Korean J Neuromuscul Disord.  2021 Dec;13(2):59-63. 10.46518/kjnmd.2021.13.2.59.

A Case of POEMS Syndrome Previously Diagnosed with Extramedullary Plasmacytoma

Affiliations
  • 1Department of Neurology, College of Medicine, Hanyang University, Seoul, Korea

Abstract

POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin changes) syndrome is a rare multisystem disorder associated with osteosclerotic myeloma. It is characterized by peripheral polyneuropathy, presence of monoclonal plasma cell proliferative disorder, and one or more of the following features – sclerotic bone lesions, Castleman disease, elevated levels of vascular endothelial growth factor, organomegaly, extravascular volume overload, endocrinopathy, skin changes, papilledema, and thrombocytosis. Extramedullary plasmacytoma (EMP) is a rare plasma cell neoplasm that arises in isolated tissues without bone marrow involvement or systemic characteristics of multiple myeloma. Herein, we report a male patient who was previously diagnosed with EMP and later developed POEMS.

Keyword

POEMS syndrome, Extramedullary plasmacytoma, Monoclonal gammopathy
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