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, 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 "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 :
- Monoclonal protein
- Skin changes
Additional features described in this syndrome include sclerotic bone lesions, Castleman's disease, papilledema, pleural effusion, edema, ascites, erythrocytosis, and thrombocytosis. This disorder has also been called osteosclerotic myeloma, Crow-Fukase syndrome, PEP syndrome (plasma cell dyscrasia, endocrinopathy, polyneuropathy), or Takatsuki syndrome. The complex interrelationships among POEMS syndrome, osteosclerotic myeloma, and Castleman's disease are still being investigated.