POEMS syndrome is a paraneoplastic syndrome characterized by polyradiculoneuropathy (P), organomegaly (O), endocrinopathy (E), clonal plasma cell disorder (M), and skin changes (S). Other features include papilledema, extravascular volume overload, sclerotic bone lesions, thrombocytosis/erythrocytosis, and elevated VEGF levels. [ Orphanet:2905 http://www.ncbi.nlm.nih.gov/pubmed/23398538 ]
Synonyms: osteosclerotic myeloma polyneuropathy-endocrinopathy-plasma cell dyscrasia syndrome PEP syndrome Crow-Fukase syndrome Takatsuki syndrome POEMS syndrome
Term information
- GARD:7411 (MONDO:GARD)
- MedDRA:10053869 (Orphanet:2905/e)
- UMLS:C0085404 (MONDO:equivalentTo)
- SCTID:79268002 (MONDO:equivalentTo)
- NANDO:1200033 (https://orcid.org/0000-0003-0011-764X)
- EFO:1001115 (MONDO:equivalentTo)
- MEDGEN:39276 (MONDO:equivalentTo)
- NORD:1586 (MONDO:NORD)
- NCIT:C80303 (MONDO:exact-label-match)
- Orphanet:2905 (MONDO:equivalentTo)
- MESH:D016878 (Orphanet:2905/e)
- DOID:14039 (MONDO:equivalentTo)
ordo_disorder, gard_rare, otar, rare, nord_rare, orphanet_rare
POEMS syndrome is associated with plasmacytomas and osteosclerotic lesions. The etiologic factors of this constellation of diseases are not well defined, but radiation therapy to localized lesions is often of benefit, and there is a report that autologous hematopoetic cell transplantation benefited a patient with refractory disease.
http://purl.obolibrary.org/obo/NCIT_C80303
http://identifiers.org/medgen/39276
http://identifiers.org/snomedct/79268002
http://identifiers.org/mesh/D016878
http://www.orpha.net/ORDO/Orphanet_2905
http://purl.obolibrary.org/obo/DOID_14039
http://linkedlifedata.com/resource/umls/id/C0085404
polyneuropathy organomegaly
polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes syndrome