MCID: ACQ017
MIFTS: 37

Acquired Von Willebrand Syndrome malady

Categories: Rare diseases, Blood diseases

Aliases & Classifications for Acquired Von Willebrand Syndrome

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Sources:
31ICD10 via Orphanet, 48NIH Rare Diseases, 54Orphanet, 69UMLS via Orphanet
See all MalaCards sources

Aliases & Descriptions for Acquired Von Willebrand Syndrome:

Name: Acquired Von Willebrand Syndrome 48 54
Acquired Von Willebrand Disease 48 54
 
Willebrand Disease, Acquired 48

Characteristics:

Orphanet epidemiological data:

54
acquired von willebrand syndrome:
Inheritance: Not applicable; Age of onset: Adult

Classifications:



External Ids:

Orphanet54 ORPHA99147
UMLS via Orphanet69 C0272362
ICD10 via Orphanet31 D68.4

Summaries for Acquired Von Willebrand Syndrome

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NIH Rare Diseases:48 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 essential thrombocythemia and myeloproliferative neoplasm. An important gene associated with Acquired Von Willebrand Syndrome is VWF (Von Willebrand Factor), and among its related pathways are Integrin alphaIIb beta3 signaling and Platelet activation. Affiliated tissues include heart, prostate and thyroid, and related mouse phenotypes are hematopoietic system and homeostasis/metabolism.

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 Suite gene sharing:

(show top 50)    (show all 71)
idRelated DiseaseScoreTop Affiliating Genes
1essential thrombocythemia29.7F8, F9, GP1BA, VWF
2myeloproliferative neoplasm29.2F8, GP1BA, JAK2, VWF
3vcl-related familial hypertrophic cardiomyopathy10.2F8, VWF
4vcp-related amyotrophic lateral sclerosis/frontotemporal dementia10.2F8, VWF
5high anorectal malformation10.2F8, VWF
6headache10.1F8, VWF
7alpha-2-macroglobulin deficiency10.1F8, VWF
8hypertrichosis10.1F8, VWF
9heart disease10.1
10trismus-pseudocamptodactyly syndrome10.1GP1BA, VWF
11primary peritoneal carcinoma10.1F8, F9
12renal adenoma10.1F8, VWF
13severe nonproliferative diabetic retinopathy10.1GP1BA, VWF
14monocular exotropia10.1GP1BA, VWF
15thyroid cancer, anaplastic10.1F8, VWF
16autonomic neuropathy10.1F8, F9
17hypothyroidism10.1
18polycythemia10.1
19cardiomyopathy10.1
20dyskinetic cerebral palsy10.1F11, F8
21spinocerebellar ataxia 2710.0F8, F9
22immunodeficiency, common variable, 610.0F8, VWF
23immunodeficiency 2110.0GP1BA, VWF
24systemic lupus erythematosus10.0
25multiple myeloma10.0
26lymphoma10.0
27lupus erythematosus10.0
28prostatic cyst10.0F11, F8
29cranial nerve palsy10.0F8, F9
30vcl-related dilated cardiomyopathy10.0F8, GP1BA, VWF
31pineal region meningioma10.0F8, F9, VWF
32myotonic dystrophy10.0F8, F9, VWF
33pregnancy loss, recurrent 110.0F8, F9, VWF
34giant papillary conjunctivitis10.0F8, F9, VWF
35hemolytic anemia9.9
36leukemia9.9
37prostatitis9.9
38angiodysplasia9.9
39autoimmune hemolytic anemia9.9
40aids dementia complex9.9F11, F8, VWF
41oguchi disease-29.9F11, F9, VWF
42acrodysplasia with ossification abnormalities, short stature and fibular hypoplasia9.9F11, F8, F9
43acrodysplasia scoliosis9.9F11, F8, F9
44scapuloperoneal myopathy, x-linked dominant9.9F11, F8, F9
45pregnancy loss, recurrent 29.9F11, F8, F9
46haemonchiasis9.9GP1BA, VWF
47fiedler's myocarditis9.8JAK2, VWF
48pyridoxamine 5'-phosphate oxidase deficiency9.8F8, F9, GP1BA, VWF
49ewing sarcoma9.8
50chronic lymphocytic leukemia9.8

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



Diseases related to acquired von willebrand syndrome

Symptoms & Phenotypes for Acquired Von Willebrand Syndrome

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MGI Mouse Phenotypes related to Acquired Von Willebrand Syndrome according to GeneCards Suite gene sharing:

41
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00053977.9F11, F8, F9, GP1BA, JAK2, VWF
2MP:00053767.9F11, F8, F9, GP1BA, JAK2, VWF
3MP:00053877.6F11, F8, F9, JAK2, VWF

Drugs & Therapeutics for Acquired Von Willebrand Syndrome

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Drugs for Acquired Von Willebrand Syndrome (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show all 7)
idNameStatusPhaseClinical TrialsCas NumberPubChem Id
1Natriuretic AgentsPhase 41697
2VasopressinsPhase 4294
3HemostaticsPhase 41443
4Deamino Arginine VasopressinPhase 477
5Arginine VasopressinPhase 4299
6CoagulantsPhase 41500
7arginineNutraceuticalPhase 4425

Interventional clinical trials:

idNameStatusNCT IDPhase
1Severe Aortic Stenosis and Acquired Von Willebrand´s Disease: The Impact of Desmopressin in Valve-Replacement SurgeryCompletedNCT01994330Phase 4
2Acquired Von Willebrand Syndrome in Severe Aortic StenosisCompletedNCT00805051
3ADAMTS13 in Thrombotic Thrombocytopenic PurpuraCompletedNCT00426686
4Frequency of Hemorrhages Associated With the Functional Anomalies of Willebrand Factor in Emergency PatientsNot yet recruitingNCT03070912

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:

36
Heart, Prostate, Thyroid, Skin

Publications for Acquired Von Willebrand Syndrome

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Articles related to Acquired Von Willebrand Syndrome:

(show top 50)    (show all 153)
idTitleAuthorsYear
1
Acquired Von Willebrand syndrome in patients on long-term support with HeartMate II. (28082469)
2017
2
Pediatric Acquired von Willebrand Syndrome in Cardiopulmonary Disorders: Do Laboratory Abnormalities Predict Bleeding Risk? (28060126)
2017
3
Intractable bleeding tendency due to acquired von Willebrand syndrome after Jarvik 2000 implant. (27021290)
2016
4
Acquired von Willebrand Syndrome. (28028990)
2016
5
Factors related to the development of acquired von Willebrand syndrome in patients with essential thrombocythemia and polycythemia vera. (27919526)
2016
6
Severe Aortic Valve Stenosis: Sustained Cure of Acquired von Willebrand Syndrome After Surgical Valve Replacement. (27481874)
2016
7
Acquired von Willebrand syndrome associated with left ventricular assist device. (27143258)
2016
8
Distinct mechanisms account for acquired von Willebrand syndrome in plasma cell dyscrasias. (27040683)
2016
9
Acquired von Willebrand Syndrome: An Under-Recognized Cause of Major Bleeding in the Cardiac Intensive Care Unit. (27834763)
2016
10
Acquired von Willebrand syndrome in children with aortic and pulmonary stenosis. (27841910)
2016
11
An 11-year experience of acquired von Willebrand syndrome in children diagnosed with Wilms tumour in a tertiary referral centre. (27616321)
2016
12
Diagnostic Challenges in Acquired von Willebrand Syndrome: A Complex Case of Prostate Carcinoma Associated-Acquired von Willebrand Syndrome. (27806384)
2016
13
Lenalidomide as a novel treatment for refractory Acquired von Willebrand Syndrome associated with monoclonal gammopathy. (26991062)
2016
14
Long-term remission of acquired von Willebrand syndrome associated with multiple myeloma using bortezomib and dexamethasone therapy. (27641423)
2016
15
Bleeding diathesis associated with acquired von Willebrand Syndrome in three patients with chronic lymphocytic leukemia. (25926060)
2015
16
Unexpectedly High Prevalence of Acquired von Willebrand Syndrome in Patients with Severe Aortic Stenosis as Evaluated with a Novel Large Multimer Index. (26269004)
2015
17
Acquired von Willebrand Syndrome (AVWS) as an Important Diagnostic Category of Disease. (25740338)
2015
18
Acquired von Willebrand syndrome in paediatric patients with congenital heart disease: challenges in the diagnosis and management of this rare condition. (25495773)
2015
19
Bleeding complications after arthroscopy in a JAK2V617F-positive patient with essential thrombocythemia and acquired von Willebrand syndrome (AVWS). (25432436)
2015
20
Acquired von Willebrand Syndrome During the Course of Myelofibrosis: Analysis of 32 Cases. (26771972)
2015
21
Acquired Von Willebrand Syndrome In Aortic Stenosis: Case Report And Review. (26434092)
2015
22
Continuous-flow left ventricular assist devices as a cause of acquired von Willebrand syndrome and GI bleeding. (25708771)
2015
23
Prevalence of acquired von Willebrand syndrome during essential thrombocythemia: a retrospective analysis of 170 consecutive patients. (26658493)
2015
24
Factors predisposing to acquired von Willebrand syndrome during the course of polycythemia vera - retrospective analysis of 142 consecutive cases. (26269222)
2015
25
Treatment of the acquired von Willebrand syndrome. (26577336)
2015
26
Treatment of acquired von Willebrand syndrome in aortic stenosis with transcatheter aortic valve replacement. (25946442)
2015
27
Autophagy: A potential link between Acquired von Willebrand syndrome and congenital heart disease. (25804464)
2015
28
Treatment of Acquired von Willebrand Syndrome and Prevention of Bleeding Postautologous Stem Cell Transplant during Severe Pancytopenia with IVIG. (25922770)
2015
29
Impact of Acquired von Willebrand Syndrome in Severe Aortic Stenosis. (26370453)
2015
30
Thalidomide for treatment of gastrointestinal bleedings due to angiodysplasia: a case report in acquired von Willebrand syndrome and review of the literature. (25929157)
2015
31
Resolution of acquired von Willebrand syndrome after transcatheter aortic valve implantation through a left transcarotid approach. (25129274)
2014
32
Salvage therapy with high dose Intravenous Immunoglobulins in acquired Von Willebrand Syndrome and unresponsive severe intestinal bleeding. (24926417)
2014
33
Correction of acquired von Willebrand syndrome by transcatheter aortic valve implantation. (25480995)
2014
34
Remission of recurrent gastrointestinal bleeding after septal reduction therapy in patients with hypertrophic obstructive cardiomyopathy-associated acquired von Willebrand syndrome. (25387993)
2014
35
Shear-stress induced acquired von Willebrand syndrome in children with congenital heart disease. (25228244)
2014
36
Shear stress-associated acquired von Willebrand syndrome in patients with mitral regurgitation. (25251907)
2014
37
An atypical manifestation of acquired von Willebrand syndrome (AVWS) associated with systemic lupus erythematosus (SLE). (23625134)
2014
38
Acquired von Willebrand syndrome in adult patients with congenital heart disease. (25139318)
2014
39
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
40
Platelet dysfunction and acquired von Willebrand syndrome in patients with left ventricular assist devicesa8 (25543177)
2014
41
Early Diagnosis of Acquired von Willebrand Syndrome (AVWS) is Elementary for Clinical Practice in Patients Treated with ECMO Therapy. (25186021)
2014
42
Lymphoplasmacytic lymphoma exposed by haemoptysis and acquired von Willebrand syndrome. (24469392)
2014
43
Acquired von Willebrand syndrome in patients with a centrifugal or axial continuous flow left ventricular assist device. (24720921)
2014
44
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
45
Immediate Recovery of Acquired von Willebrand Syndrome after Left Ventricular Assist Device Explantation: Implications for Heart Transplantation. (25251586)
2014
46
A two-step approach (Enzyme-linked immunosorbent assay and confirmation assay) to detect antibodies against von Willebrand factor in patients with Acquired von Willebrand Syndrome. (25300810)
2014
47
Acquired Von Willebrand syndrome secondary to mitral and aortic regurgitation. (24994586)
2014
48
Acquired von Willebrand syndrome: von Willebrand factor propeptide to von Willebrand factor antigen ratio predicts remission status. (24951428)
2014
49
Acquired von Willebrand Syndrome in a Child Following Berlin Heart EXCOR Pediatric Ventricular Assist Device Implantation: Case Report and Concise Literature Review. (25324261)
2014
50
Continuous-flow Left Ventricular Assist Device, Valvular Disease, and Acquired von Willebrand Syndrome. (25476464)
2014

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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Pathways related to Acquired Von Willebrand Syndrome according to GeneCards Suite gene sharing:

idSuper pathwaysScoreTop Affiliating Genes
1
Show member pathways
9.5GP1BA, VWF
29.5GP1BA, VWF
39.5GP1BA, VWF
49.5GP1BA, VWF
59.0F11, JAK2
6
Show member pathways
8.9F11, F8, F9
7
Show member pathways
8.5F11, F8, F9, VWF
8
Show member pathways
7.9F11, F8, F9, GP1BA, VWF
9
Show member pathways
7.1F11, F8, F9, GP1BA, JAK2, VWF

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 Suite gene sharing:

idNameGO IDScoreTop Affiliating Genes
1platelet alpha granule lumenGO:00310939.6F8, VWF

Biological processes related to Acquired Von Willebrand Syndrome according to GeneCards Suite gene sharing:

idNameGO IDScoreTop Affiliating Genes
1platelet degranulationGO:000257610.0F8, VWF
2platelet activationGO:00301689.6F8, GP1BA, VWF
3regulation of blood coagulationGO:00301939.3F11, GP1BA
4blood coagulation, intrinsic pathwayGO:00075978.6F11, F8, F9, GP1BA, VWF
5hemostasisGO:00075998.5F11, F8, F9, GP1BA, VWF
6blood coagulationGO:00075967.7F11, F8, F9, GP1BA, JAK2, VWF

Molecular functions related to Acquired Von Willebrand Syndrome according to GeneCards Suite gene sharing:

idNameGO IDScoreTop Affiliating Genes
1serine-type endopeptidase activityGO:00042528.9F11, F8, F9

Sources for Acquired Von Willebrand Syndrome

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2CDC
6CNVD
10DGIdb
15ExPASy
16FDA
17FMA
27GTR
28HGMD
29HMDB
30ICD10
31ICD10 via Orphanet
32ICD9CM
33IUPHAR
34KEGG
37MedGen
39MeSH
40MESH via Orphanet
41MGI
44NCI
45NCIt
46NDF-RT
49NINDS
50Novoseek
52OMIM
53OMIM via Orphanet
57PubMed
58QIAGEN
63SNOMED-CT via Orphanet
67Tumor Gene Family of Databases
68UMLS
69UMLS via Orphanet