shanghai 200433, P.R. China.

Department of Gerontology, Changhai Hospital, Second Military Medical University, Shanghai 200433, P.R. China.

出版信息

Oncol Lett. 2015 Sep;10(3):1789-1792. doi: 10.3892/ol.2015.3413. Epub 2015 Jun 23.

Abstract

POEMS syndrome is a rare multisystem disorder associated with the clinical signs of polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin changes. However, there is often a delay in the diagnosis due to a lack of overall consideration of the symptoms collectively. For this reason, POEMS syndrome is frequently mistaken for other diseases, such as chronic inflammatory demyelinating polyneuropathy. The present study reports the case of a 40-year-old female patient, who presented with a progressive lack of strength in the lower limbs and a unilateral cervical lump. The patient's enlarged cervical lymph nodes were mistaken for local hemangioma. However, subsequently POEMS syndrome with vascular transformation of the lymph node sinuses (VTS) was diagnosed. The patient received glucocorticoid treatment (20 mg prednisone acetate, daily), which is ongoing. The most recent follow-up examination revealed that the patient's strength had improved and at the time of writing the patient remained alive. The study discusses the clinical manifestations, auxiliary examinations and reason for the misdiagnosis. Hematoxylin and eosin staining and cluster of differentiation 31 immunostaining were adopted to identify the VTS. To the best of our knowledge, this is the first report of POEMS syndrome with VTS.

摘要

POEMS综合征是一种罕见的多系统疾病,与多发性神经病、器官肿大、内分泌病、单克隆丙种球蛋白病和皮肤改变等临床体征相关。然而,由于缺乏对这些症状的综合考虑,诊断往往会延迟。因此,POEMS综合征经常被误诊为其他疾病,如慢性炎症性脱髓鞘性多发性神经病。本研究报告了一例40岁女性患者,该患者出现进行性下肢无力和单侧颈部肿块。患者肿大的颈部淋巴结被误诊为局部血管瘤。然而,随后诊断为伴有淋巴结窦血管转化(VTS)的POEMS综合征。患者接受糖皮质激素治疗(每日20mg醋酸泼尼松),治疗仍在进行中。最近的随访检查显示患者的肌力有所改善,在撰写本文时患者仍然存活。该研究讨论了临床表现、辅助检查及误诊原因。采用苏木精-伊红染色和分化簇31免疫染色来识别VTS。据我们所知,这是首例伴有VTS的POEMS综合征报告。

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