MCID: ACQ017
MIFTS: 37

Acquired Von Willebrand Syndrome malady

Categories: Rare diseases, Blood diseases

Aliases & Classifications for Acquired Von Willebrand Syndrome

Aliases & Descriptions for Acquired Von Willebrand Syndrome:

Name: Acquired Von Willebrand Syndrome 50 56
Acquired Von Willebrand Disease 50 56
Willebrand Disease, Acquired 50

Characteristics:

Orphanet epidemiological data:

56
acquired von willebrand syndrome
Inheritance: Not applicable; Age of onset: Adult;

Classifications:



External Ids:

Orphanet 56 ORPHA99147
UMLS via Orphanet 70 C0272362
ICD10 via Orphanet 34 D68.4

Summaries for Acquired Von Willebrand Syndrome

NIH Rare Diseases : 50 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/superpathways are Response to elevated platelet cytosolic Ca2+ and Collagen chain trimerization. The drugs Natriuretic Agents and Coagulants have been mentioned in the context of this disorder. Affiliated tissues include heart, skin and thyroid, and related phenotypes are hematopoietic system and homeostasis/metabolism

Related Diseases for Acquired Von Willebrand Syndrome

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

(show top 50) (show all 71)
id Related Disease Score Top Affiliating Genes
1 essential thrombocythemia 29.7 F8 F9 GP1BA VWF
2 myeloproliferative neoplasm 29.2 F8 GP1BA JAK2 VWF
3 vcl-related familial hypertrophic cardiomyopathy 10.2 F8 VWF
4 vcp-related amyotrophic lateral sclerosis/frontotemporal dementia 10.2 F8 VWF
5 high anorectal malformation 10.2 F8 VWF
6 headache 10.1 F8 VWF
7 alpha-2-macroglobulin deficiency 10.1 F8 VWF
8 hypertrichosis 10.1 F8 VWF
9 heart disease 10.1
10 trismus-pseudocamptodactyly syndrome 10.1 GP1BA VWF
11 primary peritoneal carcinoma 10.1 F8 F9
12 renal adenoma 10.1 F8 VWF
13 severe nonproliferative diabetic retinopathy 10.1 GP1BA VWF
14 monocular exotropia 10.1 GP1BA VWF
15 thyroid cancer, anaplastic 10.1 F8 VWF
16 autonomic neuropathy 10.1 F8 F9
17 hypothyroidism 10.1
18 polycythemia 10.1
19 cardiomyopathy 10.1
20 dyskinetic cerebral palsy 10.1 F11 F8
21 spinocerebellar ataxia 27 10.0 F8 F9
22 immunodeficiency, common variable, 6 10.0 F8 VWF
23 immunodeficiency 21 10.0 GP1BA VWF
24 systemic lupus erythematosus 10.0
25 multiple myeloma 10.0
26 lymphoma 10.0
27 lupus erythematosus 10.0
28 prostatic cyst 10.0 F11 F8
29 cranial nerve palsy 10.0 F8 F9
30 vcl-related dilated cardiomyopathy 10.0 F8 GP1BA VWF
31 pineal region meningioma 10.0 F8 F9 VWF
32 myotonic dystrophy 10.0 F8 F9 VWF
33 pregnancy loss, recurrent 1 10.0 F8 F9 VWF
34 giant papillary conjunctivitis 10.0 F8 F9 VWF
35 prostatitis 9.9
36 angiodysplasia 9.9
37 hemolytic anemia 9.9
38 leukemia 9.9
39 autoimmune hemolytic anemia 9.9
40 aids dementia complex 9.9 F11 F8 VWF
41 oguchi disease-2 9.9 F11 F9 VWF
42 acrodysplasia with ossification abnormalities, short stature and fibular hypoplasia 9.9 F11 F8 F9
43 acrodysplasia scoliosis 9.9 F11 F8 F9
44 scapuloperoneal myopathy, x-linked dominant 9.9 F11 F8 F9
45 pregnancy loss, recurrent 2 9.9 F11 F8 F9
46 haemonchiasis 9.9 GP1BA VWF
47 fiedler's myocarditis 9.8 JAK2 VWF
48 pyridoxamine 5'-phosphate oxidase deficiency 9.8 F8 F9 GP1BA VWF
49 melanoma 9.8
50 ewing sarcoma 9.8

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

MGI Mouse Phenotypes related to Acquired Von Willebrand Syndrome:

44
id Description MGI Source Accession Score Top Affiliating Genes
1 hematopoietic system MP:0005397 9.63 F11 F8 F9 GP1BA JAK2 VWF
2 homeostasis/metabolism MP:0005376 9.43 F11 F8 F9 GP1BA JAK2 VWF
3 immune system MP:0005387 9.02 F11 F8 F9 JAK2 VWF

Drugs & Therapeutics for Acquired Von Willebrand Syndrome

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

(show all 7)
id Name Status Phase Clinical Trials Cas Number PubChem Id
1 Natriuretic Agents Phase 4
2 Coagulants Phase 4
3 Hemostatics Phase 4
4 Deamino Arginine Vasopressin Phase 4
5 Vasopressins Phase 4
6 Arginine Vasopressin Phase 4
7 arginine Nutraceutical Phase 4

Interventional clinical trials:


id Name Status NCT ID Phase
1 Severe Aortic Stenosis and Acquired Von Willebrand´s Disease: The Impact of Desmopressin in Valve-Replacement Surgery Completed NCT01994330 Phase 4
2 Acquired Von Willebrand Syndrome in Severe Aortic Stenosis Completed NCT00805051
3 ADAMTS13 in Thrombotic Thrombocytopenic Purpura Completed NCT00426686
4 Frequency of Hemorrhages Associated With the Functional Anomalies of Willebrand Factor in Emergency Patients Not yet recruiting NCT03070912

Search NIH Clinical Center for Acquired Von Willebrand Syndrome

Genetic Tests for Acquired Von Willebrand Syndrome

Anatomical Context for Acquired Von Willebrand Syndrome

MalaCards organs/tissues related to Acquired Von Willebrand Syndrome:

39
Heart, Skin, Thyroid, Prostate

Publications for Acquired Von Willebrand Syndrome

Articles related to Acquired Von Willebrand Syndrome:

(show top 50) (show all 153)
id Title Authors Year
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

Expression for Acquired Von Willebrand Syndrome

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

Pathways for Acquired Von Willebrand Syndrome

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

id Super pathways Score Top Affiliating Genes
1
Show member pathways
12.51 F11 F8 F9 GP1BA JAK2 VWF
2
Show member pathways
12.3 F11 F8 F9
3 11.6 F11 F8 F9 VWF
4 11.55 GP1BA VWF
5
Show member pathways
11.42 GP1BA VWF
6
Show member pathways
11.23 F11 F8 F9 GP1BA VWF
7 11.05 F11 JAK2
8 10.44 GP1BA VWF
9 10.26 GP1BA VWF

GO Terms for Acquired Von Willebrand Syndrome

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

id Name GO ID Score Top Affiliating Genes
1 platelet alpha granule lumen GO:0031093 8.62 F8 VWF

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

id Name GO ID Score Top Affiliating Genes
1 hemostasis GO:0007599 9.55 F11 F8 F9 GP1BA VWF
2 platelet activation GO:0030168 9.43 F8 GP1BA VWF
3 blood coagulation GO:0007596 9.43 F11 F8 F9 GP1BA JAK2 VWF
4 platelet degranulation GO:0002576 9.37 F8 VWF
5 regulation of blood coagulation GO:0030193 9.32 F11 GP1BA
6 blood coagulation, intrinsic pathway GO:0007597 9.02 F11 F8 F9 GP1BA VWF

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

id Name GO ID Score Top Affiliating Genes
1 serine-type endopeptidase activity GO:0004252 8.8 F11 F8 F9

Sources for Acquired Von Willebrand Syndrome

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
16 ExPASy
18 FMA
28 GO
29 GTR
30 HGMD
31 HMDB
32 HPO
33 ICD10
34 ICD10 via Orphanet
35 ICD9CM
36 IUPHAR
37 KEGG
38 LifeMap
40 MedGen
42 MeSH
43 MESH via Orphanet
44 MGI
46 NCI
47 NCIt
48 NDF-RT
51 NINDS
52 Novoseek
54 OMIM
55 OMIM via Orphanet
59 PubMed
60 QIAGEN
65 SNOMED-CT via Orphanet
67 TGDB
68 Tocris
69 UMLS
70 UMLS via Orphanet
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