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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NIH Rare Diseases:42 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 angiodysplasia and hypothyroidism. 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:
42NIH Rare Diseases, 48Orphanet, 26ICD10 via Orphanet, 63UMLS via Orphanet
See all sources

Acquired Von Willebrand Syndrome, Aliases & Descriptions:

Name: Acquired Von Willebrand Syndrome 42 48
Acquired Von Willebrand Disease 42 48
 
Willebrand Disease, Acquired 42


Classifications:

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


Characteristics (Orphanet epidemiological data):

48
acquired von willebrand syndrome:
Inheritance: Sporadic; Age of onset: Adulthood


External Ids:

ICD10 via Orphanet26 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
1angiodysplasia31.1VWF
2hypothyroidism31.0F8, VWF
3hemolytic anemia30.7F3, VWF
4lupus erythematosus30.5F3, F2, CD40LG
5thrombocytosis30.2F3, VWF, F2
6congenital heart disease29.9CD40LG, F8, F3, VWF, F2
7systemic lupus erythematosus29.9F2, CD40LG, F8, F3, VWF
8thrombophlebitis10.4F8
9multiple myeloma10.4
10wilms tumor10.4
11autoimmune hemolytic anemia10.4
12polycythemia vera10.4
13myeloma10.4
14polycythemia10.4
15glanzmann's thrombasthenia10.4VWF
16cardiac tamponade10.4F8
17hepatitis e10.4CD40LG
18hemorrhagic fever10.4CD40LG
19scrub typhus10.3CD40LG
20factor xii deficiency10.3F3
21leukemia10.3
22malt lymphoma10.3
23monoclonal gammopathy of uncertain significance10.3
24patent ductus arteriosus10.3
25lymphoplasmacytic lymphoma10.3
26noonan syndrome10.3
27amyloidosis10.3
28aortic valve stenosis10.3
29melanoma10.3
30nephroblastoma10.3
31sarcoma10.3
32thyroiditis10.3
33al amyloidosis10.3
34hepatocellular carcinoma10.3
35cryoglobulinemia10.3
36erythromelalgia10.3
37congenital mesoblastic nephroma10.3
38pericardial effusion10.3
39essential thrombocythemia10.3
40mesoblastic nephroma10.3
41hepatitis a10.2F2, F8
42viral hepatitis10.2F2, F8
43blood platelet disease10.2VWF, F8
44portal vein thrombosis10.2F8, F2
45blood protein disease10.1CD40LG, F2
46hemophilia10.1
47type 2a von willebrand disease10.1
48hypersensitivity reaction type ii disease10.1VWF, F8
49autoimmune hepatitis10.1CD40LG, F2
50peripheral vascular disease10.1VWF, 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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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:

32
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:

36
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 119)
idTitleAuthorsYear
1
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
2
Acquired von Willebrand syndrome in patients with a centrifugal or axial continuous flow left ventricular assist device. (24720921)
2014
3
An atypical manifestation of acquired von Willebrand syndrome (AVWS) associated with systemic lupus erythematosus (SLE). (23625134)
2014
4
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
5
Shear-stress induced acquired von Willebrand syndrome in children with congenital heart disease. (25228244)
2014
6
Acquired von Willebrand syndrome: von Willebrand factor propeptide to von Willebrand factor antigen ratio predicts remission status. (24951428)
2014
7
Acquired Von Willebrand syndrome secondary to mitral and aortic regurgitation. (24994586)
2014
8
Acquired von Willebrand Syndrome in a Child Following Berlin Heart EXCOR Pediatric Ventricular Assist Device Implantation: Case Report and Concise Literature Review. (25324261)
2014
9
Current diagnostic and therapeutic approaches to patients with acquired von Willebrand syndrome: a 2013 update. (23397553)
2013
10
Acquired von Willebrand syndrome in patients with Gaucher disease. (24275154)
2013
11
Acquired von Willebrand Syndrome with a Type 2B Phenotype: Diagnostic and Therapeutic Dilemmas. (24296552)
2013
12
Acquired von Willebrand syndrome in a patient with monoclonal gammopathy of undetermined significance. (23249617)
2013
13
Acquired von Willebrand syndrome: a rare disorder of heterogeneous etiology. (23793308)
2013
14
Hematological evaluation of acquired von Willebrand syndrome before oral surgery: Management of an unusual case. (22623844)
2012
15
Diagnosis and treatment of acquired von Willebrand syndrome. (23439003)
2012
16
Acquired Von Willebrand syndrome is an early-onset problem in ventricular assist device patients. (21515067)
2011
17
Hypertrophic obstructive cardiomyopathy, acquired von Willebrand syndrome, and gastrointestinal bleeding. (21364110)
2011
18
Acquired von Willebrand syndrome associated with monoclonal gammopathy: a single-center study of 36 patients. (22033454)
2011
19
Acquired von Willebrand syndrome in continuous-flow ventricular assist device recipients. (20868825)
2010
20
Acquired von Willebrand syndrome in patients with ventricular assist device or total artificial heart. (20352153)
2010
21
Acquired von Willebrand syndrome in patients with an axial flow left ventricular assist device. (20739614)
2010
22
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
23
Bortezomib effectiveness in one patient with acquired von Willebrand syndrome associated to monoclonal gammopathy of undetermined significance. (20425807)
2010
24
Mechanisms underlying acquired von Willebrand syndrome associated with an IgM paraprotein. (19572993)
2009
25
Acquired von Willebrand syndrome after exchange of the HeartMate XVE to the HeartMate II ventricular assist device. (19303790)
2009
26
Is rituximab effective in acquired von Willebrand syndrome? (19120364)
2009
27
Acquired von Willebrand syndrome and Wilms tumor: not always benign. (19006222)
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
Acquired von Willebrand syndrome 2004: International Registry--diagnosis and management from online to bedside. (15029273)
2004
37
Acquired von Willebrand syndrome with autoimmune hemolytic anemia]. (15103936)
2004
38
Acquired von Willebrand syndrome: its pathophysiology, laboratory features and management. (14739623)
2003
39
Diagnosis and management of acquired von Willebrand syndrome. (16224398)
2003
40
Acquired von Willebrand syndrome in children with patent ductus arteriosus. (12067958)
2002
41
Acquired von Willebrand syndrome: experience from 2 years in a single laboratory compared with data from the literature and an international registry. (11992245)
2002
42
Acquired von Willebrand syndromes: clinical features, aetiology, pathophysiology, classification and management. (11686107)
2001
43
Acquired von Willebrand syndrome type 1 in hypothyroidism: reversal after treatment with thyroxine. (11292187)
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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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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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
1recombinate449.9VWF, F8
2kogenate449.8VWF, F8
3tributylphosphate449.8F8, VWF
4deae-sephadex a-50449.7F8, F2
5brodifacoum449.5F2, F3
6mnpt449.5F3, F2
7napsagatran449.5F3, F2
8aprosulate449.5F3, F2
9hemochron449.4F3, F2
10certoparin449.4F2, F3
11appt449.4F2, F3
12ivig449.2VWF, CD40LG, F8
13inogatran449.2F2, F3
14acetaminophen44 2 50 24 1113.2VWF, F8, F2
15heparin sodium449.1F3, F2
16dextran 70449.1F2, F3
17rituximab44 50 1111.1VWF, CD40LG, F8
18dipyridamole44 61 28 1112.0F2, F3, VWF
19kaolin449.0F8, F3, F2
20dermatan sulfate449.0VWF, F3, F2
21kininogen449.0F2, VWF, F3
22ecarin448.8F2, F3
23aprotinin44 119.8F2, VWF, F3
24polyethylene glycol448.7F8, CD40LG, F2
25cyclosporin a44 28 6110.7F8, CD40LG, VWF, F2
26hydroxyethyl starch448.6VWF, F8, F2, F3
27tranexamic acid44 119.6F8, VWF, F2, F3
28ristocetin448.6F8, VWF, F2, F3
29desmopressin44 61 28 1111.6F8, F3, VWF, F2
30warfarin44 50 24 1111.6F3, VWF, F2, F8
31lactate448.6F8, CD40LG, VWF, F2
32homocysteine44 249.5VWF, F2, F8, F3
33citrate448.5F3, F8, F2, VWF
34phospholipid448.5F8, F3, F2, VWF
35ppack448.5F3, CD40LG, VWF, F2
36tirofiban44 119.5F2, VWF, F3, CD40LG
37abciximab44 119.5F3, VWF, F2, CD40LG
38clopidogrel44 50 24 1111.5F3, VWF, F2, CD40LG
39cardiolipin44 119.4CD40LG, F8, F2, F3
40hirudin448.0F3, VWF, F2, CD40LG, F8
41phosphatidylserine44 28 1110.0F3, F8, CD40LG, VWF, F2
42aspirin44 50 28 2411.0F3, VWF, F2, CD40LG, F8
43epinephrine44 24 1110.0CD40LG, F2, VWF, F8, F3
44fibrinogen447.9F2, CD40LG, F3, F8, VWF
45creatinine447.9CD40LG, F8, VWF, F3, F2
46aspartate447.9F2, VWF, F3, F8, CD40LG
47heparin44 28 24 1110.9F8, F2, VWF, F3, CD40LG
48alanine447.9VWF, F8, F3, F2, CD40LG
49adp44 28 249.9F2, VWF, F8, CD40LG, F3
50serine447.7VWF, 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: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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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
51PubMed
52QIAGEN
58SNOMED-CT via Orphanet
62UMLS
63UMLS via Orphanet