MCID: INT221
MIFTS: 53

Intravascular Large B-Cell Lymphoma malady

Blood diseases, Cancer diseases, Rare diseases, Immune diseases categories
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Summaries for Intravascular Large B-Cell Lymphoma

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33MalaCards
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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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49Orphanet, 26ICD10 via Orphanet, 59SNOMED-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 49
angioendotheliomatosis proliferans systemisata 49
malignant angioendotheliomatosis 49
angiotropic large cell lymphoma 49
intravascular lymphomatosis 49
tappeiner-pfleger disease 49


External Ids:

ICD10 via Orphanet26 C83.3
SNOMED-CT via Orphanet59 399648005

Related Diseases for Intravascular Large B-Cell Lymphoma

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17GeneCards, 18GeneDecks
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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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42NIH Clinical Center, 6ClinicalTrials
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Drug clinical trials:

Search ClinicalTrials for Intravascular Large B-Cell Lymphoma

Search NIH Clinical Center 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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33MalaCards
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MalaCards organs/tissues related to Intravascular Large B-Cell Lymphoma:

33
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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37MGI
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MGI Mouse Phenotypes related to Intravascular Large B-Cell Lymphoma:

37
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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52PubMed
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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, 15Gene 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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50PathCards, 38NCBI BioSystems Database, 60Thomson Reuters, 53QIAGEN, 54R&D Systems, 30KEGG, 57SinoBiological, 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 pathway60
G protein signaling N RAS regulation pathway60
9.5BCL2, IL2RB, IL2RA
4
Show member pathways
9.5BCL2, IL2RB, IL2RA
5
Show member pathways
IL-2 Signaling pathway38
9.5BCL2, IL2RB, IL2RA
6
Show member pathways
IL2 signaling events mediated by STAT538
9.5IL2RA, IL2RB, BCL2
79.4MME, IL2RA, CD5
89.3BCL2, BCL6, CCND1
9
Show member pathways
DNA damage response (only ATM dependent)38
Wnt Signaling Pathway and Pluripotency38
9.3CCND1, BCL6, BCL2
10
Show member pathways
Immune response IL 12 induced IFN gamma production60
9.2IL2RB, IL2RA, B2M
11
Show member pathways
Ras signaling in the CD4+ TCR pathway38
9.2IL2RB, IL2RA, B2M
12
Show member pathways
9.2B2M, TNFRSF8, BCL2
139.1IL2RB, IL2RA, CCND1
14
Show member pathways
Type III interferon signaling38
9.1CCND1, IL2RA, IL2RB
15
Show member pathways
IL-9 Signaling Pathway38
Development Thrombopoietin regulated cell processes60
IL-7 Signaling Pathway38
Immune response IL 9 signaling pathway60
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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45Novoseek, 51PharmGKB, 11DrugBank, 3BitterDB, 29IUPHAR, 24HMDB, 61Tocris 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
1lentinan4510.1IL2RA, IL2RB
2mycophenolate4510.0IL2RB, IL2RA
3ricin459.7IL2RB, CD5, TNFRSF8
4il 10459.6IL2RA, IL2RB, CXCR3
52-chlorodeoxyadenosine459.6IL2RA, BCL2, B2M
6cuprophan459.6IL2RB, B2M, IL2RA
7ginsenoside459.6MME, CCND1, BCL2
8polysulfone459.6IL2RB, IL2RA, B2M
9infliximab45 51 1111.5IL2RB, CXCR3, IL2RA
102,5-oligoadenylate459.5B2M, IL2RA, IL2RB
11rituximab45 51 1111.5IL2RA, BCL2, B2M, BCL6
12ifn-alpha459.4CXCR3, IL2RB, IL2RA
13methacholine45 51 1111.4MME, IL2RB, MPO
14methimazole45 3 1111.4IL2RA, B2M, MPO
15neopterin459.3ACPP, B2M, IL2RA, IL2RB
16erythromycin45 3 51 1112.3IL2RA, MPO, IL2RB
17pentoxifylline45 1110.2MPO, IL2RB, IL2RA
18tacrolimus45 51 1111.1CCND1, IL2RB, IL2RA, CXCR3
19adriamycin459.0CCND1, B2M, MME, BCL2
20chloride458.9MME, B2M, IL2RB, MPO
21histamine45 29 2410.9CXCR3, MME, MPO, IL2RA, IL2RB
22prednisolone45 29 1110.8MPO, IL2RB, IL2RA, KMT2A
23formaldehyde45 249.8B2M, MPO, CCND1, IL2RA
24n acetylcysteine458.8IL2RB, BCL2, CCND1, MPO, IL2RA
25cyclophosphamide45 51 1110.7IL2RA, IL2RB, B2M, BCL2, BCL6, MPO
26cyclosporin a45 29 6110.6MME, IL2RA, B2M, IL2RB, TNFRSF8
27progesterone45 29 61 24 1112.6MME, CCND1, BCL2, IL2RB, ACPP, CXCR3
28lactate458.6IL2RB, MME, ACPP, MPO, B2M, BCL6
29phosphatidylinositol458.5MME, CXCR3, CCND1, IL2RA, IL2RB, BCL2
30pge2458.5IL2RB, IL2RA, MME, MPO, B2M, CXCR3
31cycloheximide458.5IL2RB, B2M, BCL6, MME, CCND1, BCL2
32indomethacin45 29 61 1111.4B2M, IL2RA, MPO, MME
33aspartate458.4KMT2A, IL2RA, IL2RB, ACPP, B2M
34testosterone45 61 24 1111.3MME, CCND1, ACPP, B2M, IL2RA
35arginine458.3CXCR3, ACPP, IL2RA, IL2RB, MME, KMT2A
36creatinine458.2B2M, MME, CXCR3, ACPP, IL2RB, MPO
37vincristine45 51 1110.1KMT2A, IL2RA, BCL6, B2M, BCL2, CCND1
38cisplatin45 51 61 1111.0IL2RB, IL2RA, ACPP, MPO, BCL2, CCND1
39steroid458.0CXCR3, CCND1, ACPP, IL2RA, IL2RB, MME
40genistein45 29 61 3 24 1112.9IL2RB, B2M, BCL2, KMT2A, CCND1, IL2RA
41zinc45 248.9MME, TNFRSF8, BCL6, KMT2A, IL2RB, IL2RA
42calcium45 51 24 1110.7CXCR3, IL2RB, CD5, MME, ACPP, B2M
43doxorubicin45 51 119.6B2M, KMT2A, MME, CCND1, IL2RA, BCL2
44thymidine45 248.5B2M, MME, TNFRSF8, IL2RB, IL2RA, ACPP
45vegf457.3ACPP, BCL2, B2M, MPO, MME, CXCR3
46retinoic acid45 248.2BCL6, B2M, MPO, KMT2A, MME, BCL2
47estrogen457.0B2M, BCL2, IL2RA, BCL6, ACPP, CCND1
48paraffin456.9BCL6, ACPP, CD5, BCL2, IL2RA, B2M
49tyrosine456.7MME, KMT2A, CD5, CXCR3, BCL6, ACPP
50serine456.1MPO, KMT2A, CXCR3, CCND1, ACPP, IL2RB

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

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16Gene 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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Sources for Intravascular Large B-Cell Lymphoma

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4CDC
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
52PubMed
53QIAGEN
59SNOMED-CT via Orphanet
62UMLS
63UMLS via Orphanet