Síndrome POEMS: revisão de literatura sobre esta doença rara e de apresentação diversa

Abstract

POEMS syndrome is a rare condition, with an estimated incidence of 0.3 cases per 100,000 individuals, more common in males and older adults. Its pathogenesis is not well understood and is associated with a monoclonal plasma cell dyscrasia. The acronym, described by Bardwick in 1980, refers to most of its manifestations: polyneuropathy, organomegaly, endocrinopathy, monoclonal component, and skin changes. Diagnosis is challenging, requiring the presence of two mandatory criteria (polyneuropathy and monoclonal component) and at least one major and one minor criterion. The syndrome has a varied presentation with well-established diagnostic criteria and may be underdiagnosed when this hypothesis is not considered. Stratification is limited to phenotype, with variable frequency of clinical and laboratory manifestations. Therapy should be risk-adapted to minimize toxicity and aimed at eradicating the plasma cell disorder, similar to therapies used in multiple myeloma and amyloidosis. An appropriate therapeutic approach is essential to prevent irreversible neurological sequelae and improve survival.