Diagnosis and treatment of POEMS syndrome
10.3760/cma.j.cn113694-20230109-00014
- VernacularTitle:POEMS综合征的诊断和治疗
- Author:
Yuzhou GUAN
1
;
Jingwen NIU
Author Information
1. 中国医学科学院北京协和医院神经科，北京100730
- Keywords:
POEMS syndrome;
Diagnosis;
Treatment
- From:
Chinese Journal of Neurology
2023;56(4):448-452
- CountryChina
- Language:Chinese
-
Abstract:
POEMS syndrome is a rare paraneoplastic syndrome due to plasma cell neoplasm. It is a disorder involving multiple organs, mainly manifesting as demyelinating peripheral neuropathy. The major diagnostic criteria for the syndrome are polyradiculoneuropathy, clonal plasma cell disorder, sclerotic bone lesions, elevated vascular endothelial growth factor, and the presence of Castleman disease. Minor features include organomegaly, endocrinopathy, characteristic skin changes, papilledema, extravascular volume overload, and thrombocytosis. A good history and physical examination followed by appropriate testing-radiographic assessment of bones, measurement of vascular endothelial growth factor, bone marrow biopsy and electromyography can differentiate this syndrome from other conditions such as chronic inflammatory demyelinating polyradiculoneuropathy, other plasma disorders (including amyloidosis, myeloma, and monoclonal gammopathy of undetermined significance neuropathy). Early diagnosis and treatment could greatly improve the prognosis.
