AVWS
MCID: ACQ017
MIFTS: 48

Acquired Von Willebrand Syndrome (AVWS)

Categories: Blood diseases, Cardiovascular diseases, Genetic diseases, Immune diseases, Rare diseases

Aliases & Classifications for Acquired Von Willebrand Syndrome

MalaCards integrated aliases for Acquired Von Willebrand Syndrome:

Name: Acquired Von Willebrand Syndrome 12 20 58 15
Acquired Von Willebrand Disease 20 58
Willebrand Disease, Acquired 20
Avws 12

Characteristics:

Orphanet epidemiological data:

58
acquired von willebrand syndrome
Inheritance: Not applicable; Age of onset: Adult;

Classifications:

Orphanet: 58  
Rare haematological diseases


Summaries for Acquired Von Willebrand Syndrome

GARD : 20 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.

MalaCards based summary : Acquired Von Willebrand Syndrome, also known as acquired von willebrand disease, is related to hemophilia and al amyloidosis. 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 Coagulants have been mentioned in the context of this disorder. Affiliated tissues include heart, myeloid and kidney, and related phenotypes are prolonged prothrombin time and abnormality of cardiovascular system morphology

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 310)
# Related Disease Score Top Affiliating Genes
1 hemophilia 30.8 F8 F7
2 al amyloidosis 30.7 VWF CALR
3 von willebrand's disease 30.6 VWF GP1BA F8 F7 F3 F2
4 angiodysplasia 30.5 VWF F8 F3 ADAMTS13
5 polycythemia 30.4 JAK2 F2 CALR
6 myeloproliferative neoplasm 30.4 VWF U2AF1 JAK2 CALR
7 acquired hemophilia 30.3 F8 F3
8 acquired hemophilia a 30.3 F8 F3
9 von willebrand disease, type 1 30.2 VWF GP1BA F8
10 thrombocytopenic purpura, autoimmune 30.1 GP1BA F8 ADAMTS13
11 myelofibrosis 30.1 U2AF1 JAK2 CALR
12 aortic valve insufficiency 30.1 VWF F3 F2
13 hemophilia a 30.0 VWF F8 F7 F3
14 hemopericardium 29.9 F3 F2
15 esophageal varix 29.8 F3 F2
16 polycythemia vera 29.8 VWF U2AF1 JAK2 F2 CALR
17 thrombocytosis 29.8 VWF JAK2 F3 F2 CALR
18 mitral valve stenosis 29.7 VWF F3 F2
19 active peptic ulcer disease 29.7 VWF F7 F3 F2
20 thrombasthenia 29.7 GP1BA F3 F2
21 hemolytic anemia 29.7 VWF F3 F2 ADAMTS13
22 deficiency anemia 29.4 U2AF1 JAK2 GP1BA F2 ADAMTS13
23 glanzmann thrombasthenia 1 29.3 VWF GP1BA F8 F7 F3
24 factor viii deficiency 29.3 VWF F8 F7 F3 F2
25 portal vein thrombosis 29.2 JAK2 F7 F2 CALR
26 pulmonary embolism 29.2 VWF GP1BA F8 F3 F2
27 essential thrombocythemia 29.2 VWF U2AF1 JAK2 GP1BA F3 F2
28 systemic lupus erythematosus 29.1 VWF JAK2 F3 F2 CALR ADAMTS13
29 portal hypertension 29.1 VWF JAK2 F3 F2 ADAMTS13
30 purpura 29.1 VWF GP1BA F3 F2 ADAMTS13
31 budd-chiari syndrome 29.0 JAK2 F7 F3 F2 CALR
32 factor xiii deficiency 29.0 VWF F8 F7 F3 F2
33 disseminated intravascular coagulation 28.9 VWF F7 F3 F2 ADAMTS13
34 heart disease 28.8 VWF GP1BA F8 F7 F3 F2
35 thrombosis 28.4 VWF JAK2 GP1BA F8 F7 F3
36 hemorrhagic disease 28.0 VWF JAK2 GP1BA F8 F7 F3
37 thrombocytopenia 27.8 VWF U2AF1 JAK2 GP1BA F8 F7
38 rare hemorrhagic disorder 10.8
39 monoclonal gammopathy of uncertain significance 10.6
40 hypothyroidism 10.6
41 myeloma, multiple 10.5
42 aortic valve disease 2 10.5
43 lupus erythematosus 10.4
44 atrial standstill 1 10.4
45 lymphoproliferative syndrome 10.4
46 mitral valve insufficiency 10.4
47 macroglobulinemia 10.4
48 leukemia, chronic lymphocytic 10.3
49 fainting 10.3 VWF F8
50 pediatric angiosarcoma 10.3 VWF F8

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

Human phenotypes related to Acquired Von Willebrand Syndrome:

58 31 (show all 27)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 prolonged prothrombin time 58 31 frequent (33%) Frequent (79-30%) HP:0008151
2 abnormality of cardiovascular system morphology 58 31 frequent (33%) Frequent (79-30%) HP:0030680
3 hematological neoplasm 58 31 frequent (33%) Frequent (79-30%) HP:0004377
4 reduced factor viii activity 58 31 frequent (33%) Frequent (79-30%) HP:0003125
5 reduced von willebrand factor activity 58 31 frequent (33%) Frequent (79-30%) HP:0008330
6 impaired ristocetin cofactor assay activity 58 31 frequent (33%) Frequent (79-30%) HP:0030129
7 hypotension 58 31 occasional (7.5%) Occasional (29-5%) HP:0002615
8 gastrointestinal angiodysplasia 58 31 occasional (7.5%) Occasional (29-5%) HP:0000471
9 hematuria 58 31 occasional (7.5%) Occasional (29-5%) HP:0000790
10 mitral regurgitation 58 31 occasional (7.5%) Occasional (29-5%) HP:0001653
11 pulmonic stenosis 58 31 occasional (7.5%) Occasional (29-5%) HP:0001642
12 epistaxis 58 31 occasional (7.5%) Occasional (29-5%) HP:0000421
13 bruising susceptibility 58 31 occasional (7.5%) Occasional (29-5%) HP:0000978
14 aortic valve stenosis 58 31 occasional (7.5%) Occasional (29-5%) HP:0001650
15 aortic regurgitation 58 31 occasional (7.5%) Occasional (29-5%) HP:0001659
16 hypochromic anemia 58 31 occasional (7.5%) Occasional (29-5%) HP:0001931
17 refractory anemia 58 31 occasional (7.5%) Occasional (29-5%) HP:0005505
18 normocytic anemia 58 31 occasional (7.5%) Occasional (29-5%) HP:0001897
19 metrorrhagia 58 31 occasional (7.5%) Occasional (29-5%) HP:0100608
20 menorrhagia 58 31 occasional (7.5%) Occasional (29-5%) HP:0000132
21 melena 58 31 occasional (7.5%) Occasional (29-5%) HP:0002249
22 joint hemorrhage 58 31 occasional (7.5%) Occasional (29-5%) HP:0005261
23 persistent bleeding after trauma 58 31 occasional (7.5%) Occasional (29-5%) HP:0001934
24 asthenia 58 31 occasional (7.5%) Occasional (29-5%) HP:0025406
25 intracranial hemorrhage 58 31 very rare (1%) Very rare (<4-1%) HP:0002170
26 gastrointestinal hemorrhage 58 Occasional (29-5%)
27 subcutaneous hemorrhage 58 Occasional (29-5%)

MGI Mouse Phenotypes related to Acquired Von Willebrand Syndrome:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 homeostasis/metabolism MP:0005376 9.28 ADAMTS13 CALR F2 F3 F7 F8

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 Vasopressins Phase 4
2 Coagulants Phase 4
3 Arginine Vasopressin Phase 4
4 Deamino Arginine Vasopressin Phase 4
5 Hemostatics Phase 4
6
Arginine Investigational, Nutraceutical Phase 4 74-79-3 6322
7 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 A Double-blind, Placebo-controlled Pilot Trial to Investigate the Administration of Von Willebrand Factor Concentrate (Willfact®, LFB France) in Adult Patients During Extracorporeal Membrane Oxygenation Active, not recruiting NCT03613584 Phase 2 Von Willebrand Factor;Saline Solution
3 Does an Acquired Von Willebrand Syndrome Influence Perioperative Blood Loss in Patients With Severe Aortic Stenosis Undergoing Aortic Valve Replacement? Completed NCT00805051
4 Use Of Three Types Of Synthetic Mesh Materials In Sling Surgery: A Prospective Randomized Clinical Trial Evaluating Effectiveness And Complications Completed NCT01348334
5 Study of the Frequency of Hemorrhages Associated With the Functional Anomalies of Willebrand Factor in Emergency Patients of a Veino Arterial or Veinous ECMO 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:

40
Heart, Myeloid, Kidney, Bone Marrow, Thyroid, Breast, Endothelial

Publications for Acquired Von Willebrand Syndrome

Articles related to Acquired Von Willebrand Syndrome:

(show top 50) (show all 568)
# Title Authors PMID Year
1
Differential diagnostic and treatment difficulties in a patient with acquired von Willebrand syndrome. 61
33663351 2021
2
Anticoagulation and bone marrow biopsy: is it safe to proceed? 61
33594950 2021
3
Flow-diverting stent and delayed intracranial bleeding: the case for discussing acquired von Willebrand disease. 61
32406779 2021
4
What factors determine the pregnancy outcome in patients with essential thrombocythemia? 61
33792458 2021
5
Management of a COVID-19 Patient during ECMO: Paying Attention to Acquired von Willebrand Syndrome. 61
33116032 2021
6
Use of recombinant Von Willebrand factor during transcatheter aortic valve replacement in a patient with acquired von Willebrand syndrome. 61
33626595 2021
7
Hypercoagulopathy, acquired coagulation disorders and anticoagulation before, during and after extracorporeal membrane oxygenation in COVID-19: a case series. 61
33719738 2021
8
Acquired von Willebrand syndrome in ECMO patients: A 3-year cohort study. 61
33338698 2021
9
The pathobiology of thrombosis, microvascular disease, and hemorrhage in the myeloproliferative neoplasms. 61
33649757 2021
10
Von Willebrand Factor Concentrate Administration for Acquired Von Willebrand Syndrome- Related Bleeding During Adult Extracorporeal Membrane Oxygenation. 61
32758410 2021
11
Von Willebrand disease: What does the future hold? 61
33662989 2021
12
A reappraisal of the pharmacologic management of gastrointestinal bleeding in patients with continuous flow left ventricular assist devices. 61
32870436 2021
13
Acquired bleeding disorders. 61
32476241 2021
14
Acquired von Willebrand syndrome and factor VIII in patients with moderate to severe mitral regurgitation undergoing transcatheter mitral valve repair. 61
33372698 2021
15
Acquired von Willebrand syndrome in monoclonal gammopathy - A scoping review on hemostatic management. 61
33733035 2021
16
Successful Perioperative Management of Orthotopic Cardiac Transplantation in a Pediatric Patient With Concurrent Congenital von Willebrand Disease and Acquired von Willebrand Syndrome Using Recombinant von Willebrand Factor. 61
33618961 2021
17
IVIg increases interleukin-11 levels, which in turn contribute to increased platelets, VWF and FVIII in mice and humans. 61
33512707 2021
18
To Treat or Not? Remission Induction of Acquired von Willebrand Syndrome Secondary to Chronic Lymphocytic Leukemia: A Case Report. 61
33558201 2021
19
Acquired von Willebrand syndrome in association with multiple myeloma: remission after stem cell transplant. 61
33504531 2021
20
Device-Induced Hemostatic Disorders in Mechanically Assisted Circulation. 61
33571008 2021
21
Spinal subarachnoid haematoma after neuraxial anaesthesia in a patient with polycythaemia vera. 61
33479701 2021
22
Thrombocytosis with acquired von Willebrand disease in an adolescent with sickle cell disease. 61
33489197 2021
23
Heyde Syndrome: An Unusual Cause of Gastrointestinal Bleeding. 61
33629596 2021
24
Management of a Pediatric Patient With a Left Ventricular Assist Device and Symptomatic Acquired von Willebrand Syndrome Presenting for Orthotopic Heart Transplant. 61
32772894 2020
25
Contact activation and acquired von Willebrand syndrome during neonatal extracorporeal circulation. 61
33463037 2020
26
Dynamics of Blood Flows in Aortic Stenosis: Mild, Moderate, and Severe. 61
33164999 2020
27
Detection of acquired von Willebrand syndrome after ventricular assist device by total thrombus-formation analysis system. 61
32700467 2020
28
Acquired von Willebrand syndrome secondary to mitral and aortic regurgitation. 61
32652743 2020
29
Acquired von Willebrand Syndrome Hiding Inherited von Willebrand Disease Can Explain Severe Bleeding in Patients With Aortic Stenosis. 61
32640909 2020
30
How I treat gastrointestinal bleeding in congenital and acquired von Willebrand disease. 61
32584960 2020
31
Pathologic Shear and Elongation Rates Do Not Cause Cleavage of Von Willebrand Factor by ADAMTS13 in a Purified System. 61
32952737 2020
32
Triple-Negative Essential Thrombocythemia Complicated by Thrombosis and Acquired von Willebrand Disease in a Young Man. 61
32829377 2020
33
Acquired von Willebrand syndrome: focused for hematologists. 61
32554559 2020
34
Acquired von Willebrand Syndrome in an Infant With Coarctation of the Aorta and Williams Syndrome. 61
29113538 2020
35
Advances in managing rare acquired bleeding disorders. 61
32286895 2020
36
Acquired Von Willebrand Syndrome Secondary to Langerhans Cell Histiocytosis. 61
31900848 2020
37
Essential Thrombocythemia and Acquired von Willebrand Syndrome: The Shadowlands between Thrombosis and Bleeding. 61
32629973 2020
38
Acquired von Willebrand Syndrome and Platelet Function Defects during Extracorporeal Life Support (Mechanical Circulatory Support). 61
32455462 2020
39
Pulsatility in ventricular assistance devices: A translational review focused on applied haemodynamics. 61
32653240 2020
40
Anticoagulant treatment and bleeding complications in patients with left ventricular assist devices. 61
32449431 2020
41
A Case of Acquired von Willebrand Disease Secondary to Myeloproliferative Neoplasm. 61
32774267 2020
42
Acquired von Willebrand syndrome and lymphoproliferative disorders: A case report. 61
32477542 2020
43
Essential thrombocythemia A retrospective case series. 61
32124556 2020
44
Changes in Red Blood Cell Properties and Platelet Function during Extracorporeal Membrane Oxygenation. 61
32325803 2020
45
Anticoagulation strategy in a patient on the HeartMate III® left ventricular assist device with acquired von Willebrand syndrome and recurring gastrointestinal bleeding episodes: sometimes less is more. 61
31177280 2020
46
Factors defining occurrence of ischemic and hemorrhagic strokes during continuous flow left ventricular assist device support. 61
31435873 2020
47
Pharmacovigilance and drug-induced rare diseases: Strengths of the French Network of Regional Pharmacovigilance Centres. 61
32113688 2020
48
Diagnosis and Management of Acquired von Willebrand Disease in Heart Disease: A Review of the Literature. 61
30458570 2020
49
Patients with aortic stenosis have von Willebrand factor abnormalities and increased proliferation of endothelial colony forming cells. 61
31860769 2020
50
Bleeding signs due to acquired von Willebrand syndrome at diagnosis of chronic myeloid leukaemia in children. 61
31617211 2020

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
12.78 VWF JAK2 GP1BA F8 F7 F3
2
Show member pathways
12.49 F8 F7 F3 F2
3 11.67 VWF GP1BA F2
4 11.52 VWF F8 F7 F3 F2
5
Show member pathways
11.42 VWF GP1BA F2
6
Show member pathways
11.3 VWF GP1BA F8 F7 F3 F2
7 10.61 F7 F2
8 10.48 VWF GP1BA
9 10.32 VWF GP1BA

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 region GO:0005576 9.87 VWF F8 F7 F3 F2 CALR
2 cell surface GO:0009986 9.67 GP1BA F3 CALR ADAMTS13
3 collagen-containing extracellular matrix GO:0062023 9.55 VWF F7 F3 F2 CALR
4 endoplasmic reticulum lumen GO:0005788 9.35 F8 F7 F2 CALR ADAMTS13
5 serine-type peptidase complex GO:1905286 9.26 F7 F3
6 extracellular space GO:0005615 9.23 VWF GP1BA F8 F7 F3 F2

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

(show all 15)
# Name GO ID Score Top Affiliating Genes
1 positive regulation of cell migration GO:0030335 9.72 JAK2 F7 F3
2 ER to Golgi vesicle-mediated transport GO:0006888 9.69 F8 F7 F2
3 platelet activation GO:0030168 9.65 VWF GP1BA F8 F2 ADAMTS13
4 protein processing GO:0016485 9.58 F7 F3 ADAMTS13
5 blood coagulation GO:0007596 9.56 VWF JAK2 GP1BA F8 F7 F3
6 acute-phase response GO:0006953 9.54 F8 F2
7 response to tumor necrosis factor GO:0034612 9.52 JAK2 ADAMTS13
8 fibrinolysis GO:0042730 9.49 GP1BA F2
9 positive regulation of blood coagulation GO:0030194 9.48 F7 F2
10 regulation of blood coagulation GO:0030193 9.46 GP1BA F2
11 blood coagulation, intrinsic pathway GO:0007597 9.46 VWF GP1BA F8 F2
12 positive regulation of positive chemotaxis GO:0050927 9.43 F7 F3
13 positive regulation of platelet-derived growth factor receptor signaling pathway GO:0010641 9.37 F7 F3
14 blood coagulation, extrinsic pathway GO:0007598 9.32 F7 F3
15 hemostasis GO:0007599 9.17 VWF GP1BA F8 F7 F3 F2

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

# Name GO ID Score Top Affiliating Genes
1 calcium ion binding GO:0005509 9.46 F7 F2 CALR ADAMTS13
2 serine-type endopeptidase activity GO:0004252 9.13 F7 F3 F2
3 integrin binding GO:0005178 8.8 VWF CALR ADAMTS13

Sources for Acquired Von Willebrand Syndrome

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
20 GARD
28 GO
29 GTR
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 LOVD
41 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
53 NINDS
54 Novoseek
56 OMIM via Orphanet
57 OMIM® (Updated 20-May-2021)
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 Tocris
70 UMLS
71 UMLS via Orphanet
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