MCID: ACQ017
MIFTS: 47

Acquired Von Willebrand Syndrome malady

Rare diseases, Blood diseases categories

Summaries for Acquired Von Willebrand Syndrome

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NIH Rare Diseases:43 Acquired von willebrand syndrome (avws) is a bleeding disorder that can occur due to a variety of conditions, but is not caused by a vwf gene mutation. it is most often seen in persons over age 40 years with no prior bleeding history. this condition may result from lymphoproliferative disorders, autoimmune disorders (including systemic lupus erythrematosus, scleroderma, and antiphospholipid antibody syndrome), heart conditions such as aortic valvular stenosis, increased blood platelet count such as essential thrombocythemia, and certain drugs. about 300 cases of this condition have been reported in the medical literature thus far. treatment depends on the underlying cause of the condition. last updated: 9/15/2011

MalaCards based summary: Acquired Von Willebrand Syndrome, also known as acquired von willebrand disease, is related to hypothyroidism and congenital heart disease. An important gene associated with Acquired Von Willebrand Syndrome is VWF (von Willebrand factor), and among its related pathways are Response to elevated platelet cytosolic Ca2+ and Platelet Aggregation Inhibitor Pathway, Pharmacodynamics. The compounds recombinate and kogenate have been mentioned in the context of this disorder. Affiliated tissues include heart, thyroid and skin, and related mouse phenotypes are nervous system and cardiovascular system.

Aliases & Classifications for Acquired Von Willebrand Syndrome

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Sources:
43NIH Rare Diseases, 49Orphanet, 28ICD10 via Orphanet, 63UMLS via Orphanet
See all sources

Acquired Von Willebrand Syndrome, Aliases & Descriptions:

Name: Acquired Von Willebrand Syndrome 43 49
Acquired Von Willebrand Disease 43 49
 
Willebrand Disease, Acquired 43


Classifications:

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


Characteristics (Orphanet epidemiological data):

49
acquired von willebrand syndrome:
Inheritance: Not applicable; Age of onset: Adult


External Ids:

Orphanet49 99147
ICD10 via Orphanet28 D68.4
UMLS via Orphanet63 C0272362

Related Diseases for Acquired Von Willebrand Syndrome

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Diseases related to Acquired Von Willebrand Syndrome via text searches within MalaCards or GeneCards/GeneDecks gene sharing:

(show top 50)    (show all 96)
idRelated DiseaseScoreTop Affiliating Genes
1hypothyroidism30.9VWF, F8
2congenital heart disease30.8F2, VWF, F3, F8, CD40LG
3lupus erythematosus30.8CD40LG, F3, F2
4systemic lupus erythematosus30.7F2, VWF, F3, F8, CD40LG
5hemolytic anemia30.7F3, VWF
6angiodysplasia30.5VWF
7thrombocytosis30.4F2, VWF, F3
8hemophilia a30.3F2, VWF, F3, F8
9polycythemia vera10.4
10wilms tumor10.4
11multiple myeloma10.4
12autoimmune hemolytic anemia10.4
13myeloma10.4
14polycythemia10.4
15ewing sarcoma10.3
16leukemia10.3
17malt lymphoma10.3
18monoclonal gammopathy of uncertain significance10.3
19patent ductus arteriosus10.3
20lymphoplasmacytic lymphoma10.3
21amyloidosis10.3
22melanoma10.3
23sarcoma10.3
24thyroiditis10.3
25al amyloidosis10.3
26essential thrombocythemia10.3
27hepatocellular carcinoma10.3
28cryoglobulinemia10.3
29erythromelalgia10.3
30congenital mesoblastic nephroma10.3
31pericardial effusion10.3
32mesoblastic nephroma10.3
33hemophilia10.1
34thrombophlebitis10.1F8
35glanzmann thrombasthenia10.0VWF
36cardiac tamponade10.0F8
37hepatitis e10.0CD40LG
38hemorrhagic fever10.0CD40LG
39scrub typhus10.0CD40LG
40factor xiii deficiency10.0F3
41hepatitis a10.0F8, F2
42viral hepatitis10.0F2, F8
43blood platelet disease10.0VWF, F8
44portal vein thrombosis10.0F8, F2
45atrial fibrillation10.0VWF, F2
46blood protein disease10.0F2, CD40LG
47factor xii deficiency10.0F3, VWF
48factor viii deficiency10.0F8, F3
49hypersensitivity reaction type ii disease10.0F8, VWF
50autoimmune hepatitis10.0F2, CD40LG

Graphical network of the top 20 diseases related to Acquired Von Willebrand Syndrome:



Diseases related to acquired von willebrand syndrome

Symptoms for Acquired Von Willebrand Syndrome

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Drugs & Therapeutics for Acquired Von Willebrand Syndrome

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Drug clinical trials:

Search ClinicalTrials for Acquired Von Willebrand Syndrome

Search NIH Clinical Center for Acquired Von Willebrand Syndrome

Genetic Tests for Acquired Von Willebrand Syndrome

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Anatomical Context for Acquired Von Willebrand Syndrome

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MalaCards organs/tissues related to Acquired Von Willebrand Syndrome:

33
Heart, Thyroid, Skin

Animal Models for Acquired Von Willebrand Syndrome or affiliated genes

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MGI Mouse Phenotypes related to Acquired Von Willebrand Syndrome:

37
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00036318.1CD40LG, F3, VWF, F2
2MP:00053858.0CD40LG, F3, VWF, F2
3MP:00053847.8F2, VWF, F3, CD40LG
4MP:00053977.7CD40LG, F8, F3, VWF, F2
5MP:00053877.6F2, VWF, F3, F8, CD40LG
6MP:00053767.6CD40LG, F8, F3, VWF, F2
7MP:00107687.5F2, VWF, F3, F8, CD40LG

Publications for Acquired Von Willebrand Syndrome

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Articles related to Acquired Von Willebrand Syndrome:

(show top 50)    (show all 127)
idTitleAuthorsYear
1
Acquired von Willebrand Syndrome (AVWS) as an Important Diagnostic Category of Disease. (25740338)
2015
2
Autophagy: A potential link between Acquired von Willebrand syndrome and congenital heart disease. (25804464)
2015
3
Acquired von Willebrand syndrome in a case of polycythemia vera resulting in recurrent and massive bleeding events in the pleural and abdominal cavity. (25215616)
2014
4
Acquired von Willebrand syndrome in patients with a centrifugal or axial continuous flow left ventricular assist device. (24720921)
2014
5
Correction of acquired von Willebrand syndrome by transcatheter aortic valve implantation. (25480995)
2014
6
An atypical manifestation of acquired von Willebrand syndrome (AVWS) associated with systemic lupus erythematosus (SLE). (23625134)
2014
7
Development of acquired von Willebrand syndrome during short-term micro axial pump support: implications for bleeding in a patient bridged to a long-term continuous-flow left ventricular assist device. (24614358)
2014
8
Shear-stress induced acquired von Willebrand syndrome in children with congenital heart disease. (25228244)
2014
9
Acquired von Willebrand syndrome: von Willebrand factor propeptide to von Willebrand factor antigen ratio predicts remission status. (24951428)
2014
10
Current diagnostic and therapeutic approaches to patients with acquired von Willebrand syndrome: a 2013 update. (23397553)
2013
11
Acquired von Willebrand syndrome in patients with Gaucher disease. (24275154)
2013
12
Acquired von Willebrand Syndrome with a Type 2B Phenotype: Diagnostic and Therapeutic Dilemmas. (24296552)
2013
13
Acquired von Willebrand syndrome in a patient with monoclonal gammopathy of undetermined significance. (23249617)
2013
14
Acquired von Willebrand syndrome: a rare disorder of heterogeneous etiology. (23793308)
2013
15
Hematological evaluation of acquired von Willebrand syndrome before oral surgery: Management of an unusual case. (22623844)
2012
16
Diagnosis and treatment of acquired von Willebrand syndrome. (23439003)
2012
17
Acquired Von Willebrand syndrome is an early-onset problem in ventricular assist device patients. (21515067)
2011
18
Hypertrophic obstructive cardiomyopathy, acquired von Willebrand syndrome, and gastrointestinal bleeding. (21364110)
2011
19
Acquired von Willebrand syndrome associated with monoclonal gammopathy: a single-center study of 36 patients. (22033454)
2011
20
Acquired von Willebrand syndrome in continuous-flow ventricular assist device recipients. (20868825)
2010
21
Acquired von Willebrand syndrome in patients with ventricular assist device or total artificial heart. (20352153)
2010
22
Acquired von Willebrand syndrome in patients with an axial flow left ventricular assist device. (20739614)
2010
23
Acquired von Willebrand syndrome after continuous-flow mechanical device support contributes to a high prevalence of bleeding during long-term support and at the time of transplantation. (20598466)
2010
24
Bortezomib effectiveness in one patient with acquired von Willebrand syndrome associated to monoclonal gammopathy of undetermined significance. (20425807)
2010
25
Mechanisms underlying acquired von Willebrand syndrome associated with an IgM paraprotein. (19572993)
2009
26
Acquired von Willebrand syndrome after exchange of the HeartMate XVE to the HeartMate II ventricular assist device. (19303790)
2009
27
Is rituximab effective in acquired von Willebrand syndrome? (19120364)
2009
28
Acquired von Willebrand syndrome in cardiac patients. (18922429)
2008
29
Acquired von Willebrand syndrome. (18445010)
2008
30
Association of acquired von Willebrand syndrome with AL amyloidosis. (17205535)
2007
31
Cefotaxime as the potential cause of transient acquired von Willebrand syndrome. (16529605)
2006
32
Paediatric acquired von Willebrand syndrome. (16643216)
2006
33
Acquired von Willebrand syndrome: features and management. (16823821)
2006
34
Acquired von Willebrand syndrome in a patient with Ewing sarcoma. (16651239)
2006
35
Acquired von Willebrand syndrome type IIA in a patient with aortic valve stenosis and myeloproliferative disorder. (16237526)
2006
36
A case of acquired von Willebrand syndrome successfully treated with recombinant Factor VIIa during thyroidectomy. (15457604)
2004
37
Acquired von Willebrand syndrome 2004: International Registry--diagnosis and management from online to bedside. (15029273)
2004
38
Acquired von Willebrand syndrome with autoimmune hemolytic anemia]. (15103936)
2004
39
Acquired von Willebrand syndrome: its pathophysiology, laboratory features and management. (14739623)
2003
40
Diagnosis and management of acquired von Willebrand syndrome. (16224398)
2003
41
Acquired von Willebrand syndrome in children with patent ductus arteriosus. (12067958)
2002
42
Acquired von Willebrand syndrome: experience from 2 years in a single laboratory compared with data from the literature and an international registry. (11992245)
2002
43
Acquired von Willebrand syndromes: clinical features, aetiology, pathophysiology, classification and management. (11686107)
2001
44
Therapeutic approaches to acquired von Willebrand syndrome. (11060681)
2000
45
Effectiveness of high-dose intravenous immunoglobulin in a case of acquired von Willebrand syndrome with chronic melena not responsive to desmopressin and factor VIII concentrate. (1415174)
1992
46
Acquired von Willebrand syndrome with autoimmune hemolytic anemia]. (2246822)
1990
47
The use of high-dose intravenous gamma-globulin in acquired von Willebrand syndrome. (2447856)
1988
48
Acquired von Willebrand syndrome due to an inhibitor specific for von Willebrand factor antigens. (3080872)
1986
49
Pathogenesis of antibody-induced acquired von Willebrand Syndrome. (6782864)
1980
50
Acquired von Willebrand syndrome with inhibitors both to factor VIII clotting activity and ristocetin-induced platelet aggregation. (1087568)
1976

Variations for Acquired Von Willebrand Syndrome

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Expression for genes affiliated with Acquired Von Willebrand Syndrome

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Search GEO for disease gene expression data for Acquired Von Willebrand Syndrome.

Pathways for genes affiliated with Acquired Von Willebrand Syndrome

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Compounds for genes affiliated with Acquired Von Willebrand Syndrome

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Compounds related to Acquired Von Willebrand Syndrome according to GeneCards/GeneDecks:

(show top 50)    (show all 110)
idCompoundScoreTop Affiliating Genes
1recombinate459.9VWF, F8
2kogenate459.8VWF, F8
3tributylphosphate459.8F8, VWF
4deae-sephadex a-50459.7F8, F2
5brodifacoum459.5F2, F3
6mnpt459.5F3, F2
7napsagatran459.5F3, F2
8aprosulate459.5F3, F2
9hemochron459.4F3, F2
10certoparin459.4F2, F3
11appt459.4F2, F3
12ivig459.2VWF, CD40LG, F8
13inogatran459.2F2, F3
14acetaminophen45 3 51 26 1313.2VWF, F8, F2
15heparin sodium459.1F3, F2
16dextran 70459.1F2, F3
17rituximab45 51 1311.1VWF, CD40LG, F8
18dipyridamole45 61 30 1312.0F2, F3, VWF
19kaolin459.0F8, F3, F2
20dermatan sulfate459.0VWF, F3, F2
21kininogen459.0F2, VWF, F3
22ecarin458.8F2, F3
23aprotinin45 139.8F2, VWF, F3
24polyethylene glycol458.7F8, CD40LG, F2
25cyclosporin a45 30 6110.7F8, CD40LG, VWF, F2
26hydroxyethyl starch458.6VWF, F8, F2, F3
27tranexamic acid45 139.6F8, VWF, F2, F3
28ristocetin458.6F8, VWF, F2, F3
29desmopressin45 61 30 1311.6F8, F3, VWF, F2
30warfarin45 51 26 1311.6F3, VWF, F2, F8
31lactate458.6F8, CD40LG, VWF, F2
32homocysteine45 269.5VWF, F2, F8, F3
33citrate458.5F3, F8, F2, VWF
34phospholipid458.5F8, F3, F2, VWF
35ppack458.5F3, CD40LG, VWF, F2
36tirofiban45 139.5F2, VWF, F3, CD40LG
37abciximab45 139.5F3, VWF, F2, CD40LG
38clopidogrel45 51 26 1311.5F3, VWF, F2, CD40LG
39cardiolipin45 139.4CD40LG, F8, F2, F3
40hirudin458.0F3, VWF, F2, CD40LG, F8
41phosphatidylserine45 30 1310.0F3, F8, CD40LG, VWF, F2
42aspirin45 51 30 2611.0F3, VWF, F2, CD40LG, F8
43epinephrine45 26 1310.0CD40LG, F2, VWF, F8, F3
44fibrinogen457.9F2, CD40LG, F3, F8, VWF
45creatinine457.9CD40LG, F8, VWF, F3, F2
46aspartate457.9F2, VWF, F3, F8, CD40LG
47heparin45 30 26 1310.9F8, F2, VWF, F3, CD40LG
48alanine457.9VWF, F8, F3, F2, CD40LG
49adp45 30 269.9F2, VWF, F8, CD40LG, F3
50serine457.7VWF, F3, F2, F8, CD40LG

GO Terms for genes affiliated with Acquired Von Willebrand Syndrome

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Cellular components related to Acquired Von Willebrand Syndrome according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1platelet alpha granule lumenGO:00310939.5F8, VWF
2extracellular spaceGO:00056157.7CD40LG, F8, F3, F2

Biological processes related to Acquired Von Willebrand Syndrome according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1platelet degranulationGO:00025769.5F8, VWF
2acute-phase responseGO:00069539.3F8, F2
3response to woundingGO:00096119.2VWF, F2
4blood coagulation, intrinsic pathwayGO:00075979.1F8, VWF, F2
5platelet activationGO:00301688.4CD40LG, F8, VWF, F2
6blood coagulationGO:00075968.2F2, VWF, F3, F8

Molecular functions related to Acquired Von Willebrand Syndrome according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1protease bindingGO:00020209.2F3, VWF

Products for genes affiliated with Acquired Von Willebrand Syndrome

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Sources for Acquired Von Willebrand Syndrome

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4CDC
15ExPASy
16FMA
24GTR
25HGMD
26HMDB
27ICD10
28ICD10 via Orphanet
29ICD9CM
30IUPHAR
31KEGG
35MeSH
36MESH via Orphanet
37MGI
40NCIt
41NDF-RT
44NINDS
45Novoseek
47OMIM
48OMIM via Orphanet
52PubMed
53QIAGEN
58SNOMED-CT via Orphanet
62UMLS
63UMLS via Orphanet