Abstract
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.
Highlights
POEMS syndrome is a rare atypical plasma cell dyscrasia with characteristic paraneoplastic manifestations
Desde entonces se consideran marcadores cutáneos específicos del POEMS, si bien recientemente se han reportado casos excepcionales de pacientes con estas lesiones que no padecen del síndrome[11,12,13,14]
Hay casos de tratamiento con lenalidomida en pacientes con severa polineuropatía, observándose respuestas clínicas desde el tercer ciclo y con baja toxicidad[23]
Summary
POEMS syndrome (polyneuropathy, organomegaly, endocrine abnormality, M-protein, plasma cell dyscrasia, and skin lesions) is a rare atypical plasma cell dyscrasia with characteristic paraneoplastic manifestations. En Chile hay 3 casos publicados que se han presentado con polineuropatía sensitivo motora progresiva asociado a diversas endocrinopatías y 2 de ellos con sobrevida de pocos meses. Se presenta un caso de POEMS con hemangiomas glomeruloides y lesión osteolítica.
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