POEMS syndrome (Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal protein, Skin changes) is characterized by the presence of a monoclonal plasma cell disorder, peripheral neuropathy, and one or more of the following features: osteosclerotic myeloma, Castleman's disease (angiofollicular lymph node hyperplasia), increased levels of serum vascular endothelial growth factor (VEGF), organomegaly, endocrinopathy, edema, typical skin changes, and papilledema .
The clinical features, diagnosis, and treatment of POEMS syndrome will be discussed here. A discussion of Castleman's disease is presented separately. (See "Multicentric Castleman's disease".)
In 1938, a patient was described with sensorimotor peripheral neuropathy, hyperpigmentation, elevated cerebrospinal fluid protein, and a solitary plasmacytoma . Eighteen years later, two patients were reported with peripheral neuropathy, hyperpigmentation, elevation of cerebrospinal fluid protein, and plasmacytomas with new bone formation . In 1980, the acronym "POEMS" was suggested for this disorder, representing the following constellation of findings :