MCID: ACQ017
MIFTS: 36

Acquired Von Willebrand Syndrome

Categories: Rare diseases, Blood diseases

Aliases & Classifications for Acquired Von Willebrand Syndrome

MalaCards integrated aliases 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 genemutation. 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 myeloproliferative neoplasm and essential thrombocythemia. 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 Arginine Vasopressin and Coagulants have been mentioned in the context of this disorder. Affiliated tissues include heart, prostate 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 76)
id Related Disease Score Top Affiliating Genes
1 myeloproliferative neoplasm 29.4 F8 GP1BA VWF
2 essential thrombocythemia 29.2 F8 F9 GP1BA VWF
3 ubqln2-related amyotrophic lateral sclerosis/frontotemporal dementia 10.4 F8 VWF
4 uchl1-related parkinson disease susceptibility 10.4 F8 VWF
5 mild hemophilia b 10.3 F8 VWF
6 floppy infant syndrome 10.3 F8 VWF
7 von willibrand disease, type 3 10.3 F8 VWF
8 femoral neuropathy 10.3 F8 VWF
9 alpha-2-plasmin inhibitor deficiency 10.3 GP1BA VWF
10 renal adenoma 10.2 F8 VWF
11 intermediate anorectal malformation 10.2 F8 F9
12 thumb stiff brachydactyly mental retardation 10.2 F8 VWF
13 retromolar area cancer 10.2 GP1BA VWF
14 autonomic neuropathy 10.2 F8 F9
15 monocular exotropia 10.2 GP1BA VWF
16 factor v deficiency 10.2 F8 F9
17 lymphoma 10.1
18 heart disease 10.1
19 female breast cancer 10.1 F11 F8
20 hypothyroidism 10.1
21 polycythemia 10.1
22 cardiomyopathy 10.1
23 pregnancy loss, recurrent 2 10.1 F8 VWF
24 factor x deficiency 10.1 F8 F9
25 systemic lupus erythematosus 10.0
26 multiple myeloma 10.0
27 lupus erythematosus 10.0
28 autosomal recessive disease 10.0 F8 F9
29 prostatic cyst 10.0 F11 F8
30 hemolytic anemia 9.9
31 leukemia 9.9
32 lymphoplasmacytic lymphoma 9.9
33 prostatitis 9.9
34 angiodysplasia 9.9
35 autoimmune hemolytic anemia 9.9
36 amyloidosis 9.9
37 al amyloidosis 9.9
38 partial sensory epilepsy 9.9 GP1BA VWF
39 ube2t-related fanconi anemia 9.9 F8 GP1BA VWF
40 glomangiomyoma 9.9 F8 F9 VWF
41 hyperphenylalaninemia 9.9 F8 F9 VWF
42 thrombophilia due to activated protein c resistance 9.9 F8 F9 VWF
43 immunodeficiency 21 9.8 F8 GP1BA VWF
44 glossopharyngeal nerve paralysis 9.8 F8 F9
45 ewing sarcoma 9.8
46 chronic lymphocytic leukemia 9.8
47 crohn's disease 9.8
48 splenic marginal zone lymphoma 9.8
49 inflammatory bowel disease 9.8
50 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 Arginine Vasopressin Phase 4
2 Coagulants Phase 4
3 Deamino Arginine Vasopressin Phase 4
4 Hemostatics Phase 4
5 Natriuretic Agents Phase 4
6 Vasopressins Phase 4
7 arginine Nutraceutical Phase 4

Interventional clinical trials:


id Name Status NCT ID Phase Drugs
1 Severe Aortic Stenosis and Acquired Von Willebrand´s Disease: The Impact of Desmopressin in Valve-Replacement Surgery Completed NCT01994330 Phase 4 desmopressin
2 Acquired Von Willebrand Syndrome in Severe Aortic Stenosis Completed NCT00805051
3 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, Prostate, Thyroid, Skin

Publications for Acquired Von Willebrand Syndrome

Articles related to Acquired Von Willebrand Syndrome:

(show top 50) (show all 165)
id Title Authors Year
1
The Carmat Bioprosthetic Total Artificial Heart Is Associated With Early Hemostatic Recovery and no Acquired von Willebrand Syndrome in Calves. ( 28648774 )
2017
2
Acquired Von Willebrand syndrome in patients on long-term support with HeartMate II. ( 28082469 )
2017
3
Splenic Marginal Zone Lymphoma with Acquired von Willebrand Syndrome Diagnosed via Splenic Bleeding. ( 28250305 )
2017
4
Acquired von Willebrand syndrome in haematologic malignancies - how the clinical-laboratory correlation improves a challenging diagnosis - a case series. ( 28497886 )
2017
5
Perioperative onset of acquired von Willebrand syndrome: Comparison between HVAD, HeartMate II and on-pump coronary bypass surgery. ( 28234916 )
2017
6
Successful management of intraperitoneal bleeding with platelet apheresis and von Willebrand factor supplementation in a patient with essential thrombocythemia and acquired von Willebrand syndrome. ( 28883275 )
2017
7
Crohn's disease and acquired von Willebrand syndrome: a rare dangerous affair? ( 28103720 )
2017
8
Perioperative intravenous immunoglobulin treatment in a patient with severe acquired von Willebrand syndrome: case report and review of the literature. ( 28469872 )
2017
9
Acquired von Willebrand syndrome in inflammatory bowel disease. ( 28314057 )
2017
10
Acquired von Willebrand syndrome in cardiogenic shock patients on mechanical circulatory microaxial pump support. ( 28806755 )
2017
11
Acquired von Willebrand Syndrome in IgM Monoclonal Gammopathy as the Presentation of Lymphoplasmacytic Lymphoma. ( 28695028 )
2017
12
Pediatric Acquired von Willebrand Syndrome in Cardiopulmonary Disorders: Do Laboratory Abnormalities Predict Bleeding Risk? ( 28060126 )
2017
13
Acquired von Willebrand Syndrome Associated to Secondary IgM MGUS Emerging after Autologous Stem Cell Transplantation for AL Amyloidosis. ( 28512563 )
2017
14
A case of autoimmune severe acquired von Willebrand syndrome (type 3-like). ( 28512017 )
2017
15
Acquired von Willebrand Syndrome. ( 28028990 )
2016
16
Diagnostic Challenges in Acquired von Willebrand Syndrome: A Complex Case of Prostate Carcinoma Associated-Acquired von Willebrand Syndrome. ( 27806384 )
2016
17
Lenalidomide as a novel treatment for refractory Acquired von Willebrand Syndrome associated with monoclonal gammopathy. ( 26991062 )
2016
18
Acquired von Willebrand syndrome associated with left ventricular assist device. ( 27143258 )
2016
19
Intractable bleeding tendency due to acquired von Willebrand syndrome after Jarvik 2000 implant. ( 27021290 )
2016
20
Factors related to the development of acquired von Willebrand syndrome in patients with essential thrombocythemia and polycythemia vera. ( 27919526 )
2016
21
Acquired von Willebrand Syndrome: An Under-Recognized Cause of Major Bleeding in the Cardiac Intensive Care Unit. ( 27834763 )
2016
22
Long-term remission of acquired von Willebrand syndrome associated with multiple myeloma using bortezomib and dexamethasone therapy. ( 27641423 )
2016
23
Acquired von Willebrand syndrome in children with aortic and pulmonary stenosis. ( 27841910 )
2016
24
Severe Aortic Valve Stenosis: Sustained Cure of Acquired von Willebrand Syndrome After Surgical Valve Replacement. ( 27481874 )
2016
25
Distinct mechanisms account for acquired von Willebrand syndrome in plasma cell dyscrasias. ( 27040683 )
2016
26
An 11-year experience of acquired von Willebrand syndrome in children diagnosed with Wilms tumour in a tertiary referral centre. ( 27616321 )
2016
27
Treatment of Acquired von Willebrand Syndrome and Prevention of Bleeding Postautologous Stem Cell Transplant during Severe Pancytopenia with IVIG. ( 25922770 )
2015
28
Acquired Von Willebrand Syndrome In Aortic Stenosis: Case Report And Review. ( 26434092 )
2015
29
Bleeding diathesis associated with acquired von Willebrand Syndrome in three patients with chronic lymphocytic leukemia. ( 25926060 )
2015
30
Acquired von Willebrand Syndrome During the Course of Myelofibrosis: Analysis of 32 Cases. ( 26771972 )
2015
31
Unexpectedly High Prevalence of Acquired von Willebrand Syndrome in Patients with Severe Aortic Stenosis as Evaluated with a Novel Large Multimer Index. ( 26269004 )
2015
32
Acquired von Willebrand syndrome in paediatric patients with congenital heart disease: challenges in the diagnosis and management of this rare condition. ( 25495773 )
2015
33
Thalidomide for treatment of gastrointestinal bleedings due to angiodysplasia: a case report in acquired von Willebrand syndrome and review of the literature. ( 25929157 )
2015
34
Acquired von Willebrand Syndrome (AVWS) as an Important Diagnostic Category of Disease. ( 25740338 )
2015
35
Impact of Acquired von Willebrand Syndrome in Severe Aortic Stenosis. ( 26370453 )
2015
36
Prevalence of acquired von Willebrand syndrome during essential thrombocythemia: a retrospective analysis of 170 consecutive patients. ( 26658493 )
2015
37
Treatment of the acquired von Willebrand syndrome. ( 26577336 )
2015
38
Treatment of acquired von Willebrand syndrome in aortic stenosis with transcatheter aortic valve replacement. ( 25946442 )
2015
39
Bleeding complications after arthroscopy in a JAK2V617F-positive patient with essential thrombocythemia and acquired von Willebrand syndrome (AVWS). ( 25432436 )
2015
40
Continuous-flow left ventricular assist devices as a cause of acquired von Willebrand syndrome and GI bleeding. ( 25708771 )
2015
41
Autophagy: A potential link between Acquired von Willebrand syndrome and congenital heart disease. ( 25804464 )
2015
42
Factors predisposing to acquired von Willebrand syndrome during the course of polycythemia vera - retrospective analysis of 142 consecutive cases. ( 26269222 )
2015
43
Continuous-flow Left Ventricular Assist Device, Valvular Disease, and Acquired von Willebrand Syndrome. ( 25476464 )
2014
44
Acquired Von Willebrand syndrome secondary to mitral and aortic regurgitation. ( 24994586 )
2014
45
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
46
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
47
Acquired von Willebrand syndrome: von Willebrand factor propeptide to von Willebrand factor antigen ratio predicts remission status. ( 24951428 )
2014
48
Acquired von Willebrand syndrome in adult patients with congenital heart disease. ( 25139318 )
2014
49
Shear stress-associated acquired von Willebrand syndrome in patients with mitral regurgitation. ( 25251907 )
2014
50
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

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

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 HPO
66 SNOMED-CT via Orphanet
67 TGDB
68 Tocris
69 UMLS
70 UMLS via Orphanet
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