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最初诊断为血管外容量负荷增加的POEMS综合征的临床特征:一项单中心回顾性研究

[Clinical characteristics of POEMS syndrome initially diagnosed as increased extravascular volume load: a single-center retrospective study].

作者信息

Zhang T, Zhang J, Shen X X, Jin Y Y, Zhang R, Li J Y, Chen L J

机构信息

Department of Hematology, the First Affiliated Hospital of Nanjing Medical University, Jiangsu Province Hospital, Nanjing 210029, China.

出版信息

Zhonghua Xue Ye Xue Za Zhi. 2024 Jul 14;45(7):694-697. doi: 10.3760/cma.j.cn121090-20231117-00264.

Abstract

POEMS syndrome is a rare plasma cell dysplasia. Its clinical manifestations include polyneuropathy, monoclonal protein, increased extravascular volume load, endocrinopathy, organomegaly and skin changes. The complex and atypical symptoms at presentation make early diagnosis challenging due to multiple system involvement. Peripheral neuropathy, limb numbness, is the most common initial symptom of this disease. However, case reports of increased extravascular volume load are rare. This article collected and analyzed the clinical data of two groups of patients with different initial symptoms (increased extravascular volume load and limb numbness). The clinical characteristics and treatment responses were summarized.

摘要

POEMS综合征是一种罕见的浆细胞发育异常疾病。其临床表现包括多发性神经病、单克隆蛋白、血管外容量负荷增加、内分泌病、器官肿大和皮肤改变。由于多系统受累,该疾病呈现出的复杂且不典型症状使得早期诊断具有挑战性。周围神经病变、肢体麻木是该疾病最常见的初始症状。然而,血管外容量负荷增加的病例报告较为罕见。本文收集并分析了两组具有不同初始症状(血管外容量负荷增加和肢体麻木)患者的临床资料。总结了其临床特征及治疗反应。

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本文引用的文献

1
Efficacy and Safety of Regimens Used for the Treatment of POEMS Syndrome- A Systematic Review.
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