MCID: PMS001
MIFTS: 64

Poems Syndrome malady

Rare diseases, Neuronal diseases, Blood diseases, Cancer diseases, Immune diseases, Skin diseases, Bone diseases categories
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Summaries for Poems Syndrome

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NIH Rare Diseases:42 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 based summary: Poems Syndrome, also known as crow-fukase syndrome, is related to thrombocytosis and kaposi's sarcoma, and has symptoms including thick skin/pachydermia/orange skin, irregular/in bands/reticular skin hyperpigmentation and hepatomegaly/liver enlargement (excluding storage disease). An important gene associated with Poems Syndrome is VEGFA (vascular endothelial growth factor A), and among its related pathways are HIF-1 signaling pathway and Nucleotide-binding domain, leucine rich repeat containing receptor (NLR) signaling pathways. The compounds muramyl tripeptide phosphatidylethanolamine and turpentine have been mentioned in the context of this disorder. Affiliated tissues include skin, bone and liver, and related mouse phenotypes are cardiovascular system and tumorigenesis.

Wikipedia:65 POEMS syndrome (also known as Crow?Fukase syndrome, Takatsuki disease, or PEP syndrome) is a rare... more...

Aliases & Classifications for Poems Syndrome

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Sources:
8Disease Ontology, 42NIH Rare Diseases, 10DISEASES, 44Novoseek, 48Orphanet, 62UMLS, 34MeSH, 57SNOMED-CT, 39NCIt, 35MESH via Orphanet, 26ICD10 via Orphanet, 63UMLS via Orphanet
See all sources

Poems Syndrome, Aliases & Descriptions:

Name: Poems Syndrome 8 42 10 44 48 62
Crow-Fukase Syndrome 42 48 62
Polyneuropathy Organomegaly 42 62
Osteosclerotic Myeloma 48 62
Polyneuropathy, Organomegaly, Endocrinopathy, M Protein, and Skin Changes Syndrome 42
 
Polyneuropathy - Endocrinopathy - Plasma Cell Dyscrasia 48
Takatsuki's Syndrome 62
Takatsuki Syndrome 48
Pep Syndrome 48


Classifications:



Characteristics (Orphanet epidemiological data):

48
poems syndrome:
Prevalence: <1/1000000; Age of onset: Adulthood


External Ids:

Disease Ontology8 DOID:14039
MeSH34 D016878
NCIt39 C80303
SNOMED-CT57 79268002
MESH via Orphanet35 D016878
ICD10 via Orphanet26 D47.7
UMLS via Orphanet63 C0085404, C1510415

Related Diseases for Poems Syndrome

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

(show top 50)    (show all 151)
idRelated DiseaseScoreTop Affiliating Genes
1thrombocytosis30.3IL6, IL1B
2kaposi's sarcoma30.3IL6, VEGFA
3myeloma30.2IL1B, CST3, BGLAP, VEGFA, IL6
4arthritis30.0IL6, IL1B, BGLAP
5diabetes mellitus29.2BGLAP, VEGFA, IL6, CST3, IL1B
6rheumatoid arthritis28.8CCL11, IL6, CST3, VEGFA, BGLAP, IL1B
7polyneuropathy10.8
8hemangioma10.7
9endotheliitis10.6
10castleman's disease10.6
11hypertension10.6
12glomeruloid hemangioma10.6
13pulmonary hypertension10.5
14extramedullary plasmacytoma10.5
15osteosclerotic myeloma10.5
16neuropathy10.5
17central retinal vein occlusion10.5VEGFA
18diabetic macular edema10.5VEGFA
19degenerative disc disease10.5IL1B
20multiple myeloma10.4
21calciphylaxis10.4
22periodontal disease10.4IL6
23corneal ulcer10.4CCL11
24demyelinating polyneuropathy10.4
25plasmacytoma10.4
26peripheral neuropathy10.3
27meningioma10.3CST3
28chronic inflammatory demyelinating polyneuropathy10.2
29hematopoietic stem cell transplantation10.2
30cryoglobulinemia10.2
31cerebritis10.2
32amyloidosis10.2
33osteosclerosis10.2
34engraftment syndrome10.2
35synovitis10.2IL6, IL1B
36euthyroid sick syndrome10.2IL1B, IL6
37chorioamnionitis10.2IL1B, IL6
38spiradenoma10.2IL1B, IL6
39adult respiratory distress syndrome10.2IL1B, IL6
40respiratory failure10.2IL1B, IL6
41skin disease10.2IL1B, IL6
42pulmonary fibrosis10.2
43chronic polyneuropathy10.2
44acquired immunodeficiency syndrome10.2IL1B, IL6
45meningitis10.2IL1B, IL6
46juvenile rheumatoid arthritis10.2BGLAP, IL6
47otitis media10.2IL1B, CCL11
48peritonitis10.2IL6, IL1B
49periodontitis10.2IL6, IL1B
50congestive heart failure10.2

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



Diseases related to poems syndrome

Symptoms for Poems Syndrome

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Symptoms:

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

HPO human phenotypes related to Poems Syndrome:

(show all 20)
id Description Frequency HPO Source Accession
1 decreased nerve conduction velocity hallmark (90%) HP:0000762
2 anterior hypopituitarism hallmark (90%) HP:0000830
3 thickened skin hallmark (90%) HP:0001072
4 splenomegaly hallmark (90%) HP:0001744
5 hepatomegaly hallmark (90%) HP:0002240
6 lymphadenopathy hallmark (90%) HP:0002716
7 hypogammaglobulinemia hallmark (90%) HP:0004313
8 irregular hyperpigmentation hallmark (90%) HP:0007400
9 gynecomastia typical (50%) HP:0000771
10 hypothyroidism typical (50%) HP:0000821
11 secondary amenorrhea typical (50%) HP:0000869
12 hypertrichosis typical (50%) HP:0000998
13 lymphedema typical (50%) HP:0001004
14 type ii diabetes mellitus typical (50%) HP:0005978
15 neoplasm of the skeletal system typical (50%) HP:0010622
16 increased bone mineral density typical (50%) HP:0011001
17 arteriovenous malformation typical (50%) HP:0100026
18 nephropathy occasional (7.5%) HP:0000112
19 tapered finger occasional (7.5%) HP:0001182
20 primary adrenal insufficiency occasional (7.5%) HP:0008207

Drugs & Therapeutics for Poems Syndrome

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Drug clinical trials:

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Genetic Tests for Poems Syndrome

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Anatomical Context for Poems Syndrome

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MalaCards organs/tissues related to Poems Syndrome:

32
Skin, Bone, Liver, Breast, Lymph node, Spleen, Lung, Testes, Endothelial, Bone marrow, B cells, Heart, Kidney, Thyroid, Myeloid, Colon, Pancreas, Pituitary

Animal Models for Poems Syndrome or affiliated genes

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MGI Mouse Phenotypes related to Poems Syndrome:

36
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00053858.3CST3, IL1B, IL6, VEGFA
2MP:00020068.0CST3, IL1B, IL6, VEGFA

Publications for Poems Syndrome

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Articles related to Poems Syndrome:

(show top 50)    (show all 443)
idTitleAuthorsYear
1
Computed tomography assessment of bone lesions in patients with POEMS syndrome. (25253619)
2014
2
Correlation between serum level of vascular endothelial growth factor and subfoveal choroidal thickness in patients with POEMS syndrome. (25367833)
2014
3
The characteristics of ascites in patients with POEMS syndrome. (23811954)
2013
4
Outcomes of patients with POEMS syndrome treated initially with radiation. (23699599)
2013
5
POEMS syndrome and Castleman disease. (24006526)
2013
6
POEMS syndrome presenting with acute demyelinating polyneuropathy: increased terminal latency indices and uniform demyelination. (23812201)
2013
7
Glomeruloid hemangiomas in the absence of POEMS syndrome. (22443389)
2012
8
Hemangioma in POEMS syndrome: an atypical clinical and histological presentation. (21749469)
2012
9
POEMS syndrome with prominent acute axonal lesions. (21982617)
2012
10
Comparison of imaging methods in POEMS syndrome. (22580861)
2012
11
Mobilization of PBSCs in poor mobilizers with POEMS syndrome using G-CSF with plerixafor. (22609884)
2012
12
The clinical study of POEMS syndrome in China. (20424579)
2010
13
Crow-Fukase (POEMS) syndrome]. (20420180)
2010
14
POEMS syndrome, congestive heart failure and thalidomide -food for thought. (20823658)
2010
15
POEMS syndrome - a unique presentation of a rare paraneoplastic syndrome. (22798312)
2010
16
A case of POEMS syndrome with mixed hyaline vascular and plasma cell type Castleman's disease. (19670555)
2009
17
POEMS syndrome with an isolated lytic lesion and glomeruloid hemangiomas: report of one case]. (19701559)
2009
18
Bilateral non-simultaneous optic neuropathy and unilateral macular edema in a patient with POEMS syndrome. (19123170)
2009
19
POEMS syndrome: a case report. (19119489)
2008
20
Endocrinopathy in POEMS syndrome: the Mayo Clinic experience. (17605964)
2007
21
A case of POEMS syndrome with cystoid macular edema. (15767082)
2005
22
Polyneuropathy in POEMS syndrome: role of angiogenic factors in the pathogenesis. (15975949)
2005
23
Restricted use of Vlambda genes in POEMS syndrome. (15075094)
2004
24
Thalidomide in POEMS syndrome: case report. (15114600)
2004
25
POEMS syndrome, fibrinogen, and ischemic stroke: a critical point of view. (14732636)
2004
26
POEMS syndrome. (14715103)
2003
27
Crow-Fukase (POEMS) syndrome: a study of peripheral nerve biopsy in five new cases. (12904234)
2003
28
Long-term follow-up on a patient with incomplete POEMS syndrome undergoing high-dose therapy and autologous blood stem cell transplantation. (12360984)
2002
29
POEMS syndrome: a case report and discussion. (11378655)
2001
30
Glomeruloid haemangioma and POEMS syndrome. (11572909)
2001
31
Pulmonary hypertension in POEMS syndrome: a new feature mediated by cytokines. (9517610)
1998
32
Abnormal FSH hypersecretion as an endocrinological manifestation of POEMS syndrome. (9625458)
1998
33
Arterial obliteration in POEMS syndrome: possible role of vascular endothelial growth factor. (9558193)
1998
34
Long term melphalan-prednisolone chemotherapy for POEMS syndrome. (9328260)
1997
35
Myeloneuropathy in POEMS syndrome. (8941279)
1996
36
Acute arterial obliteration: a new feature of the POEMS syndrome? (8699962)
1996
37
Pro-inflammatory cytokines: a pathogenic key of POEMS syndrome]. (8881441)
1996
38
Amyotrophic lateral sclerosis associated with multiple myeloma, endocrinopathy and skin changes suggestive of a POEMS syndrome variant. (7595623)
1995
39
Cryoglobulinemia and other dysproteinemias, familial Mediterranean fever, and POEMS syndrome. (7718425)
1995
40
POEMS syndrome in a female Italian patient. A case report. (8539476)
1995
41
POEMS syndrome: clinical, pathological and immunological study of a case. (7708142)
1994
42
Castleman disease in POEMS syndrome with elevated interleukin-6. (8499003)
1993
43
POEMS syndrome, osteosclerotic myeloma and Castleman's disease: a case report. (1953537)
1991
44
Renal involvement in POEMS syndrome. (1944731)
1991
45
POEMS syndrome with idiopathic flushing mimicking carcinoid syndrome. (2029023)
1991
46
POEMS syndrome: a rarely diagnosed multisystem disorder. (2163715)
1990
47
The endocrinopathy of POEMS syndrome. (2555917)
1989
48
Refractory ascites due to POEMS syndrome. (2535829)
1989
49
POEMS syndrome associated with cryoglobulinemia, lymphoma, multiple seborrheic keratosis, and ichthyosis. (2848065)
1988
50
Renal ultrasound in POEMS syndrome. (3920286)
1985

Variations for Poems Syndrome

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Expression for genes affiliated with Poems Syndrome

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Expression patterns in normal tissues for genes affiliated with Poems Syndrome

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Pathways for genes affiliated with Poems Syndrome

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Pathways related to Poems Syndrome according to GeneCards/GeneDecks:

(show all 37)
idSuper pathways (with members indented)ScoreTop Affiliating Genes
19.5VEGFA, IL6
2
Show member pathways
9.5IL1B, IL6
3
Show member pathways
Immune response MIF in innate immunity response60
9.5IL6, IL1B
4
Show member pathways
thioredoxin pathway37
9.5IL1B, IL6
59.5IL6, IL1B
6
Show member pathways
IL27-mediated signaling events37
9.5IL6, IL1B
7
Show member pathways
9.5IL6, IL1B
89.5IL1B, IL6
99.5IL6, IL1B
10
Show member pathways
9.5IL6, IL1B
11
Show member pathways
9.5IL1B, IL6
12
Show member pathways
9.5IL6, IL1B
139.5IL1B, IL6
149.5IL6, IL1B
159.5IL6, IL1B
169.5IL6, IL1B
179.5IL1B, IL6
189.5IL1B, IL6
199.5IL1B, IL6
209.5IL6, IL1B
219.5IL1B, IL6
229.5IL6, IL1B
239.5IL6, IL1B
249.4CCL11, VEGFA
259.4IL6, BGLAP
269.4IL6, BGLAP
279.3CCL11, IL6
289.1IL1B, VEGFA, IL6
299.1IL6, IL1B, VEGFA
309.0IL6, BGLAP, IL1B
31
Show member pathways
8.9IL1B, IL6, CCL11
32
Show member pathways
Transcription NF kB signaling pathway60
Immune response Toll like receptor TLR ligands and common TLR signaling pathway leading to cell proinflammatory response60
8.9IL1B, IL6, CCL11
338.9CCL11, IL6, IL1B
34
Show member pathways
8.9IL1B, CCL11, IL6
35
Show member pathways
8.9CCL11, IL6, IL1B
36
Show member pathways
8.9CCL11, IL6, IL1B
37
Show member pathways
8.5VEGFA, CCL11, IL6, IL1B

Compounds for genes affiliated with Poems Syndrome

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Compounds related to Poems Syndrome according to GeneCards/GeneDecks:

(show top 50)    (show all 93)
idCompoundScoreTop Affiliating Genes
1muramyl tripeptide phosphatidylethanolamine449.9IL1B, IL6
2turpentine449.9IL1B, IL6
3tenidap44 2810.8IL1B, IL6
4anakinra44 1110.8IL1B, IL6
5taurine chloramine449.8IL1B, IL6
6vesnarinone449.7IL6, IL1B
7hbd2449.7IL1B, IL6
8mip 1alpha449.6IL1B, IL6
9pd 169316449.5VEGFA, BGLAP
10bucillamine449.5VEGFA, IL1B, IL6
11triptolide449.5IL6, VEGFA, IL1B
12gm-csf44 2810.5IL1B, IL6, VEGFA
13risedronate44 50 28 61 1113.5IL1B, BGLAP
14ns 39844 6110.5BGLAP, VEGFA, IL1B
15zoledronic acid449.4BGLAP, IL6, VEGFA
16thalidomide44 50 61 1112.4VEGFA, IL6, IL1B
17clodronate44 50 1111.4IL6, IL1B, BGLAP
18helenalin449.4IL1B, IL6
19curcumin449.4IL6, VEGFA, IL1B
20simvastatin44 50 61 28 24 1114.3BGLAP, VEGFA, IL6
21hydrocortisone44 2 61 1112.3IL1B, BGLAP, IL6
22groalpha449.3CCL11, IL6, IL1B
23budesonide44 50 1111.3BGLAP, IL1B, CCL11
24fluticasone propionate44 50 61 1112.3BGLAP, CCL11, IL1B
25poly(i-c)449.3IL6, CCL11, IL1B
26peptidoglycan44 2810.3IL6, IL1B, CCL11
27ccl344 2810.3CCL11, IL1B, IL6
28leukotriene b444 61 2411.2CCL11, IL6, IL1B
29troglitazone44 28 61 1112.2IL6, CCL11, IL1B
30herbimycin a44 6110.2IL1B, IL6, CCL11
31calcitriol44 61 24 1112.2BGLAP, IL1B, IL6
32endotoxin449.2IL1B, CCL11, IL6
33prednisolone44 28 1111.2BGLAP, IL6, CCL11
34pamidronate44 50 1111.1IL6, BGLAP
35prostaglandin449.1IL1B, IL6, CCL11
36infliximab44 50 1111.0VEGFA, IL1B, IL6, BGLAP
3715-deoxy-delta-12,14-prostaglandin j2448.9IL1B, VEGFA, CCL11, IL6
38hyaluronic acid44 249.9VEGFA, CCL11, IL6, IL1B
39ly294002448.9IL6, VEGFA, CCL11, IL1B
40actinomycin d448.8VEGFA, CCL11, IL1B, IL6
41genistein44 28 61 2 24 1113.8IL6, CCL11, IL1B, VEGFA
42histamine44 28 2410.8IL1B, BGLAP, CCL11, IL6
43methotrexate50 44 1110.7CST3, IL6, IL1B
44sb 20358044 619.4IL1B, IL6, CCL11, VEGFA, BGLAP
45pge2448.4IL6, CCL11, IL1B, VEGFA, BGLAP
46cyclosporin a44 28 6110.4CST3, IL6, BGLAP, CCL11
47alanine448.1CCL11, IL6, BGLAP, CST3
48n acetylcysteine448.1CST3, IL1B, IL6, CCL11, VEGFA
49dexamethasone44 50 28 1110.6VEGFA, CCL11, IL6, IL1B, CST3, BGLAP
50vegf447.6CST3, BGLAP, VEGFA, CCL11, IL6, IL1B

GO Terms for genes affiliated with Poems Syndrome

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

idNameGO IDScoreTop Affiliating Genes
1secretory granuleGO:0301419.6IL1B, VEGFA
2extracellular spaceGO:0056157.0IL1B, BGLAP, VEGFA, CCL11, IL6, CST3
3extracellular regionGO:0055766.8CST3, BGLAP, VEGFA, CCL11, IL6, IL1B

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

(show all 18)
idNameGO IDScoreTop Affiliating Genes
1positive regulation of vascular endothelial growth factor receptor signaling pathwayGO:0309499.9IL1B, VEGFA
2positive regulation of cell divisionGO:0517819.9IL1B, VEGFA
3positive regulation of nitric oxide biosynthetic processGO:0454299.8IL1B, IL6
4positive regulation of interleukin-6 productionGO:0327559.8IL1B, IL6
5positive regulation of peptidyl-serine phosphorylationGO:0331389.8VEGFA, IL6
6positive regulation of T cell proliferationGO:0421029.8IL6, IL1B
7positive regulation of epithelial cell proliferationGO:0506799.7IL6, VEGFA
8negative regulation of cysteine-type endopeptidase activity involved in apoptotic processGO:0431549.7VEGFA, IL6
9response to mechanical stimulusGO:0096129.6IL6, BGLAP
10positive regulation of peptidyl-tyrosine phosphorylationGO:0507319.6VEGFA, IL6
11positive regulation of endothelial cell proliferationGO:0019389.6CCL11, VEGFA
12response to glucocorticoidGO:0513849.4IL6, BGLAP
13positive regulation of sequence-specific DNA binding transcription factor activityGO:0510919.4IL1B, IL6
14positive regulation of protein phosphorylationGO:0019349.3VEGFA, IL1B
15positive regulation of angiogenesisGO:0457669.3IL1B, CCL11, VEGFA
16regulation of cell shapeGO:0083609.2IL6, CCL11, VEGFA
17inflammatory responseGO:0069549.2CCL11, IL6, IL1B
18positive regulation of transcription from RNA polymerase II promoterGO:0459449.1IL1B, IL6, VEGFA

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

idNameGO IDScoreTop Affiliating Genes
1cytokine activityGO:0051259.1IL1B, IL6, VEGFA

Products for genes affiliated with Poems Syndrome

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Sources for Poems Syndrome

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3CDC
13ExPASy
14FMA
22GTR
23HGMD
24HMDB
25ICD10
26ICD10 via Orphanet
27ICD9CM
28IUPHAR
29KEGG
34MeSH
35MESH via Orphanet
36MGI
39NCIt
40NDF-RT
43NINDS
44Novoseek
46OMIM
47OMIM via Orphanet
51PubMed
52QIAGEN
58SNOMED-CT via Orphanet
62UMLS
63UMLS via Orphanet