RDD
MCID: RSD004
MIFTS: 52

Rosai-Dorfman Disease (RDD) malady

Rare diseases, Immune diseases categories

Summaries for Rosai-Dorfman Disease

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44NIH Rare Diseases, 34MalaCards
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NIH Rare Diseases:44 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 are Jak/Stat Pathway and PDGFR-alpha signaling pathway. The compounds iron sucrose and nh4cl have been mentioned in the context of this disorder. Affiliated tissues include lymph node, bone and breast, and related mouse phenotypes are hematopoietic system and immune system.

Aliases & Classifications for Rosai-Dorfman Disease

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44NIH Rare Diseases, 46Novoseek
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Classifications:

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


Aliases & Descriptions:

rosai-dorfman disease 44 46
sinus histiocytosis with massive lymphadenopathy 44
rosa√Į-dorfman disease 44
shml 44
rdd 44


Related Diseases for Rosai-Dorfman Disease

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18GeneCards, 19GeneDecks
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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 153)
idRelated DiseaseScoreTop Affiliating Genes
1histiocytosis31.8S100B, FSCN1, CTSD
2meningioma31.3S100B, S100A1, CTSD
3langerhans-cell histiocytosis31.1S100B, S100A1
4juvenile xanthogranuloma30.9S100B, FSCN1
5sarcoma30.8S100B, S100A1, PDGFRA, FSCN1, CD163
6arthritis30.5SRF, SERPINA3, CRP, ALPP, CD163
7leukemia30.5PDGFRA, ALPP, SERPINA3, SRF, CTSD
8amyloidosis30.4SERPINA3, CTSD, CRP
9neurofibromatosis30.4S100B, PDGFRA
10tuberculosis30.4SRF, CTSD, CRP
11systemic lupus erythematosus30.3F3, ALPP, SRF, CRP
12melanoma30.3FSCN1, SERPINA3, SRF, PDGFRA, S100A1, S100B
13lung cancer30.2FSCN1, SRF, PDGFRA, ALPP, S100A1, SERPINA3
14cervicitis10.7
15thyroiditis10.7
16mediastinitis10.6
17meningitis10.6
18uveitis10.6
19laryngitis10.5
20rhinoscleroma10.5
21b-cell lymphomas10.5
22sinusitis10.5
23subacute thyroiditis10.4
24diffuse large b-cell lymphoma10.4
25hemolytic anemia10.4
26lymphoblastic leukemia10.4
27myelodysplastic syndromes10.4
28histiocytosis-lymphadenopathy plus syndrome10.4
29histiocytic sarcoma10.4
30familial sinus histiocytosis with massive lymphadenopathy10.3
31rosacea10.2
32autoimmune thyroiditis10.2
33autoimmune lymphoproliferative syndrome10.2
34factor xii deficiency10.2
35parasagittal meningioma10.2
36petroclival meningioma10.2
37suprasellar meningioma10.2
38autoimmune hemolytic anemia10.2
39lymphoplasmacyte-rich meningioma10.2
40constrictive pericarditis10.2
41choroiditis10.2
42radiculopathy10.2
43sjogren's syndrome10.2
44cerebritis10.2
45breast lymphoma10.2
46granuloma annulare10.2
47adenocarcinoma10.2
48capillary hemangioma10.2
49cerebellar ataxia10.2
50diverticulitis10.2

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



Diseases related to rosai-dorfman disease

Symptoms for Rosai-Dorfman Disease

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Drugs & Therapeutics for Rosai-Dorfman Disease

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6CenterWatch, 43NIH Clinical Center, 7ClinicalTrials, 63UMLS, 42NDF-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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34MalaCards
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MalaCards organs/tissues related to Rosai-Dorfman Disease:

34
Lymph node, Bone, Breast, Thyroid, Spinal cord, Lung, Skin, B cells, Colon, Kidney, Pancreas, Bone marrow, Liver, Trachea, Thymus, Testis, Appendix, Eye, Testes, Prostate, Ovary, Brain, Salivary gland, Heart, Pituitary

Animal Models for Rosai-Dorfman Disease or affiliated genes

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38MGI
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MGI Mouse Phenotypes related to Rosai-Dorfman Disease:

38
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00053978.2SLC29A3, CTSD, PDGFRA, SRF, F3, TF
2MP:00053877.8FSCN1, F3, SRF, PDGFRA, CRP, CTSD
3MP:00053697.8F3, STAB1, SRF, PDGFRA, S100A1
4MP:00053857.5F3, STAB1, SRF, PDGFRA, CRP, S100A1
5MP:00028737.3S100B, F3, STAB1, SRF, ALPP, PDGFRA
6MP:00053767.1TF, F3, STAB1, SRF, PDGFRA, CRP
7MP:00107687.1TF, F3, STAB1, SRF, ALPP, PDGFRA

Publications for Rosai-Dorfman Disease

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53PubMed
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Articles related to Rosai-Dorfman Disease:

(show top 50)    (show all 489)
idTitleAuthorsYear
1
Orbital Rosai-Dorfman Disease in a fifty-eight years old woman. (24353690)
2013
2
Emperipolesis is not pathognomonic for Rosai-Dorfman disease: rhinoscleroma mimicking Rosai-Dorfman disease, a clinical series. (24238175)
2013
3
Fine-needle aspiration of primary Rosai-Dorfman disease of the bone without peripheral lymphadenopathy: a challenging diagnosis. (22021127)
2013
4
Rosai-Dorfman disease presenting with extensive cutaneous manifestation - case report. (23739703)
2013
5
Rare coexistence of Rosai-Dorfman disease and nodal marginal zone lymphoma complicated by severe life-threatening autoimmune hemolytic anemia. (23270580)
2013
6
Steroid-resistant extranodal rosai-dorfman disease of cheek mass and ptosis treated with radiation therapy. (23738161)
2013
7
Rosai-dorfman disease (sinus histiocytosis with massive lymphadenopathy) of the pancreas: third reported occurrence. (22864941)
2012
8
Cutaneous Rosai-Dorfman disease: a case report. (23217947)
2012
9
A case of rosai-dorfman disease with highly elevated serum ferritin. (22389884)
2012
10
Rosai-Dorfman disease presenting as multiple breast masses in an otherwise asymptomatic male patient. (22137724)
2012
11
Fine needle aspiration diagnosis of Rosai-Dorfman Disease involving thyroid. (22438629)
2012
12
Rosai-Dorfman disease presenting as a thoracic intradural extramedullary spinal tumor but without extraspinal manifestations. (21959966)
2012
13
Symptomatic extranodal Rosai-Dorfman disease treated with steroids, radiation, and surgery. (21931038)
2011
14
Sinus histiocytosis with massive lymphadenopathy Rosai-Dorfman disease: a unique case presentation. (21906971)
2011
15
Rosai-Dorfman disease--perianaesthetic concerns. (20593637)
2010
16
Intracranial Rosai Dorfman Disease: report of three cases and literature review. (22028755)
2010
17
Primary Rosai-Dorfman disease of bone: a clinicopathologic study of 15 cases. (20679880)
2010
18
Rosai-Dorfman disease. (21103807)
2010
19
Extranodal Rosai-Dorfman disease of bone and nose: a case report and review of literature. (23596759)
2010
20
Extranodal Rosai-Dorfman disease of the kidney. (19139612)
2009
21
Rosai-Dorfman disease (sinus histiocytosis with massive lymphadenopathy) of the pancreas: second case report. (19020944)
2009
22
Rosai-Dorfman disease: sinus histiocytosis with massive lymphadenopathy. (21686390)
2009
23
Rosai-Dorfman disease of lung: a case report and review of the literatures. (19493405)
2009
24
Rosai-Dorfman disease: a case with lymphadenopathy and liver involvement. (19262248)
2009
25
Rosai-Dorfman disease causing spinal cord compression: case report. (18496169)
2008
26
Extranodal Rosai-Dorfman disease with multilevel lumbar spinal lesions. (18590411)
2008
27
Cutaneous Rosai-Dorfman disease. (18627744)
2008
28
A rare case of extranodal Rosai-Dorfman disease. (16980664)
2006
29
Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease) treated with 2-chlorodeoxyadenosine. (16302214)
2006
30
Rosai-Dorfman disease presenting as bilateral eyelid swellings. (16484848)
2006
31
Rosai-Dorfman disease mimicking a sphenoid wing meningioma. (15805669)
2005
32
Sinus histiocytosis with massive lymphadenopathy (Rosai Dorfman disease): evidence for a dendritic cell derivation? (16761737)
2005
33
Nasal and paranasal sinus manifestation of Rosai-Dorfman disease. (16218519)
2005
34
Isolated Rosai Dorfman disease of the central nervous system presenting as dural-based and intraparenchymal lesions. (15943162)
2005
35
Clinical and histopathologic spectrum of cutaneous Rosai-Dorfman disease in Taiwan. (15583585)
2004
36
Sinus histocytosis with massive lymphadenopathy (Rosai-Dorfman disease) in a patient with primary Sjogren's syndrome. (15163114)
2004
37
Extranodal sinus histiocytosis (Rosai-Dorfman disease) of the brain parenchyma. (12601463)
2003
38
Hypoesthesia in the inferior alveolar nerve as the unique clinical manifestation of Rosai-Dorfman disease. (12524619)
2003
39
Cutaneous rosai-dorfman disease is a distinct clinical entity. (12357197)
2002
40
Cutaneous sinus histiocytosis (Rosai-Dorfman disease) presenting clinically as vasculitis. (12004323)
2002
41
Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease): report of two cases with fine-needle aspiration cytology. (11135468)
2001
42
Rosai-Dorfman disease mimicking parasagittal meningioma: case presentation and review of literature. (10874144)
2000
43
Rosai dorfman disease - an unusual presentation: A case report. (23119695)
2000
44
Cutaneous Rosai-Dorfman disease--a pathologic review of 2 cases. (11063196)
2000
45
Importance of Papanicolaou-stained smears and immunocytochemistry in the diagnosis of Rosai-Dorfman disease. (10097737)
1999
46
Nasal manifestation of extranodal Rosai-Dorfman disease--diagnosis and management. (10435144)
1999
47
Review of sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease) of head and neck. (10579239)
1999
48
Immunohistologic findings and adhesion molecule pattern in primary pure cutaneous Rosai-Dorfman disease with xanthomatous features. (9700380)
1998
49
Rosai-Dorfman disease and juvenile xanthogranuloma. (9185229)
1997
50
Monokine expression in Langerhans' cell histiocytosis and sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease) (8691347)
1996

Variations for Rosai-Dorfman Disease

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Expression for genes affiliated with Rosai-Dorfman Disease

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2BioGPS, 16Gene Expression Omnibus DataSets
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Expression patterns in normal tissues for genes affiliated with Rosai-Dorfman Disease

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Pathways for genes affiliated with Rosai-Dorfman Disease

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51PathCards, 5Cell Signaling Technology, 61Thomson Reuters, 39NCBI BioSystems Database
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Pathways related to Rosai-Dorfman Disease according to GeneCards/GeneDecks:

idSuper pathways (with members indented)ScoreTop Affiliating Genes
1
Show member pathways
Development Thrombopoetin signaling via JAK STAT pathway61
9.8CRP, SERPINA3
29.4PDGFRA, SRF

Compounds for genes affiliated with Rosai-Dorfman Disease

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46Novoseek, 52PharmGKB, 12DrugBank, 25HMDB, 30IUPHAR, 62Tocris Bioscience
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Compounds related to Rosai-Dorfman Disease according to GeneCards/GeneDecks:

(show top 50)    (show all 92)
idCompoundScoreTop Affiliating Genes
1iron sucrose4610.1CRP, TF
2nh4cl469.9CTSD, CRP, TF
3phosphoethanolamine469.8CRP, ALPP
4betacarotene469.8CRP, SERPINA3, TF
5silver469.7CTSD, S100A1, SERPINA3
6guanidine hydrochloride469.6SERPINA3, F3, ALPP
7p003469.6CTSD, S100B, CRP
8alpha tocopherol469.6TF, CRP, CTSD
9citrate469.6TF, F3, CRP, S100B
10cyclophosphamide46 52 1211.6CTSD, CRP, F3, TF
11b 723469.6ALPP, S100A1, S100B
12polyethylene glycol469.4CRP, ALPP, TF
13vitamin b12469.4TF, SERPINA3, ALPP, CRP
14polysulfone469.3CRP, SRF, F3
15nocodazole46 1210.2CTSD, SRF, TF
16sialic acid469.2S100B, SRF, SERPINA3, TF
17ascorbic acid46 2510.2TF, ALPP, CRP, S100B, CTSD
18biotin46 25 1211.1S100A1, CRP, TF
19folate469.1TF, SERPINA3, ALPP, PDGFRA, CRP
20vitamin a46 25 1211.0TF, SERPINA3, SRF, CRP
21methotrexate52 46 1211.0CRP, SRF, SERPINA3, TF
22homocysteine46 2510.0CRP, SRF, F3
23latex468.9CTSD, CRP, ALPP, SRF
24phospholipid468.8F3, SRF, S100A1, S100B
25fibrinogen468.7TF, SERPINA3, F3, SRF, CRP
26thymidine46 259.7SRF, ALPP, PDGFRA, S100B, CTSD
27cysteine468.7SERPINA3, F3, ALPP, S100A1, S100B, CTSD
28progesterone46 30 62 25 1212.7SERPINA3, SRF, S100A1, S100B, CTSD
29cyclosporin a46 30 6210.7TF, SRF, ALPP, S100B, CTSD
30acetylcholine46 52 30 25 1212.6SERPINA3, SRF, CRP, S100A1, S100B
31nadph46 259.6SERPINA3, SRF, S100B, CTSD
32zinc46 259.6SERPINA3, SRF, S100A1, S100B, CTSD
33testosterone46 62 25 1211.6SRF, ALPP, CRP, S100B, CTSD
34norepinephrine46 25 1210.6TF, SRF, ALPP, CRP, S100B
35aspartate468.6TF, F3, ALPP, PDGFRA, CRP, S100B
36cycloheximide468.5F3, SRF, ALPP, CRP, CTSD
37heparin46 30 25 1211.5SERPINA3, F3, SRF, ALPP, S100B, CTSD
38dexamethasone46 52 30 1211.5CD163, S100B, ALPP, SRF, SERPINA3
39glutamate468.4TF, SRF, ALPP, S100B, CTSD
40lactate468.3TF, SRF, ALPP, CRP, S100B, CTSD
41cholesterol46 30 25 1211.3TF, SERPINA3, SRF, ALPP, CRP, CTSD
42serine467.9SERPINA3, FSCN1, F3, SRF, ALPP, S100B
43iron46 258.9TF, SERPINA3, SRF, CRP, S100A1, S100B
44creatinine467.8TF, SERPINA3, F3, SRF, ALPP, CRP
45alanine467.8TF, SERPINA3, F3, SRF, ALPP, CRP
46vegf467.7TF, F3, SRF, ALPP, PDGFRA, CRP
47estrogen467.6TF, SERPINA3, SRF, ALPP, PDGFRA, S100A1
48paraffin467.6CTSD, TF, SERPINA3, FSCN1, ALPP, PDGFRA
49retinoic acid46 258.2SERPINA3, FSCN1, F3, SRF, ALPP, PDGFRA
50calcium46 52 25 129.7SERPINA3, FSCN1, SRF, ALPP, PDGFRA, CRP

GO Terms for genes affiliated with Rosai-Dorfman Disease

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17Gene Ontology
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Cellular components related to Rosai-Dorfman Disease according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1extracellular spaceGO:0056158.9CTSD, S100B, CRP, F3, TF
2extracellular regionGO:0055768.3CTSD, TF, SERPINA3, CRP, S100B, CD163

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

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

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

idNameGO IDScoreTop Affiliating Genes
1low-density lipoprotein particle bindingGO:0301699.5CRP, STAB1
2S100 protein bindingGO:0445489.4S100B, S100A1
3protein homodimerization activityGO:0428038.1SRF, PDGFRA, CRP, S100A1, S100B
4protein bindingGO:0055155.5CTSD, TF, SERPINA3, FSCN1, F3, STAB1

Products for genes affiliated with Rosai-Dorfman Disease

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Sources for Rosai-Dorfman Disease

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4CDC
14ExPASy
15FMA
23GTR
24HGMD
25HMDB
26ICD10
27ICD10 via Orphanet
28ICD9CM
30IUPHAR
31KEGG
36MeSH
37MESH via Orphanet
38MGI
41NCIt
42NDF-RT
45NINDS
46Novoseek
48OMIM
49OMIM via Orphanet
53PubMed
54QIAGEN
60SNOMED-CT via Orphanet
63UMLS
64UMLS via Orphanet