RDD
MCID: RSD004
MIFTS: 44

Rosai-Dorfman Disease (RDD) malady

Immune category

Summaries for Rosai-Dorfman Disease

Sources:
43NIH Rare Diseases, 33MalaCards
See all sources

Fully expand this MalaCard

Export this MalaCard
NIH Rare Diseases:43 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 juvenile xanthogranuloma and arthritis. 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 skin, ovary and liver, and related mouse phenotype normal.

Aliases & Classifications for Rosai-Dorfman Disease

Sources:
43NIH Rare Diseases, 45Novoseek
See all sources

Classifications:

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


Aliases & Descriptions:

rosai-dorfman disease 43 45
sinus histiocytosis with massive lymphadenopathy 43
rosaï-dorfman disease 43
shml 43
rdd 43


Related Diseases for Rosai-Dorfman Disease

Sources:
17GeneCards, 18GeneDecks
See all sources

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

(show top 50)    (show all 178)
idRelated DiseaseScoreTop Affiliating Genes
1juvenile xanthogranuloma30.7FSCN1, S100B
2arthritis30.6CRP, CD163, SRF, SERPINA3, ALPP
3systemic lupus erythematosus30.5CRP, F3, SRF, ALPP
4vasculitis30.3CRP
5sickle cell disease30.3TF
6amyloidosis30.3CTSD, CRP, SERPINA3
7adenocarcinoma30.3SERPINA3, SRF, FSCN1, CTSD
8spinal cord disease10.7
9uveitis10.6
10rhinoscleroma10.5
11splenic disease10.5
12acute leukemia10.4
13histiocytosis-lymphadenopathy plus syndrome10.4
14ataxia10.4
15lymphoma, diffuse large cell10.4
16histiocytic sarcoma10.4
17subacute thyroiditis10.4
18familial sinus histiocytosis with massive lymphadenopathy10.3
19crohn's disease10.3
20neurofibromatosis10.3
21autoimmune lymphoproliferative syndrome10.3
22autoimmune thyroiditis10.3
23langerhans cell sarcoma10.3
24rosacea10.3
25thymus lymphoma10.3
26submandibular gland disease10.3
27radiculopathy10.3
28sjogren's syndrome10.3
29acute t cell leukemia10.3
30liver lymphoma10.3
31parasagittal meningioma10.3
32petroclival meningioma10.3
33suprasellar meningioma10.3
34autoimmune hemolytic anemia10.3
35orbital granuloma10.3
36lymphoplasmacyte-rich meningioma10.3
37breast lymphoma10.3
38pediatric lymphoma10.3
39anaplastic large cell lymphoma10.3
40n syndrome10.3
41granuloma annulare10.3
42constrictive pericarditis10.3
43acne10.3
44brain disease10.3
45t-cell leukemia10.3
46testicular disease10.3
47thrombocytopenia10.3
48thyroiditis10.3
49tracheal disease10.3
50tuberculosis10.3

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



Diseases related to rosai-dorfman disease

Clinical Features for Rosai-Dorfman Disease

Drugs & Therapeutics for Rosai-Dorfman Disease

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

Approved drugs:

Search CenterWatch for Rosai-Dorfman Disease

Drug clinical trials:

Search ClinicalTrials for Rosai-Dorfman Disease

Search NIH Clinical Center for Rosai-Dorfman Disease

Search CenterWatch for Rosai-Dorfman Disease

Genetic Tests for Rosai-Dorfman Disease

Anatomical Context for Rosai-Dorfman Disease

Sources:
33MalaCards
See all sources

MalaCards organs/tissues related to Rosai-Dorfman Disease:

33
Skin, Ovary, Liver, Spinal cord, Brain, Kidney, Lymph node, Lung, Bone marrow, Thymus, Heart, Small intestine, Colon, Pancreas, Thyroid, Salivary gland, Breast, Prostate, Testis, B lymphoblasts, B cells, Trachea, Appendix, Pituitary

Animal Models for Rosai-Dorfman Disease or affiliated genes

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

MGI Mouse Phenotypes related to Rosai-Dorfman Disease:

37
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00028737.1ALPP, SRF, F3, STAB1, PDGFRA, S100A1

Publications for Rosai-Dorfman Disease

Sources:
51PubMed
See all sources

Articles related to Rosai-Dorfman Disease:

(show top 50)    (show all 497)
idTitleAuthorsYear
1
Orbital Rosai-Dorfman Disease in a fifty-eight years old woman. (24353690)
2013
2
18F-FDG PET/CT findings in a patient with isolated intracranial Rosai-Dorfman disease. (23242068)
2013
3
Primary pulmonary artery Rosai-Dorfman disease mimicking sarcoma. (24075466)
2013
4
Radiology-pathology case report: isolated extranodal Rosai-Dorfman disease of the skull base. (23932387)
2013
5
A case of rosai-dorfman disease with highly elevated serum ferritin. (22389884)
2012
6
Fine needle aspiration diagnosis of Rosai-Dorfman Disease involving thyroid. (22438629)
2012
7
Emperipolesis: an additional common histopathologic finding in H syndrome and Rosai-Dorfman disease. (22356918)
2012
8
Reconstruction of a patient with Rosai-Dorfman disease using ramus graft and osseointegrated implants: a case report. (20553156)
2012
9
Multiple involvement of the central nervous system in Rosai-Dorfman disease. (22196495)
2012
10
Radiotherapy for steroid-resistant laryngeal Rosai-Dorfman disease. (21655154)
2011
11
Extranodal rosai-dorfman disease with cutaneous and periodontal involvement: a rare presentation. (21475597)
2011
12
Rosai-Dorfman disease manifesting as an epibulbar ocular tumour. (20955503)
2011
13
Isolated cranio-spinal involvement of Rosai-Dorfman disease: case report. (20825289)
2011
14
Rosai-Dorfman disease-like changes in mesenteric lymph nodes secondary to Salmonella infection. (22007843)
2011
15
Rosai-Dorfman Disease with nodal and extranodal involvements: A case report. (22973396)
2011
16
Fine needle aspiration diagnosis of Rosai-Dorfman disease in an osteolytic lesion of bone. (20806072)
2010
17
Rosai-Dorfman disease as a differential diagnosis in vocal cord ulceration. (21180951)
2010
18
Ethmo-spheno-intracranial Rosai-Dorfman disease. (20071801)
2010
19
Extranodal Rosai-Dorfman disease involving the heart: report of two cases. (19819734)
2010
20
Development of disseminated histiocytic sarcoma in a patient with autoimmune lymphoproliferative syndrome and associated Rosai-Dorfman disease. (20216376)
2010
21
Anaesthesia for a paediatric patient with Rosai-Dorfman disease. (20716573)
2010
22
Rosai-Dorfman disease of lung: a case report and review of the literatures. (19493405)
2009
23
A case of cutaneous Rosai-Dorfman disease refractory to imatinib therapy. (19451502)
2009
24
Rosai-Dorfman disease in an Asian Indian woman with classic generalized lymphadenopathy and nasal obstruction: a case report. (19291621)
2009
25
Multifocal nodal and extranodal Rosai-Dorfman disease initially diagnosed as histiocytic lymphoma. (19108414)
2008
26
Maxillofacial Rosai-Dorfman disease in a newly diagnosed HIV-infected patient. (17714970)
2008
27
Fatal worsening of late-onset cerebellar ataxia with neuronal intranuclear inclusions due to superimposed meningeal Rosai-Dorfman disease. (18546340)
2008
28
Thyroid involvement in Rosai-Dorfman disease. (17542678)
2007
29
Atypical presentation of Rosai-Dorfman disease in the lacrimal gland mimicking malignancy. (16550067)
2006
30
Extranodal Rosai-Dorfman disease of the kidney and coexistent poorly differentiated prostatic adenocarcinoma. (16879029)
2006
31
Extranodal Rosai-Dorfman disease: involvement of eye, nose and trachea. (16720453)
2006
32
Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease): clinico-pathological study of three cases. (16622659)
2006
33
Spinal cord compression as a result of Rosai-Dorfman disease of the upper cervical spine in a child. (15650889)
2005
34
Bilateral anterior granulomatous uveitis associated with cutaneous Rosai-Dorfman disease. (15806376)
2005
35
Extranodal Rosai-Dorfman disease with multifocal bone and epidural involvement causing recurrent spinal cord compression. (16211443)
2005
36
Rosai-Dorfman disease: successful long-term results by combination chemotherapy with prednisone, 6-mercaptopurine, methotrexate, and vinblastine: a case report. (16086087)
2005
37
Facial asymmetry with nasal and orbital involvement in a case of sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease). (16005357)
2005
38
Rosai-Dorfman disease revealed in the upper airway: a case report and review of the literature. (15364364)
2004
39
Bilateral cauliflower ear deformity: an unusual presentation of cutaneous Rosai-Dorfman disease. (15108890)
2004
40
Squash cytology of Rosai-Dorfman disease in the sellar region. (14674101)
2003
41
A case of Rosai-Dorfman disease involving the lacrimal gland in an elderly patient. (12029574)
2002
42
Orbital, adnexal, and unusual systemic involvement in Rosai-Dorfman disease. (12021656)
2002
43
Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease): report of two cases with fine-needle aspiration cytology. (11135468)
2001
44
Rosai-Dorfman disease complicated by autoimmune haemolytic anaemia: case report and review of a multisystem disease with cutaneous infiltrates. (11531802)
2001
45
Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease) in a girl previously affected by acute lymphoblastic leukemia. (11496358)
2001
46
Rosai-Dorfman disease of the breast: a mimic of breast malignancy. (10740798)
2000
47
Nasal manifestation of extranodal Rosai-Dorfman disease--diagnosis and management. (10435144)
1999
48
Sinus histiocytosis (Rosai-Dorfman disease) clinically limited to the skin. (10494712)
1999
49
Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease). (9725535)
1998
50
Cutaneous Rosai-Dorfman disease. (9667110)
1998

Genetic Variations for Rosai-Dorfman Disease

Expression for genes affiliated with Rosai-Dorfman Disease

Sources:
1BioGPS, 15Gene Expression Omnibus DataSets
See all sources
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

Sources:
38NCBI BioSystems Database
See all sources

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

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

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

(show top 50)    (show all 68)
idCompoundScoreTop Affiliating Genes
1cycloheximide4510.3ALPP
2iron sucrose4510.1CRP, TF
3p0034510.1S100B, CTSD
4sialic acid4510.1SRF
5nh4cl459.9CTSD, TF, CRP
6phosphoethanolamine459.9CRP, ALPP
7aspartate459.8PDGFRA, CTSD, ALPP
8betacarotene459.8SERPINA3, CRP, TF
9olopatadine45 1110.8S100B, S100A1
10vitamin a45 11 2411.7SERPINA3, SRF
11alpha tocopherol459.7CTSD, TF, CRP
12polysulfone459.7SRF, F3
13silver459.7SERPINA3, CTSD, S100A1
14glutamate459.7SRF
15guanidine hydrochloride459.6F3, SERPINA3, ALPP
16cyclophosphamide45 50 1111.6F3, CRP, TF, CTSD
17citrate459.6F3, CRP, TF, S100B
18b 723459.6S100B, S100A1, ALPP
19polyethylene glycol459.5ALPP, CRP, TF
20zinc protoporphyrin459.5CRP, TF
21vitamin b12459.4TF, CRP, SERPINA3, ALPP
22heparin45 29 11 2412.3SERPINA3, SRF, CTSD, S100B
23nocodazole45 1110.3SRF, TF, CTSD
24biotin45 11 2411.3S100A1, TF, CRP
25acetylcholine45 50 29 11 2413.3SERPINA3, SRF, S100A1
26cyclosporin a45 29 6011.2S100B, CTSD, TF, SRF
27ascorbic acid45 2410.2S100B, CTSD, TF, CRP, ALPP
28folate459.1PDGFRA, TF, CRP, SERPINA3, ALPP
29methotrexate45 50 1111.0TF, CRP, SRF, SERPINA3
30latex458.9ALPP, SRF, CRP, CTSD
31homocysteine45 249.8SRF, F3, CRP
32phospholipid458.8S100B, S100A1, F3, SRF
33fibrinogen458.7TF, CRP, F3, SRF, SERPINA3
34thymidine45 249.7S100B, PDGFRA, CTSD, SRF, ALPP
35dexamethasone45 50 29 1111.7S100B, CD163, SRF, ALPP
36progesterone45 60 29 11 2412.7S100B, S100A1, CTSD, SRF, SERPINA3
37cysteine458.6S100B, S100A1, CTSD, F3, SERPINA3, ALPP
38norepinephrine45 11 2410.6S100B, TF, CRP, SRF, ALPP
39testosterone45 60 11 2411.6ALPP, SRF, CRP, CTSD, S100B
40zinc45 249.5S100B, S100A1, CTSD, SRF, SERPINA3
41serine458.5S100B, FSCN1, SRF, SERPINA3, ALPP
42lactate458.4S100B, CTSD, TF, CRP, SRF, ALPP
43cholesterol45 29 11 2411.3CTSD, TF, CRP, SRF, SERPINA3, ALPP
44creatinine458.0CTSD, TF, CRP, F3, SRF, SERPINA3
45iron45 248.9S100B, S100A1, TF, CRP, CD163, SRF
46alanine457.8S100B, CTSD, TF, CRP, F3, SRF
47vegf457.7S100B, PDGFRA, CTSD, TF, CRP, F3
48paraffin457.6ALPP, S100B, S100A1, PDGFRA, CTSD, TF
49retinoic acid45 248.2S100B, S100A1, PDGFRA, CTSD, F3, FSCN1
50calcium45 50 11 249.7S100B, S100A1, PDGFRA, CTSD, CRP, CD163

GO Terms for genes affiliated with Rosai-Dorfman Disease

Sources:
16Gene Ontology
See all sources

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

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

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

  • Antibodies
  • Proteins
  • Lysates
  • Antibodies

Sources for Rosai-Dorfman Disease

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