POEMS syndrome
POEMS syndrome
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DOI：
10.1007/978-3-319-25586-6_10
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发表时间：
2020-02
期刊：
影响因子：
--
通讯作者：
MP Lunn
中科院分区：
文献类型：
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作者：
MP Lunn
POEMS综合征是一种罕见的副肿瘤疾病，由于潜在的浆细胞疾病，表现为脱髓鞘周围神经病变。该综合征最常与骨硬化性骨病变和高血管内皮生长因子相关。如果不考虑这种令人着迷的综合征的诊断，其他症状和体征似乎是无关的。如果没有适当的治疗，POEMS综合征是进行性的，致残的，并危及生命。当骨髓瘤治疗中心的药物被使用时，预后很好。
POEMS syndrome is a rare paraneoplastic condition due to an underlying plasma cell disorder that presents with a demyelinating peripheral neuropathy. The syndrome is most often associated with osteosclerotic bone lesions and high vascular endothelial growth factor. The other symptoms and signs would appear to be unrelated if one does not consider the diagnosis of this fascinating syndrome. Without adequate treatment, POEMS syndrome is progressive, disabling, and life-threatening. With treatment the prognosis is excellent when drugs from a myeloma armamentarium are employed.