MCID: ACQ017
MIFTS: 47

Acquired Von Willebrand Syndrome malady

Rare diseases, Blood diseases categories
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Summaries for Acquired Von Willebrand Syndrome

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43NIH Rare Diseases, 33MalaCards
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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: Acquired Von Willebrand Syndrome, also known as acquired von willebrand disease, is related to hypothyroidism and lupus erythematosus. 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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43NIH Rare Diseases, 49Orphanet, 26ICD10 via Orphanet, 59SNOMED-CT via Orphanet, 63UMLS via Orphanet
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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: Sporadic; Age of onset: Adulthood


Aliases & Descriptions:

acquired von willebrand syndrome 43 49
acquired von willebrand disease 43 49
willebrand disease, acquired 43


External Ids:

ICD10 via Orphanet26 D68.4
SNOMED-CT via Orphanet59 234451005
UMLS via Orphanet63 C0272362

Related Diseases for Acquired Von Willebrand Syndrome

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17GeneCards, 18GeneDecks
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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
2lupus erythematosus30.7CD40LG, F3, F2
3hemolytic anemia30.6F3, VWF
4angiodysplasia30.6VWF
5systemic lupus erythematosus30.5F2, VWF, F3, F8, CD40LG
6thrombocytosis30.4F2, VWF, F3
7congenital heart disease30.2F2, VWF, F3, F8, CD40LG
8myeloproliferative disorder10.6
9autoimmune hemolytic anemia10.4
10multiple myeloma10.4
11myeloma10.4
12wilms tumor10.4
13polycythemia vera10.3
14monoclonal gammopathy of uncertain significance10.3
15noonan syndrome10.3
16patent ductus arteriosus10.3
17amyloidosis10.3
18aortic valve stenosis10.3
19leukemia10.3
20melanoma10.3
21nephroblastoma10.3
22polycythemia10.3
23sarcoma10.3
24thyroiditis10.3
25al amyloidosis10.3
26malt lymphoma10.3
27cryoglobulinemia10.2
28erythromelalgia10.2
29congenital mesoblastic nephroma10.2
30hepatocellular carcinoma10.2
31pericardial effusion10.2
32essential thrombocythemia10.2
33mesoblastic nephroma10.2
34thrombophlebitis10.1F8
35glanzmann's thrombasthenia10.1VWF
36cardiac tamponade10.1F8
37hepatitis e10.1CD40LG
38hemorrhagic fever10.1CD40LG
39hemophilia10.1
40type 2a von willebrand disease10.1
41scrub typhus10.1CD40LG
42factor xii deficiency10.1F3
43hepatitis a10.0F8, F2
44viral hepatitis10.0F2, F8
45blood platelet disease10.0VWF, F8
46portal vein thrombosis10.0F8, F2
47blood protein disease10.0F2, CD40LG
48hypersensitivity reaction type ii disease10.0F8, VWF
49autoimmune hepatitis10.0F2, CD40LG
50peripheral vascular disease10.0VWF, F3

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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42NIH Clinical Center, 6ClinicalTrials
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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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33MalaCards
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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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37MGI
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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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52PubMed
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Articles related to Acquired Von Willebrand Syndrome:

(show top 50)    (show all 102)
idTitleAuthorsYear
1
An atypical manifestation of acquired von Willebrand syndrome (AVWS) associated with systemic lupus erythematosus (SLE). (23625134)
2014
2
Current diagnostic and therapeutic approaches to patients with acquired von Willebrand syndrome: a 2013 update. (23397553)
2013
3
Acquired von Willebrand syndrome in patients with Gaucher disease. (24275154)
2013
4
Acquired von Willebrand Syndrome with a Type 2B Phenotype: Diagnostic and Therapeutic Dilemmas. (24296552)
2013
5
Acquired von Willebrand syndrome in a patient with monoclonal gammopathy of undetermined significance. (23249617)
2013
6
Acquired von Willebrand syndrome: a rare disorder of heterogeneous etiology. (23793308)
2013
7
Acquired von Willebrand syndrome associated with Hashimoto's thyroiditis and subcutaneous mucosa-associated lymphoid tissue lymphoma. (24292759)
2013
8
Rituximab effectiveness in a patient with juvenile systemic lupus erythematosus complicated with acquired Von Willebrand syndrome. (23989733)
2013
9
Hematological evaluation of acquired von Willebrand syndrome before oral surgery: Management of an unusual case. (22623844)
2012
10
Diagnosis and treatment of acquired von Willebrand syndrome. (23439003)
2012
11
Hypertrophic obstructive cardiomyopathy, acquired von Willebrand syndrome, and gastrointestinal bleeding. (21364110)
2011
12
Acquired von Willebrand syndrome associated with monoclonal gammopathy: a single-center study of 36 patients. (22033454)
2011
13
Hypertrophic obstructive cardiomyopathy, bleeding history, and acquired von Willebrand syndrome: response to septal myectomy. (21364113)
2011
14
Acquired von Willebrand syndrome in continuous-flow ventricular assist device recipients. (20868825)
2010
15
Acquired von Willebrand syndrome in patients with ventricular assist device or total artificial heart. (20352153)
2010
16
Acquired von Willebrand syndrome in patients with an axial flow left ventricular assist device. (20739614)
2010
17
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
18
Bortezomib effectiveness in one patient with acquired von Willebrand syndrome associated to monoclonal gammopathy of undetermined significance. (20425807)
2010
19
Investigation of acquired von Willebrand Syndrome in children with hypothyroidism: reversal after treatment with thyroxine. (21175099)
2010
20
Mechanisms underlying acquired von Willebrand syndrome associated with an IgM paraprotein. (19572993)
2009
21
Acquired von Willebrand syndrome after exchange of the HeartMate XVE to the HeartMate II ventricular assist device. (19303790)
2009
22
Is rituximab effective in acquired von Willebrand syndrome? (19120364)
2009
23
Acquired von Willebrand syndrome and Wilms tumor: not always benign. (19006222)
2009
24
Acquired von Willebrand syndrome in cardiac patients. (18922429)
2008
25
Acquired von Willebrand syndrome. (18445010)
2008
26
Association of acquired von Willebrand syndrome with AL amyloidosis. (17205535)
2007
27
Cefotaxime as the potential cause of transient acquired von Willebrand syndrome. (16529605)
2006
28
Paediatric acquired von Willebrand syndrome. (16643216)
2006
29
Acquired von Willebrand syndrome: features and management. (16823821)
2006
30
Acquired von Willebrand syndrome in a patient with Ewing sarcoma. (16651239)
2006
31
Acquired von Willebrand syndrome type IIA in a patient with aortic valve stenosis and myeloproliferative disorder. (16237526)
2006
32
Use of intravenous immunoglobulin in patients with acquired von Willebrand syndrome. (15866707)
2005
33
A case of acquired von Willebrand syndrome successfully treated with recombinant Factor VIIa during thyroidectomy. (15457604)
2004
34
Acquired von Willebrand syndrome 2004: International Registry--diagnosis and management from online to bedside. (15029273)
2004
35
Acquired von Willebrand syndrome with autoimmune hemolytic anemia]. (15103936)
2004
36
Acquired von Willebrand syndrome: its pathophysiology, laboratory features and management. (14739623)
2003
37
Diagnosis and management of acquired von Willebrand syndrome. (16224398)
2003
38
Acquired von Willebrand syndrome in children with patent ductus arteriosus. (12067958)
2002
39
Acquired von Willebrand syndrome: experience from 2 years in a single laboratory compared with data from the literature and an international registry. (11992245)
2002
40
Acquired von Willebrand syndromes: clinical features, aetiology, pathophysiology, classification and management. (11686107)
2001
41
Acquired von Willebrand syndrome type 1 in hypothyroidism: reversal after treatment with thyroxine. (11292187)
2001
42
Pharmacokinetic studies with FVIII/von Willebrand factor concentrate can be a diagnostic tool to distinguish between subgroups of patients with acquired von Willebrand syndrome. (11372672)
2001
43
Treatment of acquired von Willebrand syndrome in patients with monoclonal gammopathy of uncertain significance: comparison of three different therapeutic approaches. (9763553)
1998
44
Clinical significance of inhibitors in acquired von Willebrand syndrome. (9572997)
1998
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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2BioGPS, 15Gene Expression Omnibus DataSets
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Expression patterns in normal tissues for genes affiliated with Acquired Von Willebrand Syndrome

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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50PathCards, 55Reactome, 51PharmGKB, 53QIAGEN, 30KEGG, 38NCBI BioSystems Database, 54R&D Systems
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Compounds for genes affiliated with Acquired Von Willebrand Syndrome

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45Novoseek, 3BitterDB, 51PharmGKB, 24HMDB, 11DrugBank, 61Tocris Bioscience, 29IUPHAR
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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 24 1113.2VWF, F8, F2
15heparin sodium459.1F3, F2
16dextran 70459.1F2, F3
17rituximab45 51 1111.1VWF, CD40LG, F8
18dipyridamole45 61 29 1112.0F2, F3, VWF
19kaolin459.0F8, F3, F2
20dermatan sulfate459.0VWF, F3, F2
21kininogen459.0F2, VWF, F3
22ecarin458.8F2, F3
23aprotinin45 119.8F2, VWF, F3
24polyethylene glycol458.7F8, CD40LG, F2
25cyclosporin a45 29 6110.7F8, CD40LG, VWF, F2
26hydroxyethyl starch458.6VWF, F8, F2, F3
27tranexamic acid45 119.6F8, VWF, F2, F3
28ristocetin458.6F8, VWF, F2, F3
29desmopressin45 61 29 1111.6F8, F3, VWF, F2
30warfarin45 51 24 1111.6F3, VWF, F2, F8
31lactate458.6F8, CD40LG, VWF, F2
32homocysteine45 249.5VWF, F2, F8, F3
33citrate458.5F3, F8, F2, VWF
34phospholipid458.5F8, F3, F2, VWF
35ppack458.5F3, CD40LG, VWF, F2
36tirofiban45 119.5F2, VWF, F3, CD40LG
37abciximab45 119.5F3, VWF, F2, CD40LG
38clopidogrel45 51 24 1111.5F3, VWF, F2, CD40LG
39cardiolipin45 119.4CD40LG, F8, F2, F3
40hirudin458.0F3, VWF, F2, CD40LG, F8
41phosphatidylserine45 29 1110.0F3, F8, CD40LG, VWF, F2
42aspirin45 51 29 2411.0F3, VWF, F2, CD40LG, F8
43epinephrine45 24 1110.0CD40LG, F2, VWF, F8, F3
44fibrinogen457.9F2, CD40LG, F3, F8, VWF
45creatinine457.9CD40LG, F8, VWF, F3, F2
46aspartate457.9F2, VWF, F3, F8, CD40LG
47heparin45 29 24 1110.9F8, F2, VWF, F3, CD40LG
48alanine457.9VWF, F8, F3, F2, CD40LG
49adp45 29 249.9F2, VWF, F8, CD40LG, F3
50serine457.7VWF, F3, F2, F8, CD40LG

GO Terms for genes affiliated with Acquired Von Willebrand Syndrome

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16Gene Ontology
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Cellular components related to Acquired Von Willebrand Syndrome according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1platelet alpha granule lumenGO:0310939.5F8, VWF
2extracellular spaceGO:0056157.7CD40LG, F8, F3, F2

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

idNameGO IDScoreTop Affiliating Genes
1platelet degranulationGO:0025769.5F8, VWF
2acute-phase responseGO:0069539.3F8, F2
3response to woundingGO:0096119.2VWF, F2
4blood coagulation, intrinsic pathwayGO:0075979.1F8, VWF, F2
5platelet activationGO:0301688.4CD40LG, F8, VWF, F2
6blood coagulationGO:0075968.2F2, VWF, F3, F8

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

idNameGO IDScoreTop Affiliating Genes
1protease bindingGO:0020209.2F3, 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
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