MCID: THR005
MIFTS: 57

Thrombotic Thrombocytopenic Purpura malady

Blood diseases category

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 Formation of Fibrin Clot (Clotting Cascade) and Immune response Lectin induced complement pathway. The compounds dexamethasone and endotoxin have been mentioned in the context of this disorder. Affiliated tissues include endothelial, bone marrow and bone, and related mouse phenotypes are renal/urinary system and hematopoietic system.

Aliases & Classifications for Thrombotic Thrombocytopenic Purpura

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8Disease Ontology, 60UMLS, 56SNOMED-CT, 34MeSH, 39NCIt, 25ICD10
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Classifications:

Malacards categories (disease lists): (See all malacards categories)
Anatomical: Blood diseases


Aliases & Descriptions:

thrombotic thrombocytopenic purpura 8
ttp - thrombotic thrombocytopenic purpura 8
purpura, thrombotic thrombocytopenic 60
thrombotic microangiopathy nos 8
thrombotic microangiopathies 60
thrombotic microangiopathy 8
moschcowitz's syndrome 8


External Ids:

Disease Ontology8 DOID:10772
MeSH34 D011697
NCIt39 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 228)
idRelated DiseaseScoreTop Affiliating Genes
1purpura32.4THPO, ADAMTS13
2hemolytic-uremic syndrome31.6CFH, CD46, THBD, VWF, ADAMTS13, CFI
3thrombotic thrombocytopenic purpura, acquired31.3CFH, VWF, HP, CD46, THBS1, ADAMTS13
4lupus erythematosus31.3THBD
5systemic lupus erythematosus31.3ADAMTS13, CD46, PTPN22, THBD, CFH, CFI
6thrombocytopenia31.0HP, THPO, CFH, VWF, THBD, ADAMTS13
7connective tissue disease31.0PTPN22, THBD, VWF
8thrombotic thrombocytopenic purpura, congenital31.0HP, ADAMTS13, CFH
9hepatitis30.9IL2RB, HP
10hepatitis c30.9CD9, THPO, HP
11hemolytic anemia30.9HP, VWF, CFH, ADAMTS13
12disseminated intravascular coagulation30.9SERPINF2, ADAMTS13, THBD, THPO, PPIC
13myeloma30.8CD9, CFLAR
14myocardial infarction30.8CFH, SERPINF2, VWF, ADAMTS13
15hellp syndrome30.8HP, VWF, ADAMTS13
16antiphospholipid syndrome30.8ADAMTS13, CFH, THBD, VWF
17glomerulonephritis30.7CD46, CFH, CFI
18atypical hemolytic-uremic syndrome30.7CFH, CD46, THBD, CFI
19sickle cell disease30.7VWF, HP, CFI, THBS1
20adenocarcinoma30.7CD46, THBS1, CFLAR, THBD, KRT7, CD9
21acute myocardial infarction30.5ADAMTS13, VWF, THBD, SERPINF2
22vasculitis30.5VWF, CD46, THBD, CFI
23ischemia30.5CFLAR, ADAMTS13, HP, IL2RB
24prostate cancer30.5STX1A, CD9, CFLAR, THBS1, CD46
25leukemia30.5IL2RB, CD9, CFLAR, CD46, THPO, SERPINF2
26nephrotic syndrome30.3CFI, SERPINF2, HP
27ovarian cancer30.3THBS1, KRT7, CFI, HP, CD46, CD9
28peritonitis30.3CFI
29lung cancer30.3KRT7
30uremia30.3VWF
31thymoma30.3TSR1
32pneumonia30.3IL2RB, CD9
33thrombophilia30.3THBD, SERPINF2, VWF
34collagen disease30.3VWF, THBD
35membranoproliferative glomerulonephritis30.3CD46, CFH
36liver cirrhosis30.3THPO, SERPINF2, ADAMTS13, HP
37pre-eclampsia30.3SERPINF2, HP, VWF, THBD, ADAMTS13
38graves' disease30.3PTPN22
39colon cancer30.3CFI, CD9, THBS1, CFLAR
40systemic scleroderma30.3PTPN22, THBD, VWF, IL2RB, CD46
41vascular disease30.3THBD, HP, CD46, VWF
42melanoma30.3CFI, CD46, IL2RB, CFLAR, CD9, THBS1
43malaria30.1ADAMTS13, HP, IL2RB
44meningitis30.1CFI, CD46
45squamous cell carcinoma30.1CD9, KRT7, THBS1, THBD, CFLAR, CD46
46autoimmune thrombocytopenic purpura10.8
47pancreatitis10.8
48retinitis10.8
49acute pancreatitis10.7
50mixed connective tissue disease10.6

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



Diseases related to thrombotic thrombocytopenic purpura

Clinical Features for Thrombotic Thrombocytopenic Purpura

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Drugs & Therapeutics for Thrombotic Thrombocytopenic Purpura

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5CenterWatch, 41NIH Clinical Center, 6ClinicalTrials
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Approved drugs:

Search CenterWatch for Thrombotic Thrombocytopenic Purpura

Drug clinical trials:

Search ClinicalTrials for Thrombotic Thrombocytopenic Purpura

Search NIH Clinical Center for Thrombotic Thrombocytopenic Purpura

Search CenterWatch for Thrombotic Thrombocytopenic Purpura

Genetic Tests for Thrombotic Thrombocytopenic Purpura

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Anatomical Context for Thrombotic Thrombocytopenic Purpura

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32MalaCards
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MalaCards organs/tissues related to Thrombotic Thrombocytopenic Purpura:

32
Endothelial, Bone marrow, Bone, Testes, Liver, Kidney, B cells, Lung, Heart, Prostate, Retina, Breast, Pancreatic islet, Brain, Cortex, Colon, Pancreas, Pituitary

Animal Models for Thrombotic Thrombocytopenic Purpura or affiliated genes

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36MGI
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MGI Mouse Phenotypes related to Thrombotic Thrombocytopenic Purpura:

36
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00053679.9CFI, CFH, THBS1, KRT7, PTPN22, HP
2MP:00053979.5ADAMTS13, CD9, HP, IL2RB, VWF, CFLAR
3MP:00053879.4VWF, IL2RB, HP, CD9, CFLAR, CFH
4MP:00053769.3CFLAR, VWF, HP, STX1A, CFI, CFH

Publications for Thrombotic Thrombocytopenic Purpura

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50PubMed
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Articles related to Thrombotic Thrombocytopenic Purpura:

(show top 50)    (show all 718)
idTitleAuthorsYear
1
Treatment of refractory thrombotic thrombocytopenic purpura with N-acetylcysteine: a case report. (24117464)
2013
2
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
3
ADAMTS13 unbound to larger von Willebrand factor multimers in cryosupernatant: implications for selection of plasma preparations for thrombotic thrombocytopenic purpura treatment. (23560518)
2013
4
The emerging concept of residual ADAMTS13 activity in ADAMTS13-deficient thrombotic thrombocytopenic purpura. (23415418)
2013
5
ADAMTS13 deficiency and thrombotic thrombocytopenic purpura associated with trimethoprim-sulfamethoxazole. (23262188)
2013
6
The use of 50% albumin/plasma replacement fluid in therapeutic plasma exchange for thrombotic thrombocytopenic purpura. (23857424)
2013
7
A case of adult-onset Still's disease complicated by thrombotic thrombocytopenic purpura with retinal microangiopathy and rapidly fatal cerebral edema. (22623015)
2013
8
Platelet transfusions and bleeding complications associated with plasma exchange catheter placement in patients with presumed thrombotic thrombocytopenic purpura. (23720092)
2013
9
Two cases of mixed connective tissue disease complicated with thrombotic thrombocytopenic purpura. (23842515)
2013
10
Development and validation of a predictive model for death in acquired severe ADAMTS13 deficiency-associated idiopathic thrombotic thrombocytopenic purpura: the French TMA Reference Center experience. (22580997)
2012
11
Is platelet transfusion necessary prior to a central venous catheter placement in thrombotic thrombocytopenic purpura patients? (23362594)
2012
12
Decreasing frequency of plasma exchange complications in patients treated for thrombotic thrombocytopenic purpura-hemolytic uremic syndrome, 1996 to 2011. (22501034)
2012
13
Efficacy of rituximab in acute refractory or chronic relapsing non-familial idiopathic thrombotic thrombocytopenic purpura: a systematic review with pooled data analysis. (22547089)
2012
14
Congenital thrombotic thrombocytopenic purpura associated with moyamoya syndrome in a 3-year-old girl: a case report. (22378676)
2012
15
Acute pancreatitis-induced thrombotic thrombocytopenic purpura. (22233953)
2012
16
Cyclophosphamide-rescued plasmapheresis-unresponsive secondary thrombotic thrombocytopenic purpura caused by SjAPgren's syndrome. (23185207)
2012
17
ADAMTS13 activity and the risk of thrombotic thrombocytopenic purpura relapse in pregnancy. (21275971)
2011
18
Update on thrombotic thrombocytopenic purpura. (22402460)
2011
19
Thrombotic thrombocytopenic purpura associated with disseminated varicella zoster in a multiple myeloma patient. (21260958)
2010
20
High versus standard dose methylprednisolone in the acute phase of idiopathic thrombotic thrombocytopenic purpura: a randomized study. (20033409)
2010
21
Thrombotic thrombocytopenic purpura at the Johns Hopkins Hospital from 1992 to 2008: clinical outcomes and risk factors for relapse. (20003052)
2010
22
Acute cardiomyopathy in a patient with thrombotic thrombocytopenic purpura. (20103008)
2010
23
Unusual clinical findings in thrombotic thrombocytopenic purpura. (19466967)
2010
24
Rituximab pharmacokinetics during the management of acute idiopathic thrombotic thrombocytopenic purpura. (20175870)
2010
25
Human leukocyte antigen association in idiopathic thrombotic thrombocytopenic purpura: evidence for an immunogenetic link. (19922436)
2010
26
Influenza A infection triggers thrombotic thrombocytopenic purpura by producing the anti-ADAMTS13 IgG inhibitor. (20371960)
2010
27
IgG subclass distribution of anti-ADAMTS13 antibodies in patients with acquired thrombotic thrombocytopenic purpura. (19682238)
2009
28
Clinical-pathological features and prognosis of thrombotic thrombocytopenic purpura in patients with lupus nephritis. (19745703)
2009
29
Newly diagnosed versus relapsed idiopathic thrombotic thrombocytopenic purpura: a comparison of presenting clinical characteristics and response to treatment. (19205654)
2009
30
The thrombotic thrombocytopenic purpura registry: a new national resource to inform patient care and medical research. (19290992)
2009
31
HIV associated thrombocytopenia, misdiagnosed as thrombotic thrombocytopenic purpura: a case report. (19946486)
2009
32
Ticlopidine- and clopidogrel-associated thrombotic thrombocytopenic purpura (TTP): review of clinical, laboratory, epidemiological, and pharmacovigilance findings (1989-2008). (19180126)
2009
33
Value of ADAMTS13 activity and inhibitor in the postmortem diagnosis of thrombotic thrombocytopenic purpura. (19253359)
2009
34
Thrombotic thrombocytopenic purpura in a patient with HIV from Zimbabwe. (21822450)
2009
35
Thrombotic thrombocytopenic purpura presenting with reversible posterior leukoencephalopathy syndrome. (20009443)
2009
36
Thrombotic thrombocytopenic purpura: 2008 update. (18556880)
2008
37
Purpura in a patient with disseminated breast cancer: a rapidly progressive cancer-related thrombotic thrombocytopenic purpura. (18407952)
2008
38
Cyclosporin and plasma exchange in thrombotic thrombocytopenic purpura: long-term follow-up with serial analysis of ADAMTS13 activity. (17910638)
2007
39
Post-appendectomy thrombotic thrombocytopenic purpura: a case report and review of the literature. (17034025)
2007
40
Extensive coronary thrombosis in thrombotic-thrombocytopenic purpura. (16337055)
2006
41
Novel ADAMTS-13 mutations in an adult with delayed onset thrombotic thrombocytopenic purpura. (16796708)
2006
42
ADAMTS13, thrombotic thrombocytopenic purpura, and hemolytic uremic syndrome. (15865866)
2005
43
Identification of two novel mutations in ADAMTS13 gene in a patient with hereditary thrombotic thrombocytopenic purpura. (16468327)
2005
44
Pathogenesis of thrombotic thrombocytopenic purpura: ADAMTS13 deficiency and beyond. (16388413)
2005
45
Rituximab for chronic recurring thrombotic thrombocytopenic purpura: a case report and review of the literature. (15009067)
2004
46
Thrombotic thrombocytopenic purpura in a newborn. (12556937)
2003
47
von Willebrand factor and thrombotic thrombocytopenic purpura. (10961577)
2000
48
Increased fragmentation of von Willebrand factor, due to abnormal cleavage of the subunit, parallels disease activity in recurrent hemolytic uremic syndrome and thrombotic thrombocytopenic purpura and discloses predisposition in families. The Italian Registry of Familial and Recurrent HUS/TTP. (10397728)
1999
49
Erythropoietin in thrombotic thrombocytopenic purpura and acute renal failure. (1600339)
1992
50
Thrombin generation and fibrinolysis in the thrombotic thrombocytopenic purpura and the hemolytic-uremic syndrome. (1803613)
1991

Genetic Variations for Thrombotic Thrombocytopenic Purpura

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Expression for genes affiliated with Thrombotic Thrombocytopenic Purpura

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1BioGPS, 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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53Reactome, 52R&D Systems, 37NCBI BioSystems Database, 12EMD Millipore, 29KEGG
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Compounds for genes affiliated with Thrombotic Thrombocytopenic Purpura

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44Novoseek, 49PharmGKB, 28IUPHAR, 11DrugBank, 24HMDB, 59Tocris Bioscience
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Compounds related to Thrombotic Thrombocytopenic Purpura according to GeneCards/GeneDecks:

(show top 50)    (show all 82)
idCompoundScoreTop Affiliating Genes
1dexamethasone44 49 28 1113.7CD46, CFH
2endotoxin4410.7ADAMTS13, THBD
3heparin44 28 11 2413.6ADAMTS13, CFI, HP, THPO
4desmopressin44 59 28 1113.6ADAMTS13, VWF, THBD, SERPINF2
5txb24410.5VWF, THBS1, SERPINF2, THBD
6hirudin4410.5VWF, THBS1, THBD, THPO, SERPINF2
7carbohydrates4410.5THBD, CFI, HP, VWF
86-ketoprostaglandin f1alpha4410.5VWF, THBD, SERPINF2
9matrigel4410.5THBS1, KRT7
10thromboxane44 2411.5THPO, THBD, VWF, SERPINF2, CD9
11kininogen4410.5HP, VWF, CFI, THBS1, THBD, SERPINF2
12ristocetin4410.4VWF, ADAMTS13, SERPINF2
13rituximab44 49 1112.4ADAMTS13, CFLAR, VWF, HP
14agarose4410.4HP, THPO, VWF, THBS1, THBD
15cyclophosphamide44 49 1112.4THBD, CD46, IL2RB, VWF, THBS1, ADAMTS13
16pentoxifylline44 1111.4IL2RB, CD9, HP, THBD
17hyaluronic acid44 2411.4THBS1, THPO, THBD, CFH, HP, VWF
18thyroxine44 2411.4CFI, VWF, IL2RB, THBD, SERPINF2, HP
19neopterin4410.4HP, IL2RB, CD9, THBD
20serine4410.4CD46, HP, IL2RB, CFLAR, THBS1, ADAMTS13
21methionine4410.4CFI, THBD, THBS1, HP, IL2RB, VWF
22nacl4410.3THBD, CFH, CFI, HP, ADAMTS13
23sialic acid4410.3HP, CD46, THPO, KRT7, SERPINF2, CFH
24aspirin44 49 28 2413.3IL2RB, CD9, THPO, THBD, VWF, HP
25ptca4410.3THBD, PPIC, SERPINF2
26polyethylene glycol4410.3CD9, HP, THBD, THPO
27glucose4410.3THBS1, STX1A, CFI, KRT7, THBD, CFH
28creatinine4410.3HP, THPO, CFI, VWF, IL2RB, THBD
29latex4410.3THBS1, CD9, THPO, VWF
30sucrose44 11 2412.3THPO, HP, CD9, THBD, CFI
31paraffin4410.2CD9, CD46, VWF, THBS1, THBD, KRT7
32h2o24410.2CFLAR, THPO, CD9, HP, VWF, THBD
33testosterone44 59 11 2413.2CFLAR, SERPINF2, KRT7, VWF, HP, THBS1
34p0024410.2CD9, HP, THPO, CFLAR
35vegf4410.1THBD, CFLAR, THBS1, IL2RB, HP, THPO
36gold4410.1THPO, THBS1, IL2RB, CD9
37estrogen4410.1THPO
38cysteine4410.1CFH, STX1A, THBS1, HP, THBD, CFLAR
39cyclosporin a44 28 5912.1THBS1, ADAMTS13, IL2RB, PPIC, CD46, HP
40cholesterol44 28 11 2413.1HP, THBD, VWF, STX1A, CD9, IL2RB
41alanine4410.1SERPINF2, THBD, HP, CFH, THBS1, CD46
42nitric oxide44 11 2412.0CD9, STX1A, THPO, CD46, HP, VWF
43retinoic acid44 2411.0THBS1, CFLAR, CFI, CD9, IL2RB, VWF
44polysaccharide4410.0THPO, THBD, THBS1, CFH, IL2RB, HP
45fibrinogen4410.0VWF, ADAMTS13, SERPINF2, THPO, THBD, THBS1
46lipid4410.0SERPINF2, THBD, STX1A, CD9, HP, IL2RB
47rapamycin4410.0CFLAR, CD46, THBS1, IL2RB, THPO
48progesterone44 59 28 11 2413.9THBS1, CD46, HP, KRT7, THBD, IL2RB
49calcium44 49 11 2412.9VWF, STX1A, ADAMTS13, SERPINF2, THPO, THBS1
50tyrosine449.7CFLAR, PTPN22, THBS1, THBD, GPR182, VWF

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 granule lumenGO:03109310.3VWF, SERPINF2, THBS1
2fibrinogen complexGO:00557710.2THBS1, SERPINF2
3cell surfaceGO:00998610.2SERPINF2, THBD, THBS1, CD46, ADAMTS13
4external side of plasma membraneGO:00989710.1THBS1, VWF, IL2RB, CD9
5extracellular spaceGO:00561510.1HP, CFH, THBS1, THPO, SERPINF2, ADAMTS13
6platelet alpha granuleGO:03109110.0VWF, THBS1
7extracellular regionGO:0055769.9THBS1, CFH, CFI, VWF, HP, STX1A

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

idNameGO IDScoreTop Affiliating Genes
1negative regulation of plasminogen activationGO:01075710.3THBS1, SERPINF2
2negative regulation of fibrinolysisGO:05191810.3THBD, SERPINF2, THBS1
3platelet degranulationGO:00257610.1SERPINF2, THBS1, VWF, CD9
4platelet activationGO:03016810.1ADAMTS13, CD9, VWF, THBS1, THPO, SERPINF2
5regulation of complement activationGO:0304499.9CFH, CFI, CD46
6blood coagulationGO:0075969.9SERPINF2, THPO, THBD, THBS1, VWF, CD9

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

idNameGO IDScoreTop Affiliating Genes
1protease bindingGO:00202010.2VWF, CFLAR, SERPINF2
2integrin bindingGO:00517810.2THBS1, CD9, VWF, ADAMTS13
3glycoprotein bindingGO:00194810.2THBS1, STX1A, VWF
4protein bindingGO:0055159.0VWF, HP, CD9, CFLAR, CFH, THBS1

Products for genes affiliated with Thrombotic Thrombocytopenic Purpura

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  • Antibodies
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Sources for Thrombotic Thrombocytopenic Purpura

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3CDC
13ExPASy
14FMA
22GTR
23HGMD
24HMDB
25ICD10
26ICD10 via Orphanet
27ICD9CM
28IUPHAR
29KEGG
34MeSH
35MESH via Orphanet
36MGI
39NCIt
40NDF-RT
43NINDS
44Novoseek
46OMIM
47OMIM via Orphanet
50PubMed
51QIAGEN
57SNOMED-CT via Orphanet
60UMLS
61UMLS via Orphanet