MCID: ACQ017
MIFTS: 40

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 49 55
Acquired Von Willebrand Disease 49 55
Willebrand Disease, Acquired 49

Characteristics:

Orphanet epidemiological data:

55
acquired von willebrand syndrome
Inheritance: Not applicable; Age of onset: Adult;

Classifications:



External Ids:

Orphanet 55 ORPHA99147
UMLS via Orphanet 70 C0272362
ICD10 via Orphanet 33 D68.4

Summaries for Acquired Von Willebrand Syndrome

NIH Rare Diseases : 49 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 von willebrand's disease and mild hemophilia a. 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, 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 88)
# Related Disease Score Top Affiliating Genes
1 von willebrand's disease 28.3 F11 F8 F9 GP1BA VWF
2 mild hemophilia a 10.2 F8 VWF
3 fainting 10.2 F8 VWF
4 von willebrand disease, type 2 10.2 F8 VWF
5 lymphoma 10.2
6 heart disease 10.2
7 cerebral arteritis 10.2 F8 VWF
8 von willebrand disease, type 1 10.2 F8 VWF
9 intracranial thrombosis 10.2 F8 VWF
10 pseudo-von willebrand disease 10.1 GP1BA VWF
11 polycythemia vera 10.1
12 aortic valve disease 2 10.1
13 hypothyroidism 10.1
14 essential thrombocythemia 10.1
15 polycythemia 10.1
16 severe hemophilia b 10.1 F8 F9
17 lymphangiosarcoma 10.1 F8 VWF
18 primary thrombocytopenia 10.1 GP1BA VWF
19 cardiac tamponade 10.1 F8 F9
20 systemic lupus erythematosus 10.1
21 myeloma, multiple 10.1
22 alpha/beta t-cell lymphopenia with gamma/delta t-cell expansion, severe cytomegalovirus infection, and autoimmunity 10.1
23 lupus erythematosus 10.1
24 factor v deficiency 10.1 F8 F9
25 coronary thrombosis 10.1 GP1BA VWF
26 thrombophilia due to thrombin defect 10.0 F8 VWF
27 factor vii deficiency 10.0 F8 F9
28 alcohol-related birth defect 10.0 F11 F8
29 afibrinogenemia, congenital 10.0 F8 VWF
30 anemia, autoimmune hemolytic 10.0
31 wilms tumor 5 10.0
32 wilms tumor 6 10.0
33 hemolytic anemia 10.0
34 leukemia 10.0
35 lymphoplasmacytic lymphoma 10.0
36 prostatitis 10.0
37 angiodysplasia 10.0
38 amyloidosis 10.0
39 al amyloidosis 10.0
40 x-linked disease 10.0 F8 F9
41 von willebrand disease, type 3 9.9 F8 GP1BA VWF
42 hemarthrosis 9.9 F8 F9 VWF
43 hemophilia 9.9 F8 F9 VWF
44 hemophilia a 9.8 F8 F9 VWF
45 thrombophilia due to activated protein c resistance 9.8 F8 F9 VWF
46 leukemia, chronic lymphocytic 2 9.8
47 lymphoma, mucosa-associated lymphoid type 9.8
48 leukemia, chronic lymphocytic 9.8
49 noonan syndrome 1 9.8
50 williams-beuren syndrome 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:

43
# Description MGI Source Accession Score Top Affiliating Genes
1 hematopoietic system MP:0005397 9.63 VWF F11 F8 F9 GP1BA JAK2
2 homeostasis/metabolism MP:0005376 9.43 JAK2 VWF F11 F8 F9 GP1BA
3 immune system MP:0005387 9.02 JAK2 VWF F11 F8 F9

Drugs & Therapeutics for Acquired Von Willebrand Syndrome

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

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

Interventional clinical trials:


# 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:

38
Heart, Prostate, Thyroid, Skin

Publications for Acquired Von Willebrand Syndrome

Articles related to Acquired Von Willebrand Syndrome:

(show top 50) (show all 174)
# Title Authors Year
1
Beneficial Effects of High-Density Lipoproteins on Acquired von Willebrand Syndrome in Aortic Valve Stenosis. ( 29443370 )
2018
2
Acquired Von Willebrand syndrome and response to desmopressin. ( 29239101 )
2018
3
Advantage of recombinant von Willebrand factor for peri-operative management in paediatric acquired von Willebrand syndrome. ( 29418043 )
2018
4
A role for intravenous immunoglobulin in the treatment of Acquired Von Willebrand Syndrome associated with IgM gammopathy. ( 29168278 )
2018
5
Pharmacokinetics-based clinical management of acquired von Willebrand syndrome: a case report. ( 29403324 )
2018
6
Acquired von Willebrand Syndrome Due to Aortic Valve Stenosis in a Case with Antiphospholipid Antibody. ( 29321442 )
2018
7
Platelet Secretion Defects and Acquired von Willebrand Syndrome in Patients With Ventricular Assist Devices. ( 29331958 )
2018
8
Acquired von Willebrand Syndrome Associated to Secondary IgM MGUS Emerging after Autologous Stem Cell Transplantation for AL Amyloidosis. ( 28512563 )
2017
9
Acquired von Willebrand syndrome in inflammatory bowel disease. ( 28314057 )
2017
10
Acquired von Willebrand Syndrome in IgM Monoclonal Gammopathy as the Presentation of Lymphoplasmacytic Lymphoma. ( 28695028 )
2017
11
Acquired von Willebrand syndrome in cardiogenic shock patients on mechanical circulatory microaxial pump support. ( 28806755 )
2017
12
Splenic Marginal Zone Lymphoma with Acquired von Willebrand Syndrome Diagnosed via Splenic Bleeding. ( 28250305 )
2017
13
Pediatric Acquired von Willebrand Syndrome in Cardiopulmonary Disorders: Do Laboratory Abnormalities Predict Bleeding Risk? ( 28060126 )
2017
14
Acquired von Willebrand Syndrome in an Infant With Coarctation of the Aorta and Williams Syndrome. ( 29113538 )
2017
15
Acquired von Willebrand syndrome in respiratory extracorporeal life support: a systematic review of the literature. ( 29084501 )
2017
16
A case of autoimmune severe acquired von Willebrand syndrome (type 3-like). ( 28512017 )
2017
17
Perioperative onset of acquired von Willebrand syndrome: Comparison between HVAD, HeartMate II and on-pump coronary bypass surgery. ( 28234916 )
2017
18
Acquired Von Willebrand syndrome in patients on long-term support with HeartMate II. ( 28082469 )
2017
19
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
20
Acquired von Willebrand syndrome in haematologic malignancies - how the clinical-laboratory correlation improves a challenging diagnosis - a case series. ( 28497886 )
2017
21
The Carmat Bioprosthetic Total Artificial Heart Is Associated With Early Hemostatic Recovery and no Acquired von Willebrand Syndrome in Calves. ( 28648774 )
2017
22
Perioperative intravenous immunoglobulin treatment in a patient with severe acquired von Willebrand syndrome: case report and review of the literature. ( 28469872 )
2017
23
Crohn's disease and acquired von Willebrand syndrome: a rare dangerous affair? ( 28103720 )
2017
24
Acquired von Willebrand Syndrome: An Under-Recognized Cause of Major Bleeding in the Cardiac Intensive Care Unit. ( 27834763 )
2016
25
Lenalidomide as a novel treatment for refractory Acquired von Willebrand Syndrome associated with monoclonal gammopathy. ( 26991062 )
2016
26
Acquired von Willebrand Syndrome. ( 28028990 )
2016
27
Diagnostic Challenges in Acquired von Willebrand Syndrome: A Complex Case of Prostate Carcinoma Associated-Acquired von Willebrand Syndrome. ( 27806384 )
2016
28
Distinct mechanisms account for acquired von Willebrand syndrome in plasma cell dyscrasias. ( 27040683 )
2016
29
Factors related to the development of acquired von Willebrand syndrome in patients with essential thrombocythemia and polycythemia vera. ( 27919526 )
2016
30
Intractable bleeding tendency due to acquired von Willebrand syndrome after Jarvik 2000 implant. ( 27021290 )
2016
31
Long-term remission of acquired von Willebrand syndrome associated with multiple myeloma using bortezomib and dexamethasone therapy. ( 27641423 )
2016
32
An 11-year experience of acquired von Willebrand syndrome in children diagnosed with Wilms tumour in a tertiary referral centre. ( 27616321 )
2016
33
Acquired von Willebrand syndrome in children with aortic and pulmonary stenosis. ( 27841910 )
2016
34
Acquired von Willebrand syndrome associated with left ventricular assist device. ( 27143258 )
2016
35
Severe Aortic Valve Stenosis: Sustained Cure of Acquired von Willebrand Syndrome After Surgical Valve Replacement. ( 27481874 )
2016
36
Bleeding diathesis associated with acquired von Willebrand Syndrome in three patients with chronic lymphocytic leukemia. ( 25926060 )
2015
37
Acquired Von Willebrand Syndrome In Aortic Stenosis: Case Report And Review. ( 26434092 )
2015
38
Continuous-flow left ventricular assist devices as a cause of acquired von Willebrand syndrome and GI bleeding. ( 25708771 )
2015
39
Acquired von Willebrand Syndrome (AVWS) as an Important Diagnostic Category of Disease. ( 25740338 )
2015
40
Factors predisposing to acquired von Willebrand syndrome during the course of polycythemia vera - retrospective analysis of 142 consecutive cases. ( 26269222 )
2015
41
Treatment of the acquired von Willebrand syndrome. ( 26577336 )
2015
42
Thalidomide for treatment of gastrointestinal bleedings due to angiodysplasia: a case report in acquired von Willebrand syndrome and review of the literature. ( 25929157 )
2015
43
Acquired von Willebrand Syndrome During the Course of Myelofibrosis: Analysis of 32 Cases. ( 26771972 )
2015
44
Impact of Acquired von Willebrand Syndrome in Severe Aortic Stenosis. ( 26370453 )
2015
45
Treatment of acquired von Willebrand syndrome in aortic stenosis with transcatheter aortic valve replacement. ( 25946442 )
2015
46
Prevalence of acquired von Willebrand syndrome during essential thrombocythemia: a retrospective analysis of 170 consecutive patients. ( 26658493 )
2015
47
Treatment of Acquired von Willebrand Syndrome and Prevention of Bleeding Postautologous Stem Cell Transplant during Severe Pancytopenia with IVIG. ( 25922770 )
2015
48
Unexpectedly High Prevalence of Acquired von Willebrand Syndrome in Patients with Severe Aortic Stenosis as Evaluated with a Novel Large Multimer Index. ( 26269004 )
2015
49
Autophagy: A potential link between Acquired von Willebrand syndrome and congenital heart disease. ( 25804464 )
2015
50
Bleeding complications after arthroscopy in a JAK2V617F-positive patient with essential thrombocythemia and acquired von Willebrand syndrome (AVWS). ( 25432436 )
2015

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:

# 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
11.22 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:

# 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:

# 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
27 GO
28 GTR
29 HGMD
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 MedGen
41 MeSH
42 MESH via Orphanet
43 MGI
45 NCI
46 NCIt
47 NDF-RT
50 NINDS
51 Novoseek
53 OMIM
54 OMIM via Orphanet
58 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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