MCID: PMS001
MIFTS: 61

Poems Syndrome malady

Neuronal, Blood, Cancer, Skin, Bone categories

Summaries for Poems Syndrome

Sources:
43NIH Rare Diseases, 64Wikipedia, 33MalaCards
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NIH Rare Diseases:43 Poems syndrome is a rare blood disorder. it is characterized by polyneuropathy, organomegaly, endocrinopathy, monoclonal plasma proliferative disorder, and skin changes. signs and symptoms may include progressive sensorimotor polyneuropathy, enlarged liver and spleen, swollen lymph nodes, and darkening of the skin. further testing and evaluation may show plasma cell disorder and sclerotic bone lesions. many people with poems syndrome have diabetes (20-50%) and primary gonadal failure (55-70%). other general signs and symptoms may include increased body hair, thickening of the skin, whitening of the nails, fatigue, weakness, swelling of the ankles and legs, enlarged breast tissue, generalized aches and pains, fluid in the lungs, and vision changes. sign and symptoms tend to begin in a person's 40's to 50's. poems syndrome is a rapidly progressive disorder that can become life threatening. currently the exact cause of poems syndrome is unknown. researchers believe the syndrome involves an overgrowth of bone marrow cells (plasma cells), which produce chemicals that damage other parts of the body. last updated: 2/18/2013

MalaCards: Poems Syndrome, also known as osteosclerotic myeloma, is related to vascular disease and amyloidosis, and has symptoms including hypothyroidy, hepatomegaly/liver enlargement (excluding storage disease) and splenomegaly. An important gene associated with Poems Syndrome is VEGFA (vascular endothelial growth factor A), and among its related pathways are Rheumatoid arthritis and Prion diseases. The compounds indomethacin and pge2 have been mentioned in the context of this disorder. Affiliated tissues include skin, liver and kidney, and related mouse phenotypes are tumorigenesis and immune system.

Wikipedia:64 POEMS syndrome (also known as Crow–Fukase syndrome, Takatsuki disease, or PEP syndrome) is a rare... more...

Aliases & Classifications for Poems Syndrome

Sources:
8Disease Ontology, 43NIH Rare Diseases, 10DISEASES, 45Novoseek, 49Orphanet, 61UMLS, 35MeSH, 40NCIt, 57SNOMED-CT, 36MESH via Orphanet, 26ICD10 via Orphanet, 58SNOMED-CT via Orphanet, 62UMLS via Orphanet
See all sources

Classifications:

Malacards categories (disease lists): (See all malacards categories)
Global: Cancer
Anatomical: Neuronal, Blood, Skin, Bone


Characteristics (Orphanet epidemiological data):

49
poems syndrome:
Prevalence: <1/1000000; Age of onset: Adulthood


Aliases & Descriptions:

poems syndrome 8 43 10 45 49 61
osteosclerotic myeloma 49 61
crow-fukase syndrome 43 49
polyneuropathy, organomegaly, endocrinopathy, m protein, and skin changes syndrome 43
polyneuropathy - endocrinopathy - plasma cell dyscrasia 49
polyneuropathy organomegaly 43
takatsuki syndrome 49
pep syndrome 49


External Ids:

Disease Ontology8 DOID:14039
MeSH35 D016878
NCIt40 C80303
MESH via Orphanet36 D016878
SNOMED-CT57 79268002
ICD10 via Orphanet26 D47.7
SNOMED-CT via Orphanet58 425657001, 79268002
UMLS via Orphanet62 C0085404, C1510415

Related Diseases for Poems Syndrome

Sources:
17GeneCards, 18GeneDecks
See all sources

Diseases related to Poems Syndrome via text searches within MalaCards or GeneCards/GeneDecks gene sharing:

(show top 50)    (show all 257)
idRelated DiseaseScoreTop Affiliating Genes
1vascular disease30.4CST3, TNFSF11, TNFRSF11B, VEGFA
2amyloidosis30.3CST3
3polycythemia vera30.2TIMP1
4familial mediterranean fever30.0IL1B
5osteonecrosis30.0BGLAP
6macroglobulinemia30.0MAG
7acromegaly30.0BGLAP
8mastocytosis30.0IL5
9kaposi's sarcoma30.0IL6
10thrombocytosis30.0CSF3, IL1B, IL6
11thrombocytopenia29.9CSF3, IL6
12arthritis29.9TIMP1, TNFSF11, IL1B, IL6
13n syndrome10.8
14castleman's disease10.7
15glomeruloid hemangioma10.6
16osteosclerotic myeloma10.5
17extramedullary plasmacytoma10.5
18multicentric castleman’s disease10.4
19multicentric castleman disease10.4
20char syndrome10.4
21micro syndrome10.3
22calciphylaxis10.3
23stroke, ischemic10.2
24cryoglobulinemia10.2
25chronic inflammatory demyelinating polyneuropathy10.2
26engraftment syndrome10.2
27hematopoietic stem cell transplantation10.2
28cystoid macular edema10.1
29alopecia10.1
30angiofollicular lymph hyperplasia10.1
31kid syndrome10.1
32mass syndrome10.1
33diabetic nephropathy10.0
34primary pulmonary hypertension10.0
35diabetic ketoacidosis10.0
36lateral sclerosis10.0
37pulmonary edema10.0
38optic disk drusen10.0
39hypoparathyroidism10.0
40systemic lupus erythematosus10.0
41smoldering myeloma10.0
42portal hypertension10.0
43chronic myeloid leukemia10.0
44cauda equina syndrome10.0
45trigeminal neuralgia10.0
46conn's syndrome10.0
47amyotrophic lateral sclerosis10.0
48vasculitis10.0
49amyloid neuropathy10.0
50scleredema10.0

Graphical network of the top 20 diseases related to Poems Syndrome:



Diseases related to poems syndrome

Clinical Features for Poems Syndrome

Sources:
49Orphanet
See all sources


Symptoms:

49 (show all 23)
  • hypothyroidy
  • hepatomegaly/liver enlargement (excluding storage disease)
  • splenomegaly
  • insulin-independent/type 2 diabetes
  • nerve conduction abnormality
  • renal disease/nephropathy
  • late puberty/hypogonadism/hypogenitalism
  • osteosclerosis/osteopetrosis/bone condensation
  • irregular/in bands/reticular skin hyperpigmentation
  • hirsutism/hypertrichosis/increased body hair
  • thick skin/pachydermia/orange skin
  • lymphedema
  • arteriovenous malformations/vascular malformations (excluding port-wine stains)
  • precocious menopause/secondary amenorrhea
  • agammaglobulinemia/hypogammaglobulinemia/b-cell deficiency
  • hypothalamic-hypophyseal axis functional anomalies/hypopituitarism
  • cortico-adrenal hypoplasia/insufficiency
  • hypotrichosis/atrichia/atrichiasis/scalp hairlessness
  • gynecomastia/breast/mammary gland enlargement/hyperplasia
  • bone/osseous neoplasm/tumor/carcinoma/cancer
  • decreased body hair/axillar/pubic hairlessness
  • lymphadenopathy/polyadenopathies
  • tapered fingers

Drugs & Therapeutics for Poems Syndrome

Sources:
5CenterWatch, 42NIH Clinical Center, 6ClinicalTrials, 61UMLS, 41NDF-RT
See all sources

Approved drugs:

Search CenterWatch for Poems Syndrome

Drug clinical trials:

Search ClinicalTrials for Poems Syndrome

Search NIH Clinical Center for Poems Syndrome

Search CenterWatch for Poems Syndrome

Genetic Tests for Poems Syndrome

Anatomical Context for Poems Syndrome

Sources:
33MalaCards
See all sources

MalaCards organs/tissues related to Poems Syndrome:

33
Skin, Liver, Kidney, Lymph node, Lung, Spleen, Bone marrow, Heart, Colon, Pancreas, Thyroid, Breast, Myeloid, B cells, Endothelial, Pituitary

Animal Models for Poems Syndrome or affiliated genes

Sources:
37MGI, 28inGenious Targeting Laboratory
See all sources

MGI Mouse Phenotypes related to Poems Syndrome:

37
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00020069.7CST3, IL5, IL1B, IL6, TIMP1
2MP:00053878.3IL6, TIMP1, CCL11, CCL3, CSF3, TNFSF11
3MP:00053978.3CSF3, CCL3, CCL11, TIMP1, TNFSF11, TNFRSF11B

Publications for Poems Syndrome

Sources:
51PubMed
See all sources

Articles related to Poems Syndrome:

(show top 50)    (show all 429)
idTitleAuthorsYear
1
The characteristics of ascites in patients with POEMS syndrome. (23811954)
2013
2
Ischemic stroke as clinical onset of POEMS syndrome. (24221644)
2013
3
Markedly upregulated serum interleukin-12 as a novel biomarker in POEMS syndrome. (22843279)
2012
4
Uniform demyelination and more severe axonal loss distinguish POEMS syndrome from CIDP. (22396441)
2012
5
Pachymeningeal involvement in POEMS syndrome: MRI and histopathological study. (21653206)
2012
6
Sonographic findings in a case of polyneuropathy associated with POEMS syndrome. (21626513)
2011
7
Serum vascular endothelial growth factor (VEGF) in the differential diagnosis of amyloid neuropathy and POEMS syndrome. (21838451)
2011
8
POEMS syndrome: 2011 update on diagnosis, risk-stratification, and management. (21681783)
2011
9
POEMS syndrome: are current diagnostic criteria too exclusive? (21501892)
2011
10
Successful treatment with bortezomib and thalidomide for POEMS syndrome. (21153416)
2011
11
Two more cases of evaluation of POEMS syndrome using 18-FDG PET/CT. (20510565)
2011
12
Crow-Fukase (POEMS) syndrome]. (20420180)
2010
13
Multiple myocardial infarctions in a 35 year-old woman with POEMS syndrome. (20038511)
2010
14
Sildenafil and steroid therapy effectively improved POEMS syndrome-associated pulmonary arterial hypertension. (21132469)
2010
15
Pituitary macroadenoma in a patient with POEMS syndrome in conjunction with Castleman disease: first report of a case and review of related literature. (19546057)
2010
16
Neurological recovery from pentaplegia after resection for sacral plasmacytoma presenting with POEMS syndrome. (19441989)
2009
17
Glomeruloid haemangioma is not always associated with POEMS syndrome. (19077091)
2009
18
Restrictive usage of monoclonal immunoglobulin lambda light chain germline in POEMS syndrome. (18497319)
2008
19
A case of POEMS syndrome treated by autologous hematopoietic stem-cell transplantation. (19043425)
2008
20
Treatment for POEMS (polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes) syndrome. (18843731)
2008
21
A novel report of cig-FISH and cytogenetics in POEMS syndrome. (18839437)
2008
22
POEMS syndrome complicated by extensive arterial thromboses. (17410319)
2007
23
A case report of POEMS syndrome developing during the clinical course of immune thrombocytopenic purpura. (17957571)
2007
24
Lenalidomide therapy in a patient with POEMS syndrome. (17644745)
2007
25
Two causes of demyelinating neuropathy in one patient: CMT1A and POEMS syndrome. (17803044)
2007
26
Electrophysiological features of patients with POEMS syndrome. (15792906)
2005
27
POEMS syndrome. (15384241)
2004
28
POEMS syndrome demonstrating VEGF decrease by ticlopidine. (15609708)
2004
29
Diurnal fluctuation of edema synchronized with plasma VEGF concentration in a patient with POEMS syndrome. (12521215)
2002
30
Epidural anaesthesia in a patient with POEMS syndrome. (11999604)
2002
31
Circulating levels of MMP-1, -2, -3, -9, and TIMP-1 are increased in POEMS syndrome. (11274326)
2001
32
Reversible pulmonary hypertension in POEMS syndrome--another etiology of triggered pulmonary vasculopathy? (10978936)
2000
33
POEMS syndrome with necrotizing vasculitis: a novel feature of vascular abnormalities. (10680829)
2000
34
Soluble immune mediators in POEMS syndrome with pulmonary hypertension: case report and review of the literature. (10654928)
1999
35
POEMS syndrome: cicatricial alopecia as an unusual cutaneous manifestation associated with an underlying plasmacytoma. (10321621)
1999
36
Pulmonary hypertension in POEMS syndrome: a new feature mediated by cytokines. (9517610)
1998
37
Ultrastructural characterisation of the M protein in nerve biopsy of patients with POEMS syndrome. (9647319)
1998
38
Greatly raised vascular endothelial growth factor (VEGF) in POEMS syndrome. (8596427)
1996
39
Intravenous immunoglobulin therapy in POEMS syndrome: a case report. (9037854)
1996
40
Pulmonary hypertension associated with POEMS syndrome. (8866608)
1996
41
Cryoglobulinemia and other dysproteinemias, familial Mediterranean fever, and POEMS syndrome. (7718425)
1995
42
POEMS syndrome in systemic lupus erythematosus. (1613712)
1992
43
Polyneuropathy, organomegaly, endocrinopathy, M-protein and skin change (POEMS) syndrome with IgG kappa paraproteinemia. (1437299)
1992
44
POEMS syndrome: a case report. (2276681)
1990
45
The POEMS syndrome among Chinese: association with Castleman's disease and some immunological abnormalities. (2559570)
1989
46
POEMS syndrome associated with cryoglobulinemia, lymphoma, multiple seborrheic keratosis, and ichthyosis. (2848065)
1988
47
POEMS syndrome presenting as systemic sclerosis. Clinical and pathologic study of a case with microangiopathic glomerular lesions. (3348253)
1988
48
Pathological findings in three non-Japanese patients with the POEMS syndrome. (2845640)
1988
49
Renal ultrasound in POEMS syndrome. (3920286)
1985
50
Plasma cell dyscrasia with polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes: the POEMS syndrome. Report on two cases and a review of the literature. (6248720)
1980

Genetic Variations for Poems Syndrome

Expression for genes affiliated with Poems Syndrome

Sources:
1BioGPS, 15Gene Expression Omnibus DataSets
See all sources
Expression patterns in normal tissues for genes affiliated with Poems Syndrome

Search GEO for disease gene expression data for Poems Syndrome.

Pathways for genes affiliated with Poems Syndrome

Sources:
30KEGG, 53R&D Systems, 12EMD Millipore, 52QIAGEN, 38NCBI BioSystems Database, 54Reactome
See all sources

Pathways related to Poems Syndrome according to GeneCards/GeneDecks:

(show all 22)
idSuper pathways (with members indented)ScoreTop Affiliating Genes
110.3TNFSF11
210.3NCAM1
310.1IL1B, IL5
4
Transcription Role of VDR in regulation of genes involved in osteoporosis
Hide members
10.1TNFRSF11B, TNFSF11
510.0IL5, IL1B, IL6
610.0TIMP1, TNFSF11, IL6
7
Hide members
10.0IL5, CCL11, TIMP1
8
Hide members
9.9CD36, VEGFA, TIMP1
99.9CCL3, IL1B, IL6
109.9CCL3, CD36, IL1B
119.9BGLAP, IL5, IL6
12
Immune response Oncostatin M signaling via JAK-Stat in human cells
Hide members
9.8IL6, VEGFA, CCL11, TIMP1
13
Hide members
9.8IL6, IL1B, IL5, CCL11
14
Hide members
9.8CD36, IGLV1-44, IGLV1-40
15
Hide members
9.8IL6, IL1B, IL5, NCAM1
16
Hide members
9.7IL5, TNFSF11, CCL3, TIMP1
179.7CCL11, TNFSF11, IL5, IL1B, IL6
189.5CSF3, CD36, IL5, IL1B, IL6
19
Hide members
9.2TIMP1, CCL11, CCL3, CSF3, TNFSF11, IL5
20
Hide members
9.2NCAM1, IGLV1-40, IGLV1-44, CD36, IL5, IL1B
21
Hide members
9.0IL5, TIMP1, CCL11, CCL3, CSF3, TNFSF11
22
Hide members
8.9IL5, TNFSF11, CSF3, CCL3, CCL11, TIMP1

Compounds for genes affiliated with Poems Syndrome

Sources:
45Novoseek, 60Tocris Bioscience, 29IUPHAR, 11DrugBank, 2BitterDB, 50PharmGKB, 24HMDB
See all sources

Compounds related to Poems Syndrome according to GeneCards/GeneDecks:

(show top 50)    (show all 155)
idCompoundScoreTop Affiliating Genes
1indomethacin45 60 29 1113.7IL5
2pge24510.6CCL11, VEGFA
3matrigel4510.5CCL11, NCAM1
4hydrocortisone45 2 60 1113.4TNFRSF11B, BGLAP
5prednisolone45 29 1112.1TNFRSF11B, CCL11, IL5, BGLAP
6clodronate45 50 1112.1TNFSF11, IL6, TNFRSF11B, CD36, IL1B
7prostaglandin4510.1TNFRSF11B, IL1B, TNFSF11
8mip 1alpha4510.0TNFSF11, IL6, IL1B, CCL3
9procollagen4510.0TNFSF11
10teriparatide45 1111.0BGLAP, TNFSF11, CD36, TNFRSF11B
11mcp 14510.0CCL3, TIMP1, IL5, IL6
12etanercept45 50 1112.0IL6, IL1B, TNFRSF11B, TNFSF11, TIMP1
13ibandronate45 1111.0BGLAP, CD36, TNFRSF11B, TNFSF11
14poly(i-c)459.9IL5, CCL3, IL1B, IL6, CCL11
15pamidronate45 50 1111.9BGLAP, TNFSF11, IL6, CD36, TNFRSF11B
16risedronate45 60 50 29 1113.9CD36, TNFRSF11B, BGLAP, TNFSF11, IL1B
17vitamin k2459.9TNFRSF11B, TNFSF11, BGLAP, CD36
18ns 39845 6010.9VEGFA, IL1B, TNFRSF11B, BGLAP, TNFSF11
19rantes459.9IL6, CCL11, IL1B, CCL3, CSF3
20thalidomide45 50 60 1112.9TNFRSF11B, VEGFA, IL6, CD36, IL5, IL1B
21budesonide45 50 1111.8CCL11, CD36, IL5, IL1B, BGLAP
22peptidoglycan45 2910.8IL6, IL1B, TNFSF11, CCL3, CCL11
23alendronate45 50 1111.8CD36, TNFRSF11B, TNFSF11, BGLAP, TIMP1
2415-deoxy-delta-12,14-prostaglandin j2459.8IL6, IL1B, CD36, VEGFA, CCL11
25zoledronic acid459.8IL6, TNFSF11, CD36, BGLAP, TNFRSF11B, VEGFA
26titanium459.8TNFSF11, BGLAP, TNFRSF11B, CD36, CCL3
27deoxypyridinoline459.8TNFSF11, CCL3, BGLAP, CD36, TNFRSF11B
28pmma459.7CCL3, IL6, TNFRSF11B, TNFSF11
29methylcellulose459.7TIMP1, CCL3, CSF3, IL5, IL6
30tacrolimus45 50 1111.7TIMP1, CCL3, IL5, TNFSF11
31n acetylcysteine459.7TIMP1, CCL11, CST3, TNFSF11, VEGFA, IL1B
32infliximab45 50 1111.7TNFRSF11B, VEGFA, IL1B, IL6, TIMP1, BGLAP
33herbimycin a45 6010.6IL6, TIMP1, CCL11, IL1B, IL5, CD36
34hyaluronic acid45 2410.6TIMP1, IL1B, VEGFA, MYOM2, CD36, IL6
35tgf beta1459.6TNFRSF11B, CCL3, IL6, CD36, IL1B, TIMP1
36ccl345 2910.6CCL3, IL6, IL1B, CSF3, CCL11
37calcitriol45 60 11 2412.5BGLAP, TNFSF11, TNFRSF11B, CD36, IL1B, IL6
38celecoxib45 60 29 50 11 2414.5TNFRSF11B, IL1B, CCL3, TNFSF11, VEGFA
39dexamethasone45 50 29 1112.5TIMP1, BGLAP, CCL11, TNFSF11, TNFRSF11B, VEGFA
40methotrexate45 50 1111.4IL5, TNFSF11, CSF3, CST3, TIMP1, IL6
41genistein45 29 60 2 11 2414.4TIMP1, CCL11, VEGFA, TNFRSF11B, IL1B, IL6
42cyclosporin a45 29 6011.4TNFRSF11B, CSF3, CST3, CCL3, TNFSF11, CCL11
43vitamin d459.3BGLAP, TIMP1, TNFSF11, IL6, TNFRSF11B, CD36
44histamine45 29 2411.3IL6, IL1B, IL5, NCAM1, CCL3, BGLAP
45ly294002459.2VEGFA, TNFSF11, TIMP1, CCL11, CCL3, IL6
46heparin45 29 11 2412.1TNFSF11, TIMP1, BGLAP, CCL3, TNFRSF11B, NCAM1
47sb 20358045 609.9IL6, TIMP1, CCL3, IL1B, IL5, CD36
48cysteine458.7IL5, TIMP1, BGLAP, CCL3, CST3, TNFSF11
49retinoic acid45 249.6TIMP1, IL6, BGLAP, CSF3, CCL3, CCL11
50vegf458.2CCL11, CCL3, CSF3, TNFSF11, TNFRSF11B, NCAM1

GO Terms for genes affiliated with Poems Syndrome

Sources:
16Gene Ontology
See all sources

Cellular components related to Poems Syndrome according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1extracellular regionGO:0055768.3CST3, CCL3, CCL11, BGLAP, TNFSF11, TNFRSF11B
2extracellular spaceGO:0056158.0IL6, BGLAP, CCL11, CCL3, CST3, CSF3

Biological processes related to Poems Syndrome according to GeneCards/GeneDecks:

(show all 18)
idNameGO IDScoreTop Affiliating Genes
1positive regulation of intracellular protein kinase cascadeGO:01074010.2VEGFA, TNFSF11
2positive regulation of interleukin-6 productionGO:03275510.2CD36, IL1B, IL6
3monocyte chemotaxisGO:00254810.2CCL3, TNFSF11, IL6
4negative regulation of neuron deathGO:190121510.2IL6, CSF3
5positive regulation of immunoglobulin secretionGO:05102410.2IL6, IL5
6positive regulation of sequence-specific DNA binding transcription factor activityGO:05109110.1IL6, IL1B, IL5, TNFSF11
7positive regulation of peptidyl-serine phosphorylationGO:03313810.1IL6, VEGFA, CSF3
8regulation of cell shapeGO:00836010.1CCL11, CCL3, VEGFA, IL6
9positive regulation of angiogenesisGO:04576610.0IL1B, VEGFA, CCL11
10eosinophil chemotaxisGO:04824510.0CCL11, CCL3
11platelet activationGO:0301689.9TIMP1, VEGFA, CD36, IL6
12positive regulation of protein kinase B signaling cascadeGO:0518979.9IL6, TNFSF11, CSF3, CCL3
13inflammatory responseGO:0069549.8IL6, IL1B, IL5, CCL3, CCL11
14positive regulation of peptidyl-tyrosine phosphorylationGO:0507319.8IL6, IL5, CD36, VEGFA, CSF3
15immune responseGO:0069559.7CCL11, CSF3, TNFSF11, IL5, IL1B
16platelet degranulationGO:0025769.6CD36, VEGFA, TIMP1
17cytokine-mediated signaling pathwayGO:0192219.6CSF3, TNFSF11, NCAM1, IL5, IL1B, IL6
18cell adhesionGO:0071559.5BGLAP, CCL11, NCAM1, MAG, CD36

Molecular functions related to Poems Syndrome according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1growth factor activityGO:0080839.7IL6, IL5, VEGFA, CSF3
2cytokine activityGO:0051258.9IL6, CSF3, TNFSF11, TNFRSF11B, VEGFA, IL5

Products for genes affiliated with Poems Syndrome

  • Antibodies
  • Proteins
  • Lysates
  • Antibodies

Sources for Poems Syndrome

3CDC
13ExPASy
14FMA
22GTR
23HGMD
24HMDB
25ICD10
26ICD10 via Orphanet
27ICD9CM
29IUPHAR
30KEGG
35MeSH
36MESH via Orphanet
37MGI
40NCIt
41NDF-RT
44NINDS
45Novoseek
47OMIM
48OMIM via Orphanet
51PubMed
52QIAGEN
58SNOMED-CT via Orphanet
61UMLS
62UMLS via Orphanet