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Revista médica de Chile

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CHANDIA, Mauricio; SALOMONE, Claudia; BERTIN, Pablo  and  GONZALEZ, Sergio. POEMS syndrome with an isolated lytic lesion and glomeruloid hemangiomas: Report of one case. Rev. méd. Chile [online]. 2009, vol.137, n.5, pp.680-684. ISSN 0034-9887.

POEMS syndrome (polyneuropathy, organomegaly, endocrine abnormality, M-protein, plasma cell dyscrasia, and skin lesions) is a rare atypical plasma cell dyscrasia with characteristic para neoplastic manifestations. Glomeruloid hemangioma is a typical skin change pathogenetically related with elevated levels of Vascular Endothelial Growth Factor (VEGF). We report a 69 year-old woman that presented cachexia associated with diabetes, hypothyroidism and severe sensitive motor polyneuropathy. Her skin changes included hyper pigmentation, acrocyanosis and glomeruloid hemangioma. The subsequent study revealed a monoclonal gammopathy lambda type; a unique lytic vertebral lesion and a clonal plasma cell proliferation. Treatment with prednisone 0.5 mg/kg and melphalan 0,25 mg/kg in cycles of 4 days every 4 weeks was started, but the patient was lost from follow up.

Keywords : Paraproteinemias; Plasma cells; POEMS syndrome.

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