MCID: ACQ017
MIFTS: 42

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 12 53 59
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
ICD10 33 D68.4
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 von willebrand's disease and fainting. 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

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

(show top 50) (show all 95)
# Related Disease Score Top Affiliating Genes
1 von willebrand's disease 27.1 F11 F8 F9 GP1BA VWF
2 fainting 10.3 F8 VWF
3 mild hemophilia a 10.3 F8 VWF
4 von willebrand disease, type 2 10.3 F8 VWF
5 cerebral arteritis 10.3 F8 VWF
6 von willebrand disease, type 1 10.3 F8 VWF
7 intracranial thrombosis 10.3 F8 VWF
8 pseudo-von willebrand disease 10.2 GP1BA VWF
9 lymphangiosarcoma 10.2 F8 VWF
10 heart disease 10.2
11 monoclonal gammopathy of uncertain significance 10.2
12 lymphoma 10.2
13 primary thrombocytopenia 10.2 GP1BA VWF
14 polycythemia vera 10.1
15 aortic valve disease 2 10.1
16 hypothyroidism 10.1
17 essential thrombocythemia 10.1
18 polycythemia 10.1
19 coronary thrombosis 10.1 GP1BA VWF
20 systemic lupus erythematosus 10.1
21 myeloma, multiple 10.1
22 lupus erythematosus 10.1
23 thrombophilia due to thrombin defect 10.0 F8 VWF
24 alcohol-related birth defect 10.0 F11 F8
25 anemia, autoimmune hemolytic 10.0
26 wilms tumor 5 10.0
27 wilms tumor 6 10.0
28 hemolytic anemia 10.0
29 leukemia 10.0
30 lymphoplasmacytic lymphoma 10.0
31 prostatitis 10.0
32 thrombocytosis 10.0
33 angiodysplasia 10.0
34 amyloidosis 10.0
35 al amyloidosis 10.0
36 portal hypertension 9.9 JAK2 VWF
37 thrombocytopenia-absent radius syndrome 9.9 GP1BA JAK2
38 leukemia, chronic lymphocytic 2 9.8
39 lymphoma, mucosa-associated lymphoid type 9.8
40 leukemia, chronic lymphocytic 9.8
41 noonan syndrome 1 9.8
42 pernicious anemia 9.8
43 williams-beuren syndrome 9.8
44 myelofibrosis 9.8
45 patent ductus arteriosus 1 9.8
46 leukemia, chronic myeloid 9.8
47 ewing sarcoma 9.8
48 membranous nephropathy 9.8
49 crohn's disease 9.8
50 splenic marginal zone lymphoma 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:

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 7)
# 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:


# 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 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:

41
Heart, Prostate, Thyroid, Skin

Publications for Acquired Von Willebrand Syndrome

Articles related to Acquired Von Willebrand Syndrome:

(show top 50) (show all 181)
# 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
Acquired von Willebrand syndrome in haematologic malignancies - how the clinical-laboratory correlation improves a challenging diagnosis - a case series. ( 28497886 )
2017
15
The Carmat Bioprosthetic Total Artificial Heart Is Associated With Early Hemostatic Recovery and no Acquired von Willebrand Syndrome in Calves. ( 28648774 )
2017
16
Perioperative onset of acquired von Willebrand syndrome: Comparison between HVAD, HeartMate II and on-pump coronary bypass surgery. ( 28234916 )
2017
17
Pediatric Acquired von Willebrand Syndrome in Cardiopulmonary Disorders: Do Laboratory Abnormalities Predict Bleeding Risk? ( 28060126 )
2017
18
Acquired von Willebrand Syndrome in an Infant With Coarctation of the Aorta and Williams Syndrome. ( 29113538 )
2017
19
Crohn's disease and acquired von Willebrand syndrome: a rare dangerous affair? ( 28103720 )
2017
20
Acquired von Willebrand Syndrome in IgM Monoclonal Gammopathy as the Presentation of Lymphoplasmacytic Lymphoma. ( 28695028 )
2017
21
Perioperative intravenous immunoglobulin treatment in a patient with severe acquired von Willebrand syndrome: case report and review of the literature. ( 28469872 )
2017
22
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
23
Splenic Marginal Zone Lymphoma with Acquired von Willebrand Syndrome Diagnosed via Splenic Bleeding. ( 28250305 )
2017
24
Acquired von Willebrand syndrome in inflammatory bowel disease. ( 28314057 )
2017
25
Acquired Von Willebrand syndrome in patients on long-term support with HeartMate II. ( 28082469 )
2017
26
Acquired von Willebrand Syndrome Associated to Secondary IgM MGUS Emerging after Autologous Stem Cell Transplantation for AL Amyloidosis. ( 28512563 )
2017
27
A case of autoimmune severe acquired von Willebrand syndrome (type 3-like). ( 28512017 )
2017
28
Acquired von Willebrand syndrome in respiratory extracorporeal life support: a systematic review of the literature. ( 29084501 )
2017
29
Acquired von Willebrand syndrome in cardiogenic shock patients on mechanical circulatory microaxial pump support. ( 28806755 )
2017
30
Long-term remission of acquired von Willebrand syndrome associated with multiple myeloma using bortezomib and dexamethasone therapy. ( 27641423 )
2016
31
Acquired von Willebrand Syndrome: An Under-Recognized Cause of Major Bleeding in the Cardiac Intensive Care Unit. ( 27834763 )
2016
32
Factors related to the development of acquired von Willebrand syndrome in patients with essential thrombocythemia and polycythemia vera. ( 27919526 )
2016
33
Severe Aortic Valve Stenosis: Sustained Cure of Acquired von Willebrand Syndrome After Surgical Valve Replacement. ( 27481874 )
2016
34
Acquired von Willebrand syndrome in children with aortic and pulmonary stenosis. ( 27841910 )
2016
35
Acquired von Willebrand Syndrome. ( 28028990 )
2016
36
An 11-year experience of acquired von Willebrand syndrome in children diagnosed with Wilms tumour in a tertiary referral centre. ( 27616321 )
2016
37
Distinct mechanisms account for acquired von Willebrand syndrome in plasma cell dyscrasias. ( 27040683 )
2016
38
Intractable bleeding tendency due to acquired von Willebrand syndrome after Jarvik 2000 implant. ( 27021290 )
2016
39
Lenalidomide as a novel treatment for refractory Acquired von Willebrand Syndrome associated with monoclonal gammopathy. ( 26991062 )
2016
40
Diagnostic Challenges in Acquired von Willebrand Syndrome: A Complex Case of Prostate Carcinoma Associated-Acquired von Willebrand Syndrome. ( 27806384 )
2016
41
Acquired von Willebrand syndrome associated with left ventricular assist device. ( 27143258 )
2016
42
Acquired Von Willebrand Syndrome In Aortic Stenosis: Case Report And Review. ( 26434092 )
2015
43
Acquired von Willebrand syndrome in paediatric patients with congenital heart disease: challenges in the diagnosis and management of this rare condition. ( 25495773 )
2015
44
Bleeding diathesis associated with acquired von Willebrand Syndrome in three patients with chronic lymphocytic leukemia. ( 25926060 )
2015
45
Continuous-flow left ventricular assist devices as a cause of acquired von Willebrand syndrome and GI bleeding. ( 25708771 )
2015
46
Treatment of acquired von Willebrand syndrome in aortic stenosis with transcatheter aortic valve replacement. ( 25946442 )
2015
47
Acquired von Willebrand Syndrome (AVWS) as an Important Diagnostic Category of Disease. ( 25740338 )
2015
48
Acquired von Willebrand Syndrome During the Course of Myelofibrosis: Analysis of 32 Cases. ( 26771972 )
2015
49
Treatment of the acquired von Willebrand syndrome. ( 26577336 )
2015
50
Factors predisposing to acquired von Willebrand syndrome during the course of polycythemia vera - retrospective analysis of 142 consecutive cases. ( 26269222 )
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
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
40 LOVD
42 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
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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