raremaprare-disease reference indexOrphadata 2026-06-23 · HPO 2026-09-01
ORPHA:2905

POEMS syndrome

Synonyms: Crow-Fukase syndrome · Osteosclerotic myeloma · PEP syndrome · Polyneuropathy-endocrinopathy-plasma cell dyscrasia syndrome · Takatsuki syndrome

Type
Disease
Group
Disorder
Inheritance
Unknown
Age of onset
Adult, Elderly
Status
active

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.

1

Names in Orphadata languages

12 of 12 languages
LangNameSynonyms
enPOEMS syndromeCrow-Fukase syndrome · Osteosclerotic myeloma · PEP syndrome · Polyneuropathy-endocrinopathy-plasma cell dyscrasia syndrome · Takatsuki syndrome
frSyndrome POEMSMyélome ostéosclérosant · Syndrome PEP · Syndrome de Crow-Fukase · Syndrome de Takatsuki · Syndrome de polyneuropathie-endocrinopathie-dyscrasie plasmocytaire
dePOEMS-SyndromCrow-Fukase-Syndrom · Myelom, osteosklerotisches · Polyneuropathie - Endokrinopathie - Plasmazelldyskrasie (PEP) · Takatsuki-Syndrom
esSíndrome de POEMSSíndrome de Crow-Fukase
itSindrome POEMSMieloma osteosclerotico · Polineuropatia - endocrinopatia - discrasia delle cellule plasmatiche · Sindrome PEP · Sindrome di Crow-Fukase · Sindrome di Takatsuki
nlPOEMS-syndroomOsteosclerotisch myeloom · PEP-syndroom · Polyneuropathie - endocrinopathie - plasmaceldyscrasie-syndroom · Syndroom van Crow-Fukase · Syndroom van Takatsuki
ptSíndrome POEMSMieloma osteosclerótico · Síndrome Crow-Fukase · Síndrome de polineuropatia-endocrinopatia-discrasia dos plasmócitos
plZespół POEMSPolineuropatia- endokrynopatia - dyskrazja komórek plazmatycznych · Szpiczak osteosklerotyczny · Zespół Crowa i Fukase'a · Zespół PEP · Zespół Takatsuki
csPOEMS syndromOsteosklerotický myelom · PEP syndrom · Takatsukiho syndrom · Crowův-Fukaseův syndrom · Syndrom zahrnující polyneuropatii, endokrinopatii a dyskrazii plazmatických buněk · Crow-Fukaseův syndrom
ukСиндром POEMSСиндром Кроу-Фукаса · Остеосклеротична мієлома · PEP-синдром · Синдром полінейропатії-ендокринопатії-плазмоклітинної дискразії · Синдром Такацукі
zhPOEMS综合征多发性神经病-内分泌病-浆细胞恶液质综合征
trPOEMS sendromuPEP sendromu · Takatsuki sendromu · Crow-Fukase sendromu · Osteosklerotik miyelom · Polinöropati-endokrinopati-plazma hücreli diskrazi sendromu

Definition in:

2

Cross-references

qualifier = Orphanet mapping relation
SourceCodeQualifierICD relationValidation
ICD-10D47.2NTBTattributedvalidated
ICD-112A83.YNTBTindexvalidated
MONDO0017364Evalidated
MeSHD016878Evalidated
UMLSC0085404Evalidated
GARD7411Evalidated
MedDRA10053869Evalidated

E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.

3

Classification

Preferential parent (Orphanet linearisation):

Parents in the classifications (4):

No children.

4

Genes

0 direct

Orphanet records no gene directly on this code.

5

Prevalence and incidence

2 records
TypeClassValueAreaQualificationValidationSource
Point prevalenceUnknown—EuropeClass onlyNot yet validatedORPHANET
Point prevalence1-9 / 1 000 0000.33 / 100 000JapanValue and classValidated22338030[PMID]

Value is Orphadata's ValMoy: the mean estimate per 100 000 for prevalence and incidence, the number of cases or families for case reports.

6

HPO phenotypes

51 annotations · validated 2017-07-10
HPOTerm (as annotated by Orphanet)Diagnostic
Obligate (100%)
HP:0001271PolyneuropathyDiagnostic criterion
Very frequent (99-80%)
HP:0011122Abnormality of skin physiologyDiagnostic criterion
HP:0000818Abnormality of the endocrine systemDiagnostic criterion
HP:0000135Hypogonadism
HP:0010702Increased circulating immunoglobulin concentration
HP:0005523Lymphoproliferative disorderDiagnostic criterion
HP:0003271VisceromegalyDiagnostic criterion
Frequent (79-30%)
HP:0001284Areflexia
HP:0001541AscitesDiagnostic criterion
HP:0100759Clubbing of fingers
HP:0000819Diabetes mellitus
HP:0000969EdemaDiagnostic criterion
HP:0031052Elevated vascular endothelial growth factor levelDiagnostic criterion
HP:0100639Erectile dysfunction
HP:0012378Fatigue
HP:0000771Gynecomastia
HP:0001028HemangiomaDiagnostic criterion
HP:0002240HepatomegalyDiagnostic criterion
HP:0000953Hyperpigmentation of the skinDiagnostic criterion
HP:0000998HypertrichosisDiagnostic criterion
HP:0000821Hypothyroidism
HP:0001820Leukonychia
HP:0002716LymphadenopathyDiagnostic criterion
HP:0004979Metaphyseal sclerosisDiagnostic criterion
HP:0001324Muscle weakness
HP:0012531Pain
HP:0001085PapilledemaDiagnostic criterion
HP:0003401Paresthesia
HP:0001698Pericardial effusionDiagnostic criterion
HP:0002202Pleural effusionDiagnostic criterion
HP:0008207Primary adrenal insufficiency
HP:0002092Pulmonary arterial hypertension
HP:0100925Sclerosis of foot boneDiagnostic criterion
HP:0004054Sclerosis of hand boneDiagnostic criterion
HP:0002694Sclerosis of skull baseDiagnostic criterion
HP:0004576Sclerotic vertebral endplatesDiagnostic criterion
HP:0001744SplenomegalyDiagnostic criterion
HP:0001072Thickened skin
HP:0001894ThrombocytosisDiagnostic criterion
HP:0001824Weight loss
Occasional (29-5%)
HP:0001063AcrocyanosisDiagnostic criterion
HP:0004420Arterial thrombosis
HP:0031357Glomeruloid hemangioma
HP:0100963Hyperesthesia
HP:0000870Increased circulating prolactin concentration
HP:0009125Lipodystrophy
HP:0001050PlethoraDiagnostic criterion
HP:0001901PolycythemiaDiagnostic criterion
HP:0002747Respiratory insufficiency due to muscle weakness
HP:0002091Restrictive ventilatory defect
HP:0004936Venous thrombosis

Run the differential on the 7 obligate / very frequent terms

Annotation source: 27208909[PMID]_24532337[PMID]_28589896[PMID]_7985714[PMID]

7

Sources for this card