POEMS neuropathy: optimising diagnosis and management
POEMS neuropathy: optimising diagnosis and management
复制标题
POEMS 神经病：优化诊断和管理
作者：
S. Keddie;S. D’Sa;D. Foldes;A. Carr;M. Reilly;M. Lunn
POEMS综合征是一种罕见的致残性自身炎症性疾病，其特征是典型的周围神经病变和单克隆浆细胞疾病的存在。首字母缩略词“POEMS”代表了疾病的复杂和多系统特征，包括多发性神经病、器官肿大、内分泌病、单克隆浆细胞疾病和皮肤病。POEMS的诊断是一个重大的挑战，因为临床表现的异质性和变化的POEMS功能。患者经常被误诊为炎性神经病的另一个原因，并接受一种或多种无效的免疫调节药物，导致诊断延迟和进一步的临床恶化之前的诊断。伦敦大学学院医院是欧洲报告的最大的POEMS队列之一，并设有多专科诊所，以协助诊断，治疗和持续支持。本文回顾了POEMS综合征的典型特征，并结合临床病例介绍了POEMS综合征常见的诊断难题。我们为临床医生提供了一个调查指南时，考虑POEMS作为诊断，并提出了一个治疗算法，中心的网站和程度的单克隆细胞增殖。
POEMS syndrome is a rare and disabling autoinflammatory condition characterised by a typical peripheral neuropathy and the presence of a monoclonal plasma cell disorder. The acronym ‘POEMS’ represents the complex and multisystem features of the disease, including polyneuropathy, organomegaly, endocrinopathy, a monoclonal plasma cell disorder and skin disease. The diagnosis of POEMS is a significant challenge because of the heterogeneity of clinical presentations and variation of POEMS features. Patients are often misdiagnosed with another cause of inflammatory neuropathy and receive one or more ineffective immunomodulatory medications, resulting in delayed diagnosis and further clinical deterioration before a diagnosis is made. University College London Hospitals sees one of the largest reported POEMS cohorts in Europe, and runs a multispecialist clinic to assist with diagnosis, treatment and ongoing support. This review draws upon our experience to present the typical features of POEMS syndrome and highlight diagnostic conundrums commonly experienced, supplemented with clinical cases. We provide an investigative guide for clinicians when considering POEMS as the diagnosis, and propose a treatment algorithm that centres on the site and degree of monoclonal cell proliferation.
影响因子：
20.3
作者：
Dispenzieri, A;Kyle, RA;Gertz, MA
通讯作者：
Gertz, MA
影响因子：
20.3
作者：
Dao, Linda N.;Hanson, Curtis A.;Hoyer, James D.
通讯作者：
Hoyer, James D.