Síndrome de POEMS: aspectos clínicos e as particularidades da doença esquelética

Abstract

POEMS syndrome is a rare paraneoplastic condition associated with plasma cell neoplasms, whose true incidence remains uncertain due to frequent underdiagnosis resulting from its complex clinical manifestations. The acronym POEMS corresponds to the main features of the syndrome—polyneuropathy, organomegaly, endocrinopathy, monoclonal plasma cell disorder, and skin changes—although it does not encompass the entire clinical spectrum. Its pathogenesis is not yet fully understood, limiting risk stratification to clinical phenotypes and making management particularly challenging. Consequently, diagnosis requires the integration of a detailed medical history, thorough physical examination, and specific complementary tests, particularly skeletal radiological assessment, VEGF measurement, and bone marrow evaluation. In this context, the present narrative literature review compiles the main clinical characteristics of the syndrome, with emphasis on its skeletal manifestations and imaging findings. The aim is to enhance clinical understanding and promote early detection in medical practice, thereby contributing to improved patient morbidity, quality of life and survival, given its typically chronic and prolonged course. Furthermore, this review seeks to distinguish POEMS syndrome from other conditions with similar presentations, such as chronic inflammatory demyelinating polyneuropathy (CIDP), MGUS-associated neuropathy, and lightchain amyloid neuropathy, which require distinct treatment and follow-up approaches.