2019•Recenti Progressi in MedicinaRequires access

Un raro caso di sindrome di POEMS: in assenza di sintomi di polineuropatia

Antonio Fabio Massimo Vainieri, Sergio Morelli

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Abstract

POEMS syndrome (P polyradiculoneuropathy, O organomegaly, E endocrinopathy, M clonal plasma cell disorder, and S skin changes) is a rare syndrome due to a plasma cell dyscrasia. It is characterized by polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, skin changes. Increased serum VEGF, sclerotic bone lesions, Castleman's disease, oedema, ascites, papilledema and thrombocytosis are other important clinical features. We report a case of POEMS syndrome in a 34-years old man with an unusual clinical presentation. Dyspnoea, organomegaly, and skin changes preceded the clinical manifestations of polyneuropathy. The variability of presentation of the POEMS should suggest to consider the diagnosis even in the lack of the signs of polyneuropathy.

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What this paper is about

POEMS syndrome (P polyradiculoneuropathy, O organomegaly, E endocrinopathy, M clonal plasma cell disorder, and S skin changes) is a rare syndrome due to a plasma cell dyscrasia. It is characterized by polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, skin changes. Increased serum VEGF, sclerotic bone lesions, Castleman's disease, oedema, ascites, papilledema and thrombocytosis are other important clinical features. We report a case of POEMS syndrome in a 34-years old man with an unusual clinical presentation. Dyspnoea, organomegaly, and skin changes preceded the clinical manifestations of polyneuropathy. The variability of presentation of the POEMS should suggest to consider the diagnosis even in the lack of the signs of polyneuropathy.

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Available abstract

POEMS syndrome (P polyradiculoneuropathy, O organomegaly, E endocrinopathy, M clonal plasma cell disorder, and S skin changes) is a rare syndrome due to a plasma cell dyscrasia. It is characterized by polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, skin changes. Increased serum VEGF, sclerotic bone lesions, Castleman's disease, oedema, ascites, papilledema and thrombocytosis are other important clinical features. We report a case of POEMS syndrome in a 34-years old man with an unusual clinical presentation. Dyspnoea, organomegaly, and skin changes preceded the clinical manifestations of polyneuropathy. The variability of presentation of the POEMS should suggest to consider the diagnosis even in the lack of the signs of polyneuropathy.

Key concepts: Organomegaly, POEMS syndrome, Polyneuropathy, Medicine, Plasma cell dyscrasia, Thrombocytosis, Papilledema, Dyscrasia

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