MCID: INT221
MIFTS: 53

Intravascular Large B-Cell Lymphoma malady

Blood diseases, Cancer diseases, Rare diseases, Immune diseases categories

Summaries for Intravascular Large B-Cell Lymphoma

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34MalaCards
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MalaCards: Intravascular Large B-Cell Lymphoma, also known as angioendotheliomatosis proliferans systemisata, is related to b-cell lymphomas and pancytopenia. An important gene associated with Intravascular Large B-Cell Lymphoma is BCL6 (B-cell CLL/lymphoma 6), and among its related pathways are Inflammatory Response Pathway and B Cell Receptor Signaling Pathway. The compounds lentinan and mycophenolate have been mentioned in the context of this disorder. Affiliated tissues include b cells, bone and skin, and related mouse phenotypes are tumorigenesis and liver/biliary system.

Aliases & Classifications for Intravascular Large B-Cell Lymphoma

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50Orphanet, 27ICD10 via Orphanet, 60SNOMED-CT via Orphanet
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Classifications:

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


Aliases & Descriptions:

intravascular large b-cell lymphoma 50
angioendotheliomatosis proliferans systemisata 50
malignant angioendotheliomatosis 50
angiotropic large cell lymphoma 50
intravascular lymphomatosis 50
tappeiner-pfleger disease 50


External Ids:

ICD10 via Orphanet27 C83.3
SNOMED-CT via Orphanet60 399648005

Related Diseases for Intravascular Large B-Cell Lymphoma

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18GeneCards, 19GeneDecks
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Diseases related to Intravascular Large B-Cell Lymphoma via text searches within MalaCards or GeneCards/GeneDecks gene sharing:

(show top 50)    (show all 148)
idRelated DiseaseScoreTop Affiliating Genes
1b-cell lymphomas31.8TNFRSF8, KMT2A, CXCR3, CCND1, ACPP, BCL6
2pancytopenia30.8IL2RA, KMT2A
3systemic lupus erythematosus30.5IL2RB, IL2RA, MPO, CXCR3
4anaplastic large cell lymphoma30.4BCL6, IL2RB, CXCR3, IL2RA, TNFRSF8
5hodgkin's lymphoma30.3TNFRSF8, BCL2, BCL6, IL2RB, IL2RA, KMT2A
6breast cancer30.2KMT2A, TNFRSF8, BCL6, BCL2, MME, ACPP
7prostatitis10.7
8cerebritis10.7
9senile angioma10.6
10diffuse large b-cell lymphoma10.6
11hemangioma10.6
12cholecystitis10.5
13dementia10.5
14hypopituitarism10.5
15meningitis10.5
16panniculitis10.5
17proteinuria10.5
18malignant histiocytosis10.3
19histiocytosis10.3
20mononeuritis multiplex10.3
21protein-losing enteropathy10.3
22intestinal obstruction10.3
23mononeuritis10.3
24nephrotic syndrome10.3
25vasculitis10.3
26superior vena cava syndrome10.3
27myoma10.3
28splenic infarction10.3
29choroiditis10.3
30sagittal sinus thrombosis10.3
31hemolytic anemia10.3
32hepatitis10.3
33hypertension10.3
34interstitial lung disease10.3
35ischemia10.3
36lupus erythematosus10.3
37meningioma10.3
38mononeuropathy10.3
39myopathy10.3
40thrombocytopenia10.3
41thyroiditis10.3
42ring chromosome 1110.3
43syndrome of inappropriate antidiuretic hormone10.3
44aneurysm10.3
45blindness10.3
46alk-positive anaplastic large cell lymphoma10.3
47acute disseminated encephalomyelitis10.2
48kaposi's sarcoma10.2
49pseudobulbar palsy10.2
50adult respiratory distress syndrome10.2

Graphical network of the top 20 diseases related to Intravascular Large B-Cell Lymphoma:



Diseases related to intravascular large b-cell lymphoma

Symptoms for Intravascular Large B-Cell Lymphoma

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Drugs & Therapeutics for Intravascular Large B-Cell Lymphoma

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6CenterWatch, 43NIH Clinical Center, 7ClinicalTrials
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Approved drugs:

Search CenterWatch for Intravascular Large B-Cell Lymphoma

Drug clinical trials:

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Search NIH Clinical Center for Intravascular Large B-Cell Lymphoma

Search CenterWatch for Intravascular Large B-Cell Lymphoma

Genetic Tests for Intravascular Large B-Cell Lymphoma

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Anatomical Context for Intravascular Large B-Cell Lymphoma

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34MalaCards
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MalaCards organs/tissues related to Intravascular Large B-Cell Lymphoma:

34
B cells, Bone, Skin, Bone marrow, Lung, Prostate, Uterus, Liver, Kidney, Brain, Breast, Colon, Thyroid, Spleen, Pituitary

Animal Models for Intravascular Large B-Cell Lymphoma or affiliated genes

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38MGI
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MGI Mouse Phenotypes related to Intravascular Large B-Cell Lymphoma:

38
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00020067.9BCL2, CCND1, CXCR3, KMT2A, B2M
2MP:00053707.8KMT2A, MME, IL2RB, BCL6, B2M
3MP:00053897.5IL2RB, BCL6, CCND1, KMT2A, B2M, BCL2
4MP:00107717.4CCND1, BCL2, MME, KMT2A, B2M, ACPP
5MP:00053857.3CXCR3, MPO, KMT2A, CCND1, BCL6, BCL2
6MP:00053787.3CCND1, B2M, IL2RA, IL2RB, BCL6, BCL2
7MP:00053766.9CCND1, BCL2, CXCR3, MME, KMT2A, MPO
8MP:00107686.6BCL2, BCL6, IL2RB, CCND1, CXCR3, KMT2A
9MP:00053975.9BCL2, B2M, TNFRSF8, MPO, KMT2A, CXCR3
10MP:00053875.5CCND1, BCL6, IL2RB, IL2RA, B2M, TNFRSF8

Publications for Intravascular Large B-Cell Lymphoma

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53PubMed
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Articles related to Intravascular Large B-Cell Lymphoma:

(show top 50)    (show all 171)
idTitleAuthorsYear
1
Intravascular large B-cell lymphoma confirmed by brain biopsy: a case report. (23420723)
2013
2
Intravascular large B-cell lymphoma presenting with anasarca-type edema and acute renal failure. (23885711)
2013
3
Liver dysfunction and thrombocytopenia diagnosed as intravascular large B-cell lymphoma using a timely and accurate transjugular liver biopsy. (23994980)
2013
4
Intravascular large B-cell lymphoma presenting as acute hemorrhagic cerebral infarct with delirium. (23884226)
2013
5
Mild proteinuria in a patient with glomerularlimited intravascular large B-cell lymphoma. (22541680)
2013
6
Generalized telangiectasia revealing intravascular large B-cell lymphoma. (23406023)
2013
7
Intravascular large B cell lymphoma presenting as cholecystitis: diagnostic challenges persist. (24288109)
2013
8
SIADH and partial hypopituitarism in a patient with intravascular large B-cell lymphoma: a rare cause of a common presentation. (23362070)
2013
9
Intravascular large B-cell lymphoma presenting with fulminant pseudomembranous colitis. (23611409)
2013
10
Intravascular large B-cell lymphoma with hemophagocytic syndrome (Asian variant) in a Caucasian patient. (22808298)
2012
11
Indication for random skin biopsy for the diagnosis of intravascular large B cell lymphoma. (22414723)
2012
12
Diffuse pulmonary uptake on FDG-PET with normal CT diagnosed as intravascular large B-cell lymphoma: a case report and a discussion of the causes of diffuse FDG uptake in the lungs. (22275725)
2012
13
A fatal case of hemoperitoneum after ultrasound-guided liver biopsy in a patient with intravascular large B-cell lymphoma. (21377915)
2011
14
An autopsy case of lymphomatosis cerebri showing pathological changes of intravascular large B-cell lymphoma in visceral organs. (21382094)
2011
15
Progressive multifocal cerebral infarction from intravascular large B cell lymphoma presenting in a man: a case report. (21251318)
2011
16
Haemophagocytosis-related intravascular large B-cell lymphoma associated with skin eruption. (21279301)
2011
17
Intravascular large B-cell lymphoma of the uterus: a case with favorable clinical outcome. (19948641)
2011
18
Frequency of intravascular large B-cell lymphoma in Japan: study of the Osaka Lymphoma Study Group. (21481222)
2011
19
18F-FDG PET/CT in a case of intravascular large B-cell lymphoma. (20607536)
2010
20
Intravascular large B-cell lymphoma, an exclusively small vessel disease? A case report and review of literature. (20542564)
2010
21
Central nervous system involvement in intravascular large B-cell lymphoma: a retrospective analysis of 109 patients. (20412122)
2010
22
Neurolymphomatosis involving the trigeminal nerve and deep peroneal nerve in a patient with relapsed intravascular large B-cell lymphoma. (20528901)
2010
23
Fluorodeoxyglucose-PET/CT for diagnosis of intravascular large B-cell lymphoma. (20675506)
2010
24
Intravascular large B cell lymphoma: an elusive cause of pyrexia of unknown origin diagnosed postmortem. (20868278)
2010
25
Intravascular large B-cell lymphoma within a subcutaneous cavernous haemangioma. (20735401)
2010
26
Intravascular large B-cell lymphoma of the skin: typical clinical manifestations and a favourable response to rituximab-containing therapy. (19590171)
2009
27
Intravascular large B cell lymphoma: proposed of the strategy for early diagnosis and treatment of patients with rapid deteriorating condition. (19363707)
2009
28
Gallium scan-prompted skin biopsy revealed intravascular large B-cell lymphoma in a patient who presented with fever of unknown origin. (19387217)
2009
29
Intravascular large B cell lymphoma: when lymphoma is suspected but routine diagnostic work-up is negative. (19883320)
2009
30
Intravascular large B-cell lymphoma: remission after rituximab-cyclophosphamide, doxorubicin, vincristine, and prednisolone chemotherapy. (19632742)
2009
31
Intravascular large B-cell lymphoma presenting in subcutaneous fat tissue and simulating panniculitis clinically. (20415677)
2009
32
Analysis of CXCL9 and CXCR3 expression in a case of intravascular large B-cell lymphoma. (19766346)
2009
33
HHV8-positive, EBV-positive Hodgkin lymphoma-like large B-cell lymphoma and HHV8-positive intravascular large B-cell lymphoma. (19287457)
2009
34
The addition of rituximab to anthracycline-based chemotherapy significantly improves outcome in 'Western' patients with intravascular large B-cell lymphoma. (18699850)
2008
35
Sustained remission after rituximab-containing chemotherapy for intravascular large B-cell lymphoma. (18434690)
2008
36
Prolonged survival using anti-CD20 combined chemotherapy in primary prostatic intravascular large B-cell lymphoma. (18493873)
2008
37
Can rituximab change the usually dismal prognosis of patients with intravascular large B-cell lymphoma? (18838697)
2008
38
Definition, diagnosis, and management of intravascular large B-cell lymphoma: proposals and perspectives from an international consensus meeting. (17577023)
2007
39
Intravascular large B-cell lymphoma presenting as cerebellar and cerebral infarction. (17502477)
2007
40
Asian variant of intravascular large B-cell lymphoma diagnosed by bone marrow biopsy]. (17313078)
2007
41
Intravascular large B-cell lymphoma of the uterus presenting as fever of unknown origin (FUO) and revealed by FDG-PET. (17672184)
2007
42
Intravascular large B-cell lymphoma with multisystem involvement. (16966288)
2006
43
Intravascular large B-cell lymphoma associated with a near-tetraploid karyotype, rearrangement of BCL6, and a t(11;14)(q13;q32). (17116487)
2006
44
Primary bilateral adrenal intravascular large B-cell lymphoma associated with adrenal failure. (12879956)
2003
45
A case of intravascular large B-cell lymphoma with multiple organ involvement. (14739519)
2003
46
Intravascular large B-cell lymphoma presenting as cutaneous panniculitis. (14677722)
2003
47
Intravascular large B-cell lymphoma with a fulminant clinical course: a case report with definite diagnosis post mortem. (12196378)
2002
48
Myeloperoxidase-positive intravascular large B-cell lymphoma. (11419984)
2001
49
Unusual sites of involvement by hematologic malignancies. Case 1. Intravascular large B-cell lymphoma presenting with CNS symptoms. (11579120)
2001
50
Primary cutaneous large B-cell lymphoma of the leg relapsing as cutaneous intravascular large B-cell lymphoma. (11735727)
2001

Variations for Intravascular Large B-Cell Lymphoma

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Expression for genes affiliated with Intravascular Large B-Cell Lymphoma

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2BioGPS, 16Gene Expression Omnibus DataSets
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Expression patterns in normal tissues for genes affiliated with Intravascular Large B-Cell Lymphoma

Search GEO for disease gene expression data for Intravascular Large B-Cell Lymphoma.

Pathways for genes affiliated with Intravascular Large B-Cell Lymphoma

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Sources:
51PathCards, 39NCBI BioSystems Database, 61Thomson Reuters, 54QIAGEN, 55R&D Systems, 31KEGG, 58SinoBiological, 5Cell Signaling Technology
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Pathways related to Intravascular Large B-Cell Lymphoma according to GeneCards/GeneDecks:

(show all 25)
idSuper pathways (with members indented)ScoreTop Affiliating Genes
19.8IL2RB, IL2RA
29.7BCL6, BCL2, CD5
3
Show member pathways
Immune response IL 22 signaling pathway61
G protein signaling N RAS regulation pathway61
9.5BCL2, IL2RB, IL2RA
4
Show member pathways
9.5BCL2, IL2RB, IL2RA
5
Show member pathways
IL-2 Signaling pathway39
9.5BCL2, IL2RB, IL2RA
6
Show member pathways
IL2 signaling events mediated by STAT539
9.5IL2RA, IL2RB, BCL2
79.4MME, IL2RA, CD5
89.3BCL2, BCL6, CCND1
9
Show member pathways
DNA damage response (only ATM dependent)39
Wnt Signaling Pathway and Pluripotency39
9.3CCND1, BCL6, BCL2
10
Show member pathways
Immune response IL 12 induced IFN gamma production61
9.2IL2RB, IL2RA, B2M
11
Show member pathways
Ras signaling in the CD4+ TCR pathway39
9.2IL2RB, IL2RA, B2M
12
Show member pathways
9.2B2M, TNFRSF8, BCL2
139.1IL2RB, IL2RA, CCND1
14
Show member pathways
Type III interferon signaling39
9.1CCND1, IL2RA, IL2RB
15
Show member pathways
IL-9 Signaling Pathway39
Development Thrombopoietin regulated cell processes61
IL-7 Signaling Pathway39
Immune response IL 9 signaling pathway61
9.1CCND1, IL2RA, IL2RB
169.1MPO, CCND1, BCL2
17
Show member pathways
9.0B2M, CCND1, IL2RA
188.9CCND1, IL2RA, IL2RB, BCL2
19
Show member pathways
8.9BCL2, IL2RB, IL2RA, CCND1
20
Show member pathways
8.6TNFRSF8, CXCR3, IL2RA, IL2RB, BCL2
21
Show member pathways
8.6TNFRSF8, CXCR3, IL2RA, IL2RB, BCL2
228.6MPO, KMT2A, IL2RB, BCL6
238.1BCL6, IL2RB, IL2RA, MME, MPO, B2M
24
Show member pathways
8.0TNFRSF8, CXCR3, CCND1, IL2RA, IL2RB, BCL2
25
Show member pathways
8.0TNFRSF8, CXCR3, CCND1, IL2RA, IL2RB, BCL2

Compounds for genes affiliated with Intravascular Large B-Cell Lymphoma

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Sources:
46Novoseek, 52PharmGKB, 12DrugBank, 3BitterDB, 30IUPHAR, 25HMDB, 62Tocris Bioscience
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Compounds related to Intravascular Large B-Cell Lymphoma according to GeneCards/GeneDecks:

(show top 50)    (show all 121)
idCompoundScoreTop Affiliating Genes
1lentinan4610.1IL2RA, IL2RB
2mycophenolate4610.0IL2RB, IL2RA
3ricin469.7IL2RB, CD5, TNFRSF8
4il 10469.6IL2RA, IL2RB, CXCR3
52-chlorodeoxyadenosine469.6IL2RA, BCL2, B2M
6cuprophan469.6IL2RB, B2M, IL2RA
7ginsenoside469.6MME, CCND1, BCL2
8polysulfone469.6IL2RB, IL2RA, B2M
9infliximab46 52 1211.5IL2RB, CXCR3, IL2RA
102,5-oligoadenylate469.5B2M, IL2RA, IL2RB
11rituximab46 52 1211.5IL2RA, BCL2, B2M, BCL6
12ifn-alpha469.4CXCR3, IL2RB, IL2RA
13methacholine46 52 1211.4MME, IL2RB, MPO
14methimazole46 3 1211.4IL2RA, B2M, MPO
15neopterin469.3ACPP, B2M, IL2RA, IL2RB
16erythromycin46 3 52 1212.3IL2RA, MPO, IL2RB
17pentoxifylline46 1210.2MPO, IL2RB, IL2RA
18tacrolimus46 52 1211.1CCND1, IL2RB, IL2RA, CXCR3
19adriamycin469.0CCND1, B2M, MME, BCL2
20chloride468.9MME, B2M, IL2RB, MPO
21histamine46 30 2510.9CXCR3, MME, MPO, IL2RA, IL2RB
22prednisolone46 30 1210.8MPO, IL2RB, IL2RA, KMT2A
23formaldehyde46 259.8B2M, MPO, CCND1, IL2RA
24n acetylcysteine468.8IL2RB, BCL2, CCND1, MPO, IL2RA
25cyclophosphamide46 52 1210.7IL2RA, IL2RB, B2M, BCL2, BCL6, MPO
26cyclosporin a46 30 6210.6MME, IL2RA, B2M, IL2RB, TNFRSF8
27progesterone46 30 62 25 1212.6MME, CCND1, BCL2, IL2RB, ACPP, CXCR3
28lactate468.6IL2RB, MME, ACPP, MPO, B2M, BCL6
29phosphatidylinositol468.5MME, CXCR3, CCND1, IL2RA, IL2RB, BCL2
30pge2468.5IL2RB, IL2RA, MME, MPO, B2M, CXCR3
31cycloheximide468.5IL2RB, B2M, BCL6, MME, CCND1, BCL2
32indomethacin46 30 62 1211.4B2M, IL2RA, MPO, MME
33aspartate468.4KMT2A, IL2RA, IL2RB, ACPP, B2M
34testosterone46 62 25 1211.3MME, CCND1, ACPP, B2M, IL2RA
35arginine468.3CXCR3, ACPP, IL2RA, IL2RB, MME, KMT2A
36creatinine468.2B2M, MME, CXCR3, ACPP, IL2RB, MPO
37vincristine46 52 1210.1KMT2A, IL2RA, BCL6, B2M, BCL2, CCND1
38cisplatin46 52 62 1211.0IL2RB, IL2RA, ACPP, MPO, BCL2, CCND1
39steroid468.0CXCR3, CCND1, ACPP, IL2RA, IL2RB, MME
40genistein46 30 62 3 25 1212.9IL2RB, B2M, BCL2, KMT2A, CCND1, IL2RA
41zinc46 258.9MME, TNFRSF8, BCL6, KMT2A, IL2RB, IL2RA
42calcium46 52 25 1210.7CXCR3, IL2RB, CD5, MME, ACPP, B2M
43doxorubicin46 52 129.6B2M, KMT2A, MME, CCND1, IL2RA, BCL2
44thymidine46 258.5B2M, MME, TNFRSF8, IL2RB, IL2RA, ACPP
45vegf467.3ACPP, BCL2, B2M, MPO, MME, CXCR3
46retinoic acid46 258.2BCL6, B2M, MPO, KMT2A, MME, BCL2
47estrogen467.0B2M, BCL2, IL2RA, BCL6, ACPP, CCND1
48paraffin466.9BCL6, ACPP, CD5, BCL2, IL2RA, B2M
49tyrosine466.7MME, KMT2A, CD5, CXCR3, BCL6, ACPP
50serine466.1MPO, KMT2A, CXCR3, CCND1, ACPP, IL2RB

GO Terms for genes affiliated with Intravascular Large B-Cell Lymphoma

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17Gene Ontology
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Cellular components related to Intravascular Large B-Cell Lymphoma according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1external side of plasma membraneGO:0098978.5CD5, IL2RB, IL2RA, CXCR3, B2M
2extracellular vesicular exosomeGO:0700628.3ACPP, MME, MPO, TNFRSF8, B2M
3plasma membraneGO:0058867.1B2M, TNFRSF8, MME, CXCR3, ACPP, IL2RA

Biological processes related to Intravascular Large B-Cell Lymphoma according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1positive regulation of B cell proliferationGO:03089010.0BCL2, BCL6
2response to iron ionGO:0100399.7BCL2, CCND1
3T cell differentiation in thymusGO:0330779.4BCL2, B2M
4cellular response to DNA damage stimulusGO:0069749.3BCL2, BCL6, CCND1
5apoptotic processGO:0069158.6KMT2A, CXCR3, IL2RA, BCL2

Molecular functions related to Intravascular Large B-Cell Lymphoma according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1interleukin-2 receptor activityGO:0049119.7IL2RB, IL2RA
2interleukin-2 bindingGO:0199769.5IL2RB, IL2RA
3identical protein bindingGO:0428028.6BCL2, ACPP, KMT2A, B2M

Products for genes affiliated with Intravascular Large B-Cell Lymphoma

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  • Antibodies
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Sources for Intravascular Large B-Cell Lymphoma

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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