AVWS
MCID: ACQ017
MIFTS: 46

Acquired Von Willebrand Syndrome (AVWS)

Categories: Blood diseases, Cardiovascular diseases, Genetic diseases, Immune diseases, Rare diseases, Smell/Taste diseases

Aliases & Classifications for Acquired Von Willebrand Syndrome

MalaCards integrated aliases for Acquired Von Willebrand Syndrome:

Name: Acquired Von Willebrand Syndrome 12 53 59 15
Acquired Von Willebrand Disease 53 59
Willebrand Disease, Acquired 53
Avws 12

Characteristics:

Orphanet epidemiological data:

59
acquired von willebrand syndrome
Inheritance: Not applicable; Age of onset: Adult;

Classifications:



External Ids:

Disease Ontology 12 DOID:0111146
SNOMED-CT 68 60078000
Orphanet 59 ORPHA99147
UMLS via Orphanet 74 C0272362
ICD10 via Orphanet 34 D68.4
UMLS 73 C0272362

Summaries for Acquired Von Willebrand Syndrome

NIH Rare Diseases : 53 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.

MalaCards based summary : Acquired Von Willebrand Syndrome, also known as acquired von willebrand disease, is related to essential thrombocythemia and hemophilia. 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 Vasopressins and arginine have been mentioned in the context of this disorder. Affiliated tissues include heart, bone and breast, and related phenotypes are hematopoietic system and homeostasis/metabolism

Disease Ontology : 12 A blood coagulation disease characterized by development of a defect in clotting in the absence of previous bleeding symptoms, negative familial history, and occurrence in a relatively older age. Typically this develops secondarily to other disorders, such as lymphoproliferative, myeloproliferative, cardiovascular and autoimmune disorders.

Related Diseases for Acquired Von Willebrand Syndrome

Diseases in the Von Willebrand's Disease family:

Pseudo-Von Willebrand Disease Von Willebrand Disease, Type 1
Von Willebrand Disease, Type 3 Von Willebrand Disease, Type 2
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 116)
# Related Disease Score Top Affiliating Genes
1 essential thrombocythemia 30.2 VWF JAK2 GP1BA
2 hemophilia 29.7 VWF F9 F8
3 acquired hemophilia 29.6 F9 F8 F11
4 acquired hemophilia a 29.6 F9 F8 F11
5 von willebrand's disease 29.4 VWF GP1BA F9 F8 F11
6 hemophilia a 29.4 VWF F9 F8 F11
7 glanzmann thrombasthenia 29.3 VWF GP1BA F9 F8
8 thrombosis 29.0 VWF JAK2 GP1BA F9 F8
9 monoclonal gammopathy of uncertain significance 10.4
10 heart disease 10.3
11 lymphoma 10.3
12 systemic lupus erythematosus 10.3
13 polycythemia vera 10.3
14 aortic valve disease 2 10.3
15 hypothyroidism 10.3
16 polycythemia 10.3
17 lupus erythematosus 10.3
18 myeloma, multiple 10.2
19 pulmonary hypertension 10.1
20 anemia, autoimmune hemolytic 10.1
21 wilms tumor 6 10.1
22 hemolytic anemia 10.1
23 thrombocytosis 10.1
24 angiodysplasia 10.1
25 amyloidosis 10.1
26 al amyloidosis 10.1
27 fainting 10.1 VWF F8
28 cerebral arteritis 10.1 VWF F8
29 brachydactyly, type d 10.1 F8 VWF
30 von willebrand disease, type 1 10.1 F8 VWF
31 intracranial thrombosis 10.1 F8 VWF
32 alcohol-related birth defect 10.1 F11 F8
33 lymphangiosarcoma 10.1 F8 VWF
34 hepatic vascular disease 10.0 JAK2 VWF
35 pseudo-von willebrand disease 10.0 GP1BA VWF
36 thrombophilia due to thrombin defect 10.0 F8 VWF
37 primary thrombocytopenia 10.0 GP1BA VWF
38 autoimmune disease of blood 10.0 GP1BA VWF
39 coronary thrombosis 10.0 GP1BA VWF
40 portal hypertension 10.0 JAK2 VWF
41 biliary cirrhosis, primary, 1 10.0
42 breast cancer 10.0
43 hepatocellular carcinoma 10.0
44 cryoglobulinemia, familial mixed 10.0
45 renal cell carcinoma, nonpapillary 10.0
46 rheumatoid arthritis 10.0
47 arthritis 10.0
48 mantle cell lymphoma 10.0
49 primary biliary cirrhosis 10.0
50 hemopericardium 10.0

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:

46
# 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 8)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1 Vasopressins Phase 4
2 arginine Phase 4
3 Natriuretic Agents Phase 4
4 Coagulants Phase 4
5 Deamino Arginine Vasopressin Phase 4
6 Hemostatics Phase 4
7 Arginine Vasopressin Phase 4
8 Pharmaceutical Solutions Phase 2

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 Von Willebrand Factor Concentrate During ECMO Support Recruiting NCT03613584 Phase 2 Von Willebrand Factor;Saline Solution
3 Acquired Von Willebrand Syndrome in Severe Aortic Stenosis Completed NCT00805051
4 Frequency of Hemorrhages Associated With the Functional Anomalies of Willebrand Factor in Emergency Patients Recruiting NCT03070912
5 Von Willebrand Antigen and Activity as Novel Biomarkers of Hemostasis in Inflammatory Bowel Disease Not yet recruiting NCT03715673

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:

41
Heart, Bone, Breast, Thyroid, Myeloid, B Cells, Bone Marrow

Publications for Acquired Von Willebrand Syndrome

Articles related to Acquired Von Willebrand Syndrome:

(show top 50) (show all 256)
# Title Authors Year
1
The course of acquired von Willebrand syndrome during pregnancy among patients with essential thrombocytosis. ( 29654448 )
2018
2
Beneficial Effects of High-Density Lipoproteins on Acquired von Willebrand Syndrome in Aortic Valve Stenosis. ( 29443370 )
2018
3
Acquired von Willebrand syndrome in congenital heart disease surgery: results from an observational case-series. ( 29908036 )
2018
4
Acquired von Willebrand syndrome and impaired platelet function during venovenous extracorporeal membrane oxygenation: Rapid onset and fast recovery. ( 29650295 )
2018
5
Acquired Von Willebrand syndrome and response to desmopressin. ( 29239101 )
2018
6
Advantage of recombinant von Willebrand factor for peri-operative management in paediatric acquired von Willebrand syndrome. ( 29418043 )
2018
7
Platelet Secretion Defects and Acquired von Willebrand Syndrome in Patients With Ventricular Assist Devices. ( 29331958 )
2018
8
Association between the severity of acquired von Willebrand syndrome and gastrointestinal bleeding after continuous-flow left ventricular assist device implantation. ( 29741685 )
2018
9
Acquired von Willebrand Syndrome Complicating Nephrotic Syndrome: A Case of a Patient With Membranous Nephropathy. ( 29568223 )
2018
10
Pharmacokinetics-based clinical management of acquired von Willebrand syndrome: a case report. ( 29403324 )
2018
11
Acquired von Willebrand Syndrome Due to Aortic Valve Stenosis in a Case with Antiphospholipid Antibody. ( 29321442 )
2018
12
Evaluation of the Utility of von Willebrand Factor Propeptide in the Differential Diagnosis of von Willebrand Disease and Acquired von Willebrand Syndrome. ( 29913537 )
2018
13
A role for intravenous immunoglobulin in the treatment of Acquired Von Willebrand Syndrome associated with IgM gammopathy. ( 29168278 )
2018
14
Waldenstrom macroglobulinaemia manifesting as acquired von Willebrand syndrome. ( 29984508 )
2018
15
Acquired von Willebrand syndrome during systemic mastocytosis: an analysis of 21 cases. ( 30057374 )
2018
16
Idiopathic pulmonary arterial hypertension - a unrecognized cause of high-shear high-flow haemostatic defects (otherwise referred to as acquired von Willebrand syndrome) in children. ( 30141279 )
2018
17
Administration of plasma-derived coagulation factor VIII during the perioperative period of mastectomy for breast cancer with acquired von Willebrand syndrome. ( 30225530 )
2018
18
Acquired Von Willebrand Syndrome and Blood Pump Design. ( 30370640 )
2018
19
Differences in von Willebrand factor function in type 2A von Willebrand disease and left ventricular assist device-induced acquired von Willebrand syndrome. ( 30397685 )
2018
20
Management of acquired von Willebrand syndrome. ( 30401518 )
2018
21
Severe acquired von Willebrand syndrome secondary to systemic lupus erythematosus. ( 30408840 )
2018
22
Intravenous immunoglobulin in patients with acquired Von Willebrand syndrome: A single referral centre experience. ( 30461133 )
2018
23
Combination of acquired von Willebrand syndrome (AVWS) and Glanzmann thrombasthenia in monoclonal gammopathy of uncertain significance (MGUS), a case report. ( 30534006 )
2018
24
Acquired von Willebrand Disease Secondary to Clear Cell Renal Cell Carcinoma. ( 30065959 )
2018
25
Diagnosis and Management of Acquired von Willebrand Disease in Heart Disease: A Review of the Literature. ( 30458570 )
2018
26
Acquired von Willebrand Disease Associated with Mantle Cell Lymphoma. ( 29651351 )
2018
27
Use of thromboelastography in the management of a patient with acquired von Willebrand disease undergoing cardiac bypass surgery. ( 28106909 )
2018
28
Multiple myeloma and acquired von Willebrand disease: a combined cause of preanalytical interference causing gel formation? ( 28490182 )
2018
29
Successful treatment of acquired von Willebrand disease with lenalidomide leading to dramatic resolution of intractable gastrointestinal bleeding: A case report. ( 29578269 )
2018
30
Acquired von Willebrand syndrome in haematologic malignancies - how the clinical-laboratory correlation improves a challenging diagnosis - a case series. ( 28497886 )
2017
31
The Carmat Bioprosthetic Total Artificial Heart Is Associated With Early Hemostatic Recovery and no Acquired von Willebrand Syndrome in Calves. ( 28648774 )
2017
32
Perioperative onset of acquired von Willebrand syndrome: Comparison between HVAD, HeartMate II and on-pump coronary bypass surgery. ( 28234916 )
2017
33
Pediatric Acquired von Willebrand Syndrome in Cardiopulmonary Disorders: Do Laboratory Abnormalities Predict Bleeding Risk? ( 28060126 )
2017
34
Acquired von Willebrand Syndrome in an Infant With Coarctation of the Aorta and Williams Syndrome. ( 29113538 )
2017
35
Crohn's disease and acquired von Willebrand syndrome: a rare dangerous affair? ( 28103720 )
2017
36
Acquired von Willebrand Syndrome in IgM Monoclonal Gammopathy as the Presentation of Lymphoplasmacytic Lymphoma. ( 28695028 )
2017
37
Perioperative intravenous immunoglobulin treatment in a patient with severe acquired von Willebrand syndrome: case report and review of the literature. ( 28469872 )
2017
38
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
39
Splenic Marginal Zone Lymphoma with Acquired von Willebrand Syndrome Diagnosed via Splenic Bleeding. ( 28250305 )
2017
40
Acquired von Willebrand syndrome in inflammatory bowel disease. ( 28314057 )
2017
41
Acquired Von Willebrand syndrome in patients on long-term support with HeartMate II. ( 28082469 )
2017
42
Acquired von Willebrand Syndrome Associated to Secondary IgM MGUS Emerging after Autologous Stem Cell Transplantation for AL Amyloidosis. ( 28512563 )
2017
43
A case of autoimmune severe acquired von Willebrand syndrome (type 3-like). ( 28512017 )
2017
44
Acquired von Willebrand syndrome in respiratory extracorporeal life support: a systematic review of the literature. ( 29084501 )
2017
45
Acquired von Willebrand syndrome in cardiogenic shock patients on mechanical circulatory microaxial pump support. ( 28806755 )
2017
46
Acquired von Willebrand Disease Associated with Monoclonal Gammopathy of Unknown Significance. ( 29225984 )
2017
47
Long-term remission of acquired von Willebrand syndrome associated with multiple myeloma using bortezomib and dexamethasone therapy. ( 27641423 )
2016
48
Acquired von Willebrand Syndrome: An Under-Recognized Cause of Major Bleeding in the Cardiac Intensive Care Unit. ( 27834763 )
2016
49
Factors related to the development of acquired von Willebrand syndrome in patients with essential thrombocythemia and polycythemia vera. ( 27919526 )
2016
50
Severe Aortic Valve Stenosis: Sustained Cure of Acquired von Willebrand Syndrome After Surgical Valve Replacement. ( 27481874 )
2016

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
Show member pathways
11.61 JAK2 VWF
4 11.6 F11 F8 F9 VWF
5 11.55 GP1BA VWF
6
Show member pathways
11.42 GP1BA VWF
7
Show member pathways
11.22 F11 F8 F9 GP1BA VWF
8 11.05 F11 JAK2
9 10.44 GP1BA VWF
10 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 extracellular space GO:0005615 9.26 F11 F8 F9 GP1BA
2 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
17 ExPASy
19 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
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42 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
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51 NDF-RT
54 NINDS
55 Novoseek
57 OMIM
58 OMIM via Orphanet
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 SNOMED-CT via Orphanet
71 TGDB
72 Tocris
73 UMLS
74 UMLS via Orphanet
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