RDD
MCID: RSD004
MIFTS: 52

Rosai-Dorfman Disease (RDD) malady

Rare diseases, Immune diseases categories

Summaries for Rosai-Dorfman Disease

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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 based summary: 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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Sources:
42NIH Rare Diseases, 44Novoseek, 61UMLS
See all sources

Rosai-Dorfman Disease, Aliases & Descriptions:

Name: Rosai-Dorfman Disease 42 44 61
Sinus Histiocytosis with Massive Lymphadenopathy 42 61
Shml 42 61
 
Rosa√Į-Dorfman Disease 42
Rdd 42


Classifications:

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


Related Diseases for Rosai-Dorfman Disease

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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 156)
idRelated DiseaseScoreTop Affiliating Genes
1histiocytosis31.8FSCN1, STAB1, S100B, CD163, SLC29A3
2meningioma31.7S100A1, S100B, CTSD
3langerhans-cell histiocytosis31.6S100B, S100A1
4juvenile xanthogranuloma31.4S100B, FSCN1
5sarcoma30.8CD163, S100B, S100A1, PDGFRA, FSCN1
6amyloidosis30.6CTSD, CRP, SERPINA3
7neurofibromatosis30.6PDGFRA, S100B
8breast cancer30.5SERPINA3, FSCN1, S100A1, CTSD
9tuberculosis30.4CTSD, CRP, SRF
10arthritis30.3SERPINA3, SRF, ALPP, CRP, CD163
11systemic lupus erythematosus30.3F3, SRF, ALPP, CRP
12leukemia30.2SERPINA3, SRF, ALPP, PDGFRA, CTSD
13melanoma30.0SERPINA3, FSCN1, SRF, PDGFRA, S100A1, S100B
14lung cancer29.9SERPINA3, FSCN1, SRF, ALPP, PDGFRA, S100A1
15liver cirrhosis29.7TF, SERPINA3, F3, ALPP, CTSD
16cervicitis10.7
17thyroiditis10.7
18meningitis10.6
19laryngitis10.6
20mediastinitis10.6
21uveitis10.6
22perineurioma10.5S100B
23malignant histiocytosis10.5SERPINA3
24b-cell lymphomas10.5
25sinusitis10.5
26chondroma10.5PDGFRA
27cutaneous fibrous histiocytoma10.4S100B, S100A1
28syringoma10.4S100A1, S100B
29myoepithelioma10.4S100A1, S100B
30granular cell tumor10.4S100A1, S100B
31dermatofibrosarcoma protuberans10.4S100A1, S100B
32subacute thyroiditis10.4
33acute lymphoblastic leukemia10.4
34cerebellar ataxia10.4
35diffuse large b-cell lymphoma10.4
36hemolytic anemia10.4
37myelodysplastic syndromes10.4
38lymphoplasmacyte-rich meningioma10.4
39lupus erythematosus10.4
40lymphoblastic leukemia10.4
41histiocytosis-lymphadenopathy plus syndrome10.4
42ataxia10.4
43histiocytic sarcoma10.4
44hemangiopericytoma10.4S100A1, S100B
45malignant mixed mullerian tumor10.4S100B, S100A1
46breast cyst10.4SERPINA3, CTSD
47alzheimer disease type 210.4SERPINA3, CTSD
48smooth muscle tumor10.4S100A1, S100B
49reticulosarcoma10.4CTSD, FSCN1
50pleomorphic adenoma10.4S100A1, S100B

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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Drug clinical trials:

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Search NIH Clinical Center for Rosai-Dorfman Disease

Genetic Tests for Rosai-Dorfman Disease

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Anatomical Context for Rosai-Dorfman Disease

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MalaCards organs/tissues related to Rosai-Dorfman Disease:

32
Lymph node, Bone, Breast, Spinal cord, Thyroid, Kidney, Lung, Colon, Skin, B cells, Bone marrow, Liver, Pancreas, Testis, Thymus, Trachea, Eye, Brain, Heart, Salivary gland, Ovary, Prostate, Testes, Hypothalamus, Appendix, Pituitary, Neutrophil

Animal Models for Rosai-Dorfman Disease or affiliated genes

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MGI Mouse Phenotypes related to Rosai-Dorfman Disease:

36
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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Articles related to Rosai-Dorfman Disease:

(show top 50)    (show all 511)
idTitleAuthorsYear
1
Orbital Rosai-Dorfman Disease in a fifty-eight years old woman. (24353690)
2013
2
Fine-needle aspiration of primary Rosai-Dorfman disease of the bone without peripheral lymphadenopathy: a challenging diagnosis. (22021127)
2013
3
Rosai-Dorfman disease presenting with extensive cutaneous manifestation - case report. (23739703)
2013
4
Rare coexistence of Rosai-Dorfman disease and nodal marginal zone lymphoma complicated by severe life-threatening autoimmune hemolytic anemia. (23270580)
2013
5
Steroid-resistant extranodal rosai-dorfman disease of cheek mass and ptosis treated with radiation therapy. (23738161)
2013
6
Rosai-dorfman disease (sinus histiocytosis with massive lymphadenopathy) of the pancreas: third reported occurrence. (22864941)
2012
7
Cutaneous Rosai-Dorfman disease: a case report. (23217947)
2012
8
A case of rosai-dorfman disease with highly elevated serum ferritin. (22389884)
2012
9
Rosai-Dorfman disease presenting as multiple breast masses in an otherwise asymptomatic male patient. (22137724)
2012
10
Fine needle aspiration diagnosis of Rosai-Dorfman Disease involving thyroid. (22438629)
2012
11
Rosai-Dorfman disease presenting as a thoracic intradural extramedullary spinal tumor but without extraspinal manifestations. (21959966)
2012
12
Symptomatic extranodal Rosai-Dorfman disease treated with steroids, radiation, and surgery. (21931038)
2011
13
Sinus histiocytosis with massive lymphadenopathy Rosai-Dorfman disease: a unique case presentation. (21906971)
2011
14
Rosai-Dorfman disease--perianaesthetic concerns. (20593637)
2010
15
Intracranial Rosai Dorfman Disease: report of three cases and literature review. (22028755)
2010
16
Primary Rosai-Dorfman disease of bone: a clinicopathologic study of 15 cases. (20679880)
2010
17
Rosai-Dorfman disease. (21103807)
2010
18
Extranodal Rosai-Dorfman disease of bone and nose: a case report and review of literature. (23596759)
2010
19
Extranodal Rosai-Dorfman disease of the kidney. (19139612)
2009
20
Rosai-Dorfman disease (sinus histiocytosis with massive lymphadenopathy) of the pancreas: second case report. (19020944)
2009
21
Rosai-Dorfman disease: sinus histiocytosis with massive lymphadenopathy. (21686390)
2009
22
Rosai-Dorfman disease of lung: a case report and review of the literatures. (19493405)
2009
23
Rosai-Dorfman disease: a case with lymphadenopathy and liver involvement. (19262248)
2009
24
Rosai-Dorfman disease causing spinal cord compression: case report. (18496169)
2008
25
Extranodal Rosai-Dorfman disease with multilevel lumbar spinal lesions. (18590411)
2008
26
Cutaneous Rosai-Dorfman disease. (18627744)
2008
27
Isolated intracranial Rosai-Dorfman disease mimicking suprasellar meningioma: case report with review of the literature. (18831912)
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
FDG PET of Rosai-Dorfman disease of the thymus. (14736692)
2004
38
Extranodal sinus histiocytosis (Rosai-Dorfman disease) of the brain parenchyma. (12601463)
2003
39
Hypoesthesia in the inferior alveolar nerve as the unique clinical manifestation of Rosai-Dorfman disease. (12524619)
2003
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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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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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 pathway59
9.8CRP, SERPINA3
29.4PDGFRA, SRF

Compounds for genes affiliated with Rosai-Dorfman Disease

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Compounds related to Rosai-Dorfman Disease according to GeneCards/GeneDecks:

(show top 50)    (show all 92)
idCompoundScoreTop Affiliating Genes
1iron sucrose4410.1CRP, TF
2nh4cl449.9TF, CTSD, CRP
3phosphoethanolamine449.8CRP, ALPP
4betacarotene449.8CRP, TF, SERPINA3
5silver449.7SERPINA3, CTSD, S100A1
6guanidine hydrochloride449.6F3, ALPP, SERPINA3
7p003449.6CTSD, S100B, CRP
8alpha tocopherol449.6CRP, TF, CTSD
9citrate449.6F3, CRP, S100B, TF
10cyclophosphamide44 50 1211.6TF, CTSD, F3, CRP
11b 723449.6S100B, S100A1, ALPP
12polyethylene glycol449.4TF, ALPP, CRP
13vitamin b12449.4SERPINA3, TF, ALPP, CRP
14polysulfone449.3F3, CRP, SRF
15nocodazole44 1210.2CTSD, SRF, TF
16sialic acid449.2TF, S100B, SRF, SERPINA3
17ascorbic acid44 2510.2TF, CRP, S100B, ALPP, CTSD
18biotin44 25 1211.1S100A1, TF, CRP
19folate449.1SERPINA3, TF, ALPP, PDGFRA, CRP
20vitamin a44 25 1211.0SRF, TF, SERPINA3, CRP
21methotrexate50 44 1211.0SERPINA3, SRF, TF, CRP
22homocysteine44 2510.0F3, SRF, CRP
23latex448.9CTSD, SRF, CRP, ALPP
24phospholipid448.8F3, SRF, S100B, S100A1
25fibrinogen448.7SRF, TF, SERPINA3, F3, CRP
26thymidine44 259.7CTSD, S100B, PDGFRA, ALPP, SRF
27cysteine448.7SERPINA3, F3, CTSD, ALPP, S100B, S100A1
28progesterone44 29 60 25 1212.7SERPINA3, CTSD, S100B, S100A1, SRF
29cyclosporin a44 29 6010.7TF, SRF, ALPP, S100B, CTSD
30acetylcholine44 50 29 25 1212.6SERPINA3, S100A1, SRF, S100B, CRP
31nadph44 259.6S100B, SRF, SERPINA3, CTSD
32zinc44 259.6S100A1, SRF, SERPINA3, CTSD, S100B
33testosterone44 60 25 1211.6CTSD, CRP, SRF, S100B, ALPP
34norepinephrine44 25 1210.6TF, ALPP, CRP, S100B, SRF
35aspartate448.6TF, F3, ALPP, CRP, S100B, CTSD
36cycloheximide448.5CTSD, F3, SRF, ALPP, CRP
37heparin44 29 25 1211.5F3, CTSD, S100B, ALPP, SRF, SERPINA3
38dexamethasone44 50 29 1211.5CD163, S100B, SERPINA3, ALPP, SRF
39glutamate448.4ALPP, S100B, CTSD, SRF, TF
40lactate448.3CRP, TF, SRF, ALPP, CTSD, S100B
41cholesterol44 29 25 1211.3TF, SRF, SERPINA3, CTSD, CRP, ALPP
42serine447.9CTSD, ALPP, SRF, S100B, SERPINA3, F3
43iron44 258.9CD163, TF, SERPINA3, SRF, S100B, CRP
44creatinine447.8S100B, F3, CTSD, CRP, ALPP, SRF
45alanine447.8CTSD, S100B, CRP, ALPP, SRF, F3
46vegf447.7PDGFRA, CRP, TF, F3, CTSD, S100B
47estrogen447.6SRF, S100B, TF, CTSD, SERPINA3, S100A1
48paraffin447.6CTSD, S100B, CD163, S100A1, PDGFRA, ALPP
49retinoic acid44 258.2CTSD, S100B, PDGFRA, ALPP, F3, FSCN1
50calcium44 50 25 129.7S100B, CRP, CTSD, CD163, PDGFRA, S100A1

GO Terms for genes affiliated with Rosai-Dorfman Disease

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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
29IUPHAR
30KEGG
34MeSH
35MESH via Orphanet
36MGI
39NCIt
40NDF-RT
43NINDS
44Novoseek
46OMIM
47OMIM via Orphanet
51PubMed
52QIAGEN
57SNOMED-CT via Orphanet
61UMLS
62UMLS via Orphanet