POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, skin changes) is a rare paraneoplastic syndrome due to a plasma cell disorder. Diagnosis requires peripheral neuropathy and a monoclonal plasma cell disorder along with one major and one minor criteria, but cardiac manifestations are uncommon. The pathogenesis of POEMS syndrome is not well understood but it is thought to involve overproduction of proinflammatory cytokines, such as vascular endothelial growth factor (VEGF), interleukin-1 beta (IL-1B), interleukin-6 (IL-6), and tumor necrosis factor-alpha (TNF-alpha). POEMS syndrome commonly presents in the fifth to sixth decade of life, mainly in non-Hispanic Caucasian individuals, and affects more men than women (2:1). We report a unique case of a 28-year-old African American female with a history of POEMS syndrome and a new diagnosis of dilated, non-ischemic cardiomyopathy and New York Heart Association (NYHA) class IV, stage D heart failure with an ejection fraction (EF) of 30% as a result of the natural progression of her untreated POEMS syndrome.
Keywords: castleman disease; heart failure; hematology; poems syndrome; pulmonary hypertension.
Copyright © 2021, Abdelahad et al.