MCID: THR005
MIFTS: 58

Thrombotic Thrombocytopenic Purpura malady

Genetic diseases, Blood diseases, Rare diseases, Nephrological diseases, Immune diseases categories
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Summaries for Thrombotic Thrombocytopenic Purpura

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MalaCards: Thrombotic Thrombocytopenic Purpura, also known as ttp - thrombotic thrombocytopenic purpura, is related to purpura and hemolytic-uremic syndrome. An important gene associated with Thrombotic Thrombocytopenic Purpura is ADAMTS13 (ADAM metallopeptidase with thrombospondin type 1 motif, 13), and among its related pathways are Translocation of ZAP-70 to Immunological synapse and TCR signaling. The compounds ticlopidine and beraprost sodium have been mentioned in the context of this disorder. Affiliated tissues include endothelial, liver and bone marrow, and related mouse phenotypes are digestive/alimentary and immune system.

Aliases & Classifications for Thrombotic Thrombocytopenic Purpura

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8Disease Ontology, 62UMLS, 58SNOMED-CT, 35MeSH, 40NCIt, 25ICD10
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Classifications:



Aliases & Descriptions:

thrombotic thrombocytopenic purpura 8
ttp - thrombotic thrombocytopenic purpura 8
purpura, thrombotic thrombocytopenic 62
thrombotic microangiopathy nos 8
thrombotic microangiopathies 62
thrombotic microangiopathy 8
moschcowitz's syndrome 8


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Disease Ontology8 DOID:10772
MeSH35 D011697
NCIt40 C78797
ICD1025 M31.1

Related Diseases for Thrombotic Thrombocytopenic Purpura

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17GeneCards, 18GeneDecks
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Diseases in the Thrombotic Thrombocytopenic Purpura, Congenital family:

thrombotic thrombocytopenic purpura Thrombotic Thrombocytopenic Purpura, Acquired

Diseases related to Thrombotic Thrombocytopenic Purpura via text searches within MalaCards or GeneCards/GeneDecks gene sharing:

(show top 50)    (show all 201)
idRelated DiseaseScoreTop Affiliating Genes
1purpura32.7ADAMTS13
2hemolytic-uremic syndrome31.7VWF, ADAMTS13, THBD
3systemic lupus erythematosus31.3THBD, VWF, ADAMTS13
4thrombotic thrombocytopenic purpura, acquired31.3VWF, THBD, THBS1, ADAMTS13
5connective tissue disease31.1THBD, VWF
6thrombocytopenia31.1VWF, THBD, ADAMTS13, WAS
7hemolytic anemia31.0ADAMTS13, VWF
8myocardial infarction30.9SERPINF2, VWF, ADAMTS13
9disseminated intravascular coagulation30.9ADAMTS13, THBD, SERPINF2
10antiphospholipid syndrome30.9VWF, ADAMTS13, THBD
11hellp syndrome30.8ADAMTS13, VWF
12lupus nephritis30.7THBD, ADAMTS13
13vasculitis30.6VWF, THBD
14sepsis30.6SERPINF2, THBD, ADAMTS13
15acute myocardial infarction30.5THBD, SERPINF2, VWF, ADAMTS13
16malaria30.3ADAMTS13
17thrombophilia30.3SERPINF2, THBD, VWF
18pre-eclampsia30.3ADAMTS13, VWF, SERPINF2, THBD
19vascular disease30.2THBS1, THBD, VWF, SERPINF2
20sickle cell disease30.2THBS1, VWF
21lupus erythematosus10.9
22pancreatitis10.8
23retinitis10.8
24thrombotic thrombocytopenic purpura, congenital10.7
25endotheliitis10.7
26acute pancreatitis10.7
27adult-onset still's disease10.7
28hepatitis10.7
29mixed connective tissue disease10.6
30retinal detachment10.6
31hepatitis c10.6
32multiple myeloma10.6
33myeloma10.6
34hypertension10.6
35bone marrow necrosis10.6
36hematopoietic stem cell transplantation10.5
37hemoglobin c disease10.5
38central retinal vein occlusion10.5
39cerebritis10.5
40adenocarcinoma10.5
41glomerulonephritis10.5
42influenza10.5
43malignant hypertension10.5
44prostatitis10.5
45retinal vein occlusion10.5
46scleroderma10.5
47nephritis10.5
48sickle cell - hemoglobin c disease10.5
49catastrophic antiphospholipid syndrome10.5
50graft versus host disease10.4

Graphical network of the top 20 diseases related to Thrombotic Thrombocytopenic Purpura:



Diseases related to thrombotic thrombocytopenic purpura

Symptoms for Thrombotic Thrombocytopenic Purpura

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Drugs & Therapeutics for Thrombotic Thrombocytopenic Purpura

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42NIH Clinical Center, 6ClinicalTrials
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Drug clinical trials:

Search ClinicalTrials for Thrombotic Thrombocytopenic Purpura

Search NIH Clinical Center for Thrombotic Thrombocytopenic Purpura

Genetic Tests for Thrombotic Thrombocytopenic Purpura

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Anatomical Context for Thrombotic Thrombocytopenic Purpura

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33MalaCards
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MalaCards organs/tissues related to Thrombotic Thrombocytopenic Purpura:

33
Endothelial, Liver, Bone marrow, Testes, Bone, Lung, Kidney, B cells, Heart, Prostate, Retina, Breast, Colon, Pancreas, Brain, Pancreatic islet, Pituitary, Cortex

Animal Models for Thrombotic Thrombocytopenic Purpura or affiliated genes

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37MGI
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MGI Mouse Phenotypes related to Thrombotic Thrombocytopenic Purpura:

37
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00053818.1VWF, THBD, THBS1, WAS
2MP:00053878.0WAS, THBS1, THBD, VWF, ADAMTS13
3MP:00053977.9THBD, THBS1, WAS, ADAMTS13, VWF
4MP:00053767.2ADAMTS13, VWF, THBD, THBS1, SERPINF2, WAS

Publications for Thrombotic Thrombocytopenic Purpura

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52PubMed
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Articles related to Thrombotic Thrombocytopenic Purpura:

(show top 50)    (show all 705)
idTitleAuthorsYear
1
Thrombotic thrombocytopenic purpura and other thrombotic microangiopathic hemolytic anemias: Diagnosis and classification. (24418304)
2014
2
Thrombotic thrombocytopenic purpura in children. (23422353)
2013
3
Post-cardiac surgery thrombotic thrombocytopenic purpura with digital ischaemia. (23659403)
2013
4
Acquired thrombotic thrombocytopenic purpura (TTP) induced by silicone breast implants in 37-year old woman. (23477708)
2013
5
Multiple in silico tools predict phenotypic manifestations in congenital thrombotic thrombocytopenic purpura. (23346910)
2013
6
Treatment of refractory thrombotic thrombocytopenic purpura with N-acetylcysteine: a case report. (24117464)
2013
7
Thrombotic thrombocytopenic purpura (TTP)-like illness associated with intravenous Opana ER abuse--Tennessee, 2012. (23302815)
2013
8
Inherited thrombotic thrombocytopenic purpura in pregnancy. (24378452)
2013
9
Long term outcome and sequelae in patients after acute thrombotic thrombocytopenic purpura episodes. (23599034)
2013
10
Drop of residual plasmatic activity of ADAMTS13 to undetectable levels during acute disease in a patient with adult-onset congenital thrombotic thrombocytopenic purpura. (22981442)
2013
11
Inverse correlation of free and immune complex-sequestered anti-ADAMTS13 antibodies in a patient with acquired thrombotic thrombocytopenic purpura. (22044668)
2012
12
Management of Thrombotic Thrombocytopenic Purpura with Autoantibodies to ADAMTS-13 and Concurrent Preeclampsia in Pregnancy: Multidisciplinary Team Approach. (23946903)
2012
13
Thrombotic thrombocytopenic purpura triggered by influenza A virus subtype H1N1 infection. (22094185)
2012
14
Coexistence of systemic lupus erythematosus and thrombotic thrombocytopenic purpura: a case report. (22611780)
2012
15
Transfusion medicine illustrated. Massive hemolysis due to thrombotic thrombocytopenic purpura in a patient with AIDS. (22780894)
2012
16
Advantages and limits of ADAMTS13 testing in the prognostic assessment of thrombotic thrombocytopenic purpura. (22244722)
2012
17
Role of ADAMTS13 in the pathogenesis, diagnosis, and treatment of thrombotic thrombocytopenic purpura. (23233642)
2012
18
Current management and therapeutical perspectives in thrombotic thrombocytopenic purpura. (22265954)
2012
19
Rituximab in a child with autoimmune thrombotic thrombocytopenic purpura refractory to plasma exchange. (22552883)
2012
20
Humoral immune response to ADAMTS13 in acquired thrombotic thrombocytopenic purpura. (21535387)
2011
21
Complete recovery of ischemic cardiomyopathy from thrombotic thrombocytopenic purpura. (21487455)
2011
22
Is there a link between thrombotic thrombocytopenic purpura and anti-glomerular basement membrane disease? (21430178)
2011
23
Thrombotic thrombocytopenic purpura associated with disseminated varicella zoster in a multiple myeloma patient. (21260958)
2010
24
Postoperative thrombotic thrombocytopenic purpura after open heart operations. (20103218)
2010
25
Case series: splenectomy: does it still play a role in the management of thrombotic thrombocytopenic purpura? (20858382)
2010
26
High versus standard dose methylprednisolone in the acute phase of idiopathic thrombotic thrombocytopenic purpura: a randomized study. (20033409)
2010
27
Thrombotic thrombocytopenic purpura--the role of ADAMTS13 assay in clinical practice. (20977108)
2010
28
Rituximab as pre-emptive treatment in patients with thrombotic thrombocytopenic purpura and evidence of anti-ADAMTS13 autoantibodies. (19190804)
2009
29
Minor stroke as singular manifestation of hereditary thrombotic thrombocytopenic purpura in a young man. (19648879)
2009
30
Cognitive deficits after recovery from thrombotic thrombocytopenic purpura. (19222817)
2009
31
Brain lesions are most often reversible in acute thrombotic thrombocytopenic purpura. (19564586)
2009
32
Hypersensitivity to plasma exchange in a patient with thrombotic thrombocytopenic purpura. (19073012)
2009
33
Plasma exchange therapy for thrombotic thrombocytopenic purpura in pediatric patients with liver transplantation. (18929799)
2008
34
Synergistic interactions between interferon-gamma and TRAIL modulate c-FLIP in endothelial cells, mediating their lineage-specific sensitivity to thrombotic thrombocytopenic purpura plasma-associated apoptosis. (18339897)
2008
35
Treatment of acquired thrombotic thrombocytopenic purpura (TTP) with plasma infusion plus rituximab. (18612551)
2008
36
Thrombotic thrombocytopenic purpura associated with severe acute pancreatitis in a context of decreased ADAMTS13 activity: a case report. (18989146)
2008
37
Congenital thrombotic thrombocytopenic purpura: Lessons for recognition and management of rare syndromes. (18360884)
2008
38
Cyclosporin and plasma exchange in thrombotic thrombocytopenic purpura: long-term follow-up with serial analysis of ADAMTS13 activity. (17910638)
2007
39
Pancreatitis preceding acute episodes of thrombotic thrombocytopenic purpura-hemolytic uremic syndrome: report of five patients with a systematic review of published reports. (17606444)
2007
40
ADAMTS13-binding IgG are present in patients with thrombotic thrombocytopenic purpura. (16676082)
2006
41
Thrombotic thrombocytopenic purpura. (16102032)
2005
42
Clinical features and laboratory findings of thrombotic thrombocytopenic purpura associated with ticlopidine]. (16104532)
2005
43
ADAMTS13, thrombotic thrombocytopenic purpura, and hemolytic uremic syndrome. (15865866)
2005
44
Thrombotic thrombocytopenic purpura complicating SjAPgren's syndrome with crescentic glomerulonephritis and membranous nephritis. (17143670)
2004
45
ADAMTS13 activity in thrombotic thrombocytopenic purpura-hemolytic uremic syndrome: relation to presenting features and clinical outcomes in a prospective cohort of 142 patients. (12637323)
2003
46
Acquired protein S deficiency in thrombotic thrombocytopenic purpura patients receiving solvent/detergent plasma exchange. (12877681)
2003
47
An update on the pathogenesis and management of acquired thrombotic thrombocytopenic purpura. (12858075)
2003
48
Schizocytosis in pernicious anemia mimicking thrombotic thrombocytopenic purpura. (12714141)
2003
49
Remission of thrombotic thrombocytopenic purpura in a patient with compound heterozygous deficiency of von Willebrand factor-cleaving protease by infusion of solvent/detergent plasma. (12434890)
2002
50
Thrombotic-thrombocytopenic purpura (Moschcowitz syndrome)]. (11450586)
2001

Variations for Thrombotic Thrombocytopenic Purpura

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Expression for genes affiliated with Thrombotic Thrombocytopenic Purpura

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2BioGPS, 15Gene Expression Omnibus DataSets
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Expression patterns in normal tissues for genes affiliated with Thrombotic Thrombocytopenic Purpura

Search GEO for disease gene expression data for Thrombotic Thrombocytopenic Purpura.

Pathways for genes affiliated with Thrombotic Thrombocytopenic Purpura

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50PathCards, 55Reactome, 30KEGG, 38NCBI BioSystems Database, 53QIAGEN, 54R&D Systems
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Pathways related to Thrombotic Thrombocytopenic Purpura according to GeneCards/GeneDecks:

idSuper pathways (with members indented)ScoreTop Affiliating Genes
1
Show member pathways
9.5WAS, HLA-DRB4
2
Show member pathways
9.5HLA-DRB4, WAS
3
Show member pathways
Complement Activation, Classical Pathway38
Complement and Coagulation Cascades38
8.7VWF, THBD, SERPINF2
4
Show member pathways
8.7SERPINF2, THBD, VWF
5
Show member pathways
8.6VWF, THBS1, SERPINF2
6
Show member pathways
8.1VWF, THBD, THBS1, SERPINF2

Compounds for genes affiliated with Thrombotic Thrombocytopenic Purpura

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45Novoseek, 11DrugBank, 51PharmGKB, 29IUPHAR, 24HMDB, 61Tocris Bioscience
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Compounds related to Thrombotic Thrombocytopenic Purpura according to GeneCards/GeneDecks:

(show top 50)    (show all 64)
idCompoundScoreTop Affiliating Genes
1ticlopidine45 1111.0ADAMTS13, VWF
2beraprost sodium459.9VWF, THBD
311-dehydrothromboxane b2459.9THBD, VWF
4lm 609459.9VWF, THBS1
5perindopril45 51 1111.8THBD, VWF
6paricalcitol45 1110.7THBS1, THBD
7fitc459.7THBD, VWF
8cacl2459.6THBD, THBS1
9dermatan sulfate459.6THBD, VWF
10fluvastatin45 51 29 1112.5THBD, VWF
11fibrinopeptide a459.5SERPINF2, THBD
12ptca459.4THBD, SERPINF2
13raloxifene45 51 29 1112.3THBD, VWF, THBS1
14latex459.3VWF, THBS1, WAS
15n-formylmethionyl-leucylphenylalanine459.3THBD, THBS1
16atorvastatin45 51 29 24 1113.3THBD, THBS1, VWF
17epsilon aminocaproic acid459.3SERPINF2, THBS1
18cytochalasin d45 6110.3WAS, VWF, THBS1
19agarose459.3VWF, THBS1, THBD
20ristocetin459.3SERPINF2, ADAMTS13, VWF
21hyaluronic acid45 2410.3VWF, THBD, THBS1
22prostacyclin459.3VWF, THBD, THBS1
23phosphatidylserine45 29 1111.3THBS1, THBD, VWF
24warfarin45 51 24 1112.2THBD, VWF
25matrigel459.2VWF, THBD, THBS1
266-ketoprostaglandin f1alpha459.2SERPINF2, THBD, VWF
27methionine459.2THBS1, THBD, VWF
28thromboxane45 2410.1THBD, SERPINF2, VWF
29aprotinin45 1110.1SERPINF2, THBD, VWF
30endotoxin459.1ADAMTS13, SERPINF2, THBD
31thyroxine45 2410.1VWF, THBD, SERPINF2
32creatinine459.1VWF, ADAMTS13, THBD
33paraffin459.1VWF, THBS1, THBD
34cyclophosphamide45 51 1111.0ADAMTS13, VWF, THBS1, THBD
35cyclosporin a45 29 6111.0ADAMTS13, VWF, THBS1, THBD
36heparan sulfate45 249.9THBD, THBS1, SERPINF2
37desmopressin45 61 29 1111.8THBD, ADAMTS13, SERPINF2, VWF
38polysaccharide458.7THBD, THBS1, SERPINF2
39hirudin458.6THBS1, VWF, THBD, SERPINF2
40txb2458.5SERPINF2, VWF, THBD, THBS1
41kininogen458.5THBD, SERPINF2, THBS1, VWF
42adp45 29 2410.5SERPINF2, WAS, THBD, VWF
43cholesterol45 29 24 1111.5SERPINF2, VWF, THBD, THBS1
44vegf458.4VWF, THBD, THBS1, SERPINF2
45alanine458.4VWF, SERPINF2, THBS1, THBD
46fibrinogen458.2THBS1, VWF, SERPINF2, THBD, ADAMTS13
47heparin45 29 24 1111.2THBD, ADAMTS13, SERPINF2, THBS1, VWF
48lipid458.1THBD, THBS1, SERPINF2, WAS
49calcium45 51 24 1110.9THBS1, SERPINF2, VWF, WAS, ADAMTS13
50serine457.6THBS1, VWF, THBD, SERPINF2, WAS, ADAMTS13

GO Terms for genes affiliated with Thrombotic Thrombocytopenic Purpura

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16Gene Ontology
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Cellular components related to Thrombotic Thrombocytopenic Purpura according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1platelet alpha granuleGO:0310919.3VWF, THBS1
2fibrinogen complexGO:0055779.0SERPINF2, THBS1
3platelet alpha granule lumenGO:0310938.7VWF, THBS1, SERPINF2
4external side of plasma membraneGO:0098978.7THBS1, HLA-DRB4, VWF
5cell surfaceGO:0099868.2THBD, THBS1, SERPINF2, ADAMTS13
6extracellular spaceGO:0056158.1THBS1, THBD, ADAMTS13, SERPINF2

Biological processes related to Thrombotic Thrombocytopenic Purpura according to GeneCards/GeneDecks:

(show all 7)
idNameGO IDScoreTop Affiliating Genes
1T cell receptor signaling pathwayGO:0508529.5HLA-DRB4, WAS
2negative regulation of plasminogen activationGO:0107578.9THBS1, SERPINF2
3negative regulation of fibrinolysisGO:0519188.7THBD, THBS1, SERPINF2
4platelet degranulationGO:0025768.6SERPINF2, THBS1, VWF
5immune responseGO:0069558.6WAS, HLA-DRB4, THBS1
6platelet activationGO:0301688.3SERPINF2, THBS1, VWF, ADAMTS13
7blood coagulationGO:0075967.7VWF, THBD, THBS1, SERPINF2, WAS

Molecular functions related to Thrombotic Thrombocytopenic Purpura according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1glycoprotein bindingGO:0019489.4THBS1, VWF
2integrin bindingGO:0051789.2THBS1, VWF, ADAMTS13
3calcium ion bindingGO:0055099.0THBD, THBS1, ADAMTS13
4protease bindingGO:0020208.9VWF, SERPINF2
5identical protein bindingGO:0428028.8WAS, THBS1, VWF
6protein bindingGO:0055156.8SERPINF2, HLA-DRB4, ADAMTS13, THBS1, THBD, VWF

Products for genes affiliated with Thrombotic Thrombocytopenic Purpura

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Sources for Thrombotic Thrombocytopenic Purpura

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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