RDD
MCID: RSD004
MIFTS: 33

Rosai-Dorfman Disease (RDD) malady

Immune diseases category

Summaries for Rosai-Dorfman Disease

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42NIH Rare Diseases, 32MalaCards
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NIH Rare Diseases:42 Rosai-dorfman disease was first described by rosai and dorfman in 1969. it is a benign disease which is characterized by over-production and accumulation of a specific type of white blood cell (histiocyte) in the lymph nodes of the body, most often those of the neck (cervical). other lymph node groups may also be involved and, in some cases, abnormal accumulation of histiocytes may occur in other areas of the body (extranodal). the cause of this condition remains unknown, although altered immune responses and infectious agents may play a role. shml is a self-limited and seldom life-threatening disease which commonly does not require therapy.  last updated: 7/22/2013

MalaCards: Rosai-Dorfman Disease, also known as sinus histiocytosis with massive lymphadenopathy, is related to histiocytosis and meningioma. An important gene associated with Rosai-Dorfman Disease is CTSD (cathepsin D), and among its related pathways is PDGFR-alpha signaling pathway. The compounds cycloheximide and iron sucrose have been mentioned in the context of this disorder. Affiliated tissues include lymph node, and related mouse phenotype normal.

Aliases & Classifications for Rosai-Dorfman Disease

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42NIH Rare Diseases, 44Novoseek
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Classifications:

Malacards categories (disease lists): (See all malacards categories)
Anatomical: Immune diseases


Aliases & Descriptions:

rosai-dorfman disease 42 44
sinus histiocytosis with massive lymphadenopathy 42
rosaï-dorfman disease 42
shml 42
rdd 42


Related Diseases for Rosai-Dorfman Disease

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17GeneCards, 18GeneDecks
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Diseases related to Rosai-Dorfman Disease via text searches within MalaCards or GeneCards/GeneDecks gene sharing:

(show top 50)    (show all 218)
idRelated DiseaseScoreTop Affiliating Genes
1histiocytosis31.6S100B, FSCN1, CD163, STAB1, SLC29A3
2meningioma31.1S100A1, S100B, CTSD
3langerhans-cell histiocytosis30.9S100A1, S100B
4meningitis30.8CRP
5mediastinitis30.8CRP
6sarcoma30.7FSCN1, CD163, S100A1, S100B, PDGFRA
7juvenile xanthogranuloma30.6S100B, FSCN1
8lupus erythematosus30.5F3
9arthritis30.5ALPP, SERPINA3, CD163, CRP, SRF
10leukemia30.5PDGFRA, ALPP, SERPINA3, SRF, CTSD
11systemic lupus erythematosus30.5ALPP, SRF, F3, CRP
12hepatitis30.3ALPP
13vasculitis30.3CRP
14malignant histiocytosis30.3SERPINA3
15sickle cell disease30.3TF
16pancreatitis30.2CRP, ALPP
17sarcoidosis30.2ALPP, SERPINA3
18amyloidosis30.2CRP, SERPINA3, CTSD
19adenocarcinoma30.2CTSD, FSCN1, SRF, SERPINA3
20breast cancer30.2SRF, FSCN1, ALPP, CTSD
21melanoma30.2FSCN1, SRF, SERPINA3, S100A1, PDGFRA, S100B
22lung cancer30.2SRF, ALPP, SERPINA3, S100A1, PDGFRA, FSCN1
23orbital disease10.9
24breast disease10.8
25central nervous system disease10.7
26nervous system disease10.7
27cervicitis10.7
28thyroiditis10.6
29spinal cord disease10.6
30laryngeal disease10.5
31laryngitis10.5
32uveitis10.5
33cerebellar disease10.5
34colonic disease10.5
35kidney disease10.5
36parotid disease10.5
37skin disease10.5
38rhinoscleroma10.4
39eyelid disease10.4
40nose disease10.4
41pancreas disease10.4
42splenic disease10.4
43subacute thyroiditis10.3
44diffuse large b-cell lymphoma10.3
45acute leukemia10.3
46cerebellar ataxia10.3
47hemolytic anemia10.3
48liver disease10.3
49lymphoblastic leukemia10.3
50paranasal sinus disease10.3

Graphical network of the top 20 diseases related to Rosai-Dorfman Disease:



Diseases related to rosai-dorfman disease

Clinical Features for Rosai-Dorfman Disease

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Drugs & Therapeutics for Rosai-Dorfman Disease

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Sources:
5CenterWatch, 41NIH Clinical Center, 6ClinicalTrials, 60UMLS, 40NDF-RT
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Approved drugs:

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Genetic Tests for Rosai-Dorfman Disease

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Anatomical Context for Rosai-Dorfman Disease

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32MalaCards
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MalaCards organs/tissues related to Rosai-Dorfman Disease:

32
Lymph node

Animal Models for Rosai-Dorfman Disease or affiliated genes

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36MGI
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MGI Mouse Phenotypes related to Rosai-Dorfman Disease:

36
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00028737.1ALPP, SRF, F3, STAB1, PDGFRA, S100A1

Publications for Rosai-Dorfman Disease

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Genetic Variations for Rosai-Dorfman Disease

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Expression for genes affiliated with Rosai-Dorfman Disease

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1BioGPS, 15Gene Expression Omnibus DataSets
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Expression patterns in normal tissues for genes affiliated with Rosai-Dorfman Disease

Search GEO for disease gene expression data for Rosai-Dorfman Disease.

Pathways for genes affiliated with Rosai-Dorfman Disease

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37NCBI BioSystems Database
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Pathways related to Rosai-Dorfman Disease according to GeneCards/GeneDecks:

idSuper pathways (with members indented)ScoreTop Affiliating Genes
19.4SRF, PDGFRA

Compounds for genes affiliated with Rosai-Dorfman Disease

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Sources:
44Novoseek, 11DrugBank, 24HMDB, 49PharmGKB, 28IUPHAR, 59Tocris Bioscience
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Compounds related to Rosai-Dorfman Disease according to GeneCards/GeneDecks:

(show top 50)    (show all 68)
idCompoundScoreTop Affiliating Genes
1cycloheximide4410.3ALPP
2iron sucrose4410.1CRP, TF
3p0034410.1S100B, CTSD
4sialic acid4410.1SRF
5nh4cl449.9CTSD, TF, CRP
6phosphoethanolamine449.9CRP, ALPP
7aspartate449.8PDGFRA, CTSD, ALPP
8betacarotene449.8SERPINA3, CRP, TF
9olopatadine44 1110.8S100B, S100A1
10vitamin a44 11 2411.7SERPINA3, SRF
11alpha tocopherol449.7CTSD, TF, CRP
12polysulfone449.7SRF, F3
13silver449.7SERPINA3, CTSD, S100A1
14glutamate449.7SRF
15guanidine hydrochloride449.6F3, SERPINA3, ALPP
16cyclophosphamide44 49 1111.6F3, CRP, TF, CTSD
17citrate449.6F3, CRP, TF, S100B
18b 723449.6S100B, S100A1, ALPP
19polyethylene glycol449.5ALPP, CRP, TF
20zinc protoporphyrin449.5CRP, TF
21vitamin b12449.4TF, CRP, SERPINA3, ALPP
22heparin44 28 11 2412.3SERPINA3, SRF, CTSD, S100B
23nocodazole44 1110.3SRF, TF, CTSD
24biotin44 11 2411.3S100A1, TF, CRP
25acetylcholine44 49 28 11 2413.3SERPINA3, SRF, S100A1
26cyclosporin a44 28 5911.2S100B, CTSD, TF, SRF
27ascorbic acid44 2410.2S100B, CTSD, TF, CRP, ALPP
28folate449.1PDGFRA, TF, CRP, SERPINA3, ALPP
29methotrexate44 49 1111.0TF, CRP, SRF, SERPINA3
30latex448.9ALPP, SRF, CRP, CTSD
31homocysteine44 249.8SRF, F3, CRP
32phospholipid448.8S100B, S100A1, F3, SRF
33fibrinogen448.7TF, CRP, F3, SRF, SERPINA3
34thymidine44 249.7S100B, PDGFRA, CTSD, SRF, ALPP
35dexamethasone44 49 28 1111.7S100B, CD163, SRF, ALPP
36progesterone44 59 28 11 2412.7S100B, S100A1, CTSD, SRF, SERPINA3
37cysteine448.6S100B, S100A1, CTSD, F3, SERPINA3, ALPP
38norepinephrine44 11 2410.6S100B, TF, CRP, SRF, ALPP
39testosterone44 59 11 2411.6ALPP, SRF, CRP, CTSD, S100B
40zinc44 249.5S100B, S100A1, CTSD, SRF, SERPINA3
41serine448.5S100B, FSCN1, SRF, SERPINA3, ALPP
42lactate448.4S100B, CTSD, TF, CRP, SRF, ALPP
43cholesterol44 28 11 2411.3CTSD, TF, CRP, SRF, SERPINA3, ALPP
44creatinine448.0CTSD, TF, CRP, F3, SRF, SERPINA3
45iron44 248.9S100B, S100A1, TF, CRP, CD163, SRF
46alanine447.8S100B, CTSD, TF, CRP, F3, SRF
47vegf447.7S100B, PDGFRA, CTSD, TF, CRP, F3
48paraffin447.6ALPP, S100B, S100A1, PDGFRA, CTSD, TF
49retinoic acid44 248.2S100B, S100A1, PDGFRA, CTSD, F3, FSCN1
50calcium44 49 11 249.7S100B, S100A1, PDGFRA, CTSD, CRP, CD163

GO Terms for genes affiliated with Rosai-Dorfman Disease

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16Gene Ontology
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Cellular components related to Rosai-Dorfman Disease according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1extracellular spaceGO:0056158.7SERPINA3, F3, CRP, CTSD, S100B
2extracellular regionGO:0055768.6CTSD, TF, CRP, CD163, SERPINA3, S100B

Biological processes related to Rosai-Dorfman Disease according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1cardiac myofibril assemblyGO:0550039.4SRF, PDGFRA
2acute-phase responseGO:0069539.1SERPINA3, CD163, CRP

Molecular functions related to Rosai-Dorfman Disease according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1low-density lipoprotein particle bindingGO:0301699.5STAB1, CRP
2S100 protein bindingGO:0445489.4S100A1, S100B
3protein homodimerization activityGO:0428038.1S100B, S100A1, PDGFRA, CRP, SRF
4protein bindingGO:0055156.1SERPINA3, S100B, S100A1, PDGFRA, CTSD, TF

Products for genes affiliated with Rosai-Dorfman Disease

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Sources for Rosai-Dorfman Disease

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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
50PubMed
51QIAGEN
57SNOMED-CT via Orphanet
60UMLS
61UMLS via Orphanet