AVWS
MCID: ACQ017
MIFTS: 42

Acquired Von Willebrand Syndrome (AVWS)

Categories: Blood 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 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
ICD10 via Orphanet 34 D68.4
UMLS via Orphanet 73 C0272362
Orphanet 59 ORPHA99147
UMLS 72 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 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 von willebrand's disease and von willebrand disease, type 1. 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 Hemostatics and Coagulants have been mentioned in the context of this disorder. Affiliated tissues include heart, thyroid and testes, 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 187)
# Related Disease Score Top Affiliating Genes
1 von willebrand's disease 30.8 VWF GP1BA F9 F8 F11
2 von willebrand disease, type 1 30.6 VWF F8
3 thrombophilia due to thrombin defect 30.1 VWF F8
4 factor viii deficiency 30.1 VWF F8 F11
5 essential thrombocythemia 30.0 VWF JAK2 GP1BA
6 portal hypertension 29.9 VWF JAK2
7 hemophilia 29.8 VWF F9 F8
8 pulmonary embolism 29.5 VWF F9
9 acquired hemophilia 29.1 F9 F8 F11
10 acquired hemophilia a 29.1 F9 F8 F11
11 hemophilia a 28.9 VWF F9 F8 F11
12 glanzmann thrombasthenia 28.4 VWF GP1BA F9 F8
13 hemorrhagic disease 27.7 VWF GP1BA F9 F8 F11
14 monoclonal gammopathy of uncertain significance 10.6
15 hypothyroidism 10.5
16 angiodysplasia 10.5
17 autoimmune disease 10.4
18 thrombocytosis 10.4
19 myeloma, multiple 10.4
20 polycythemia vera 10.4
21 polycythemia 10.4
22 systemic lupus erythematosus 10.4
23 aortic valve disease 2 10.4
24 lupus erythematosus 10.4
25 atrial standstill 1 10.3
26 wilms tumor 5 10.3
27 heart disease 10.3
28 myeloproliferative neoplasm 10.3
29 deficiency anemia 10.3
30 thrombocytopenia 10.3
31 macroglobulinemia 10.3
32 fainting 10.3 VWF F8
33 cerebral arteritis 10.3 VWF F8
34 brachydactyly, type d 10.2 VWF F8
35 leukemia, chronic lymphocytic 10.2
36 anemia, autoimmune hemolytic 10.2
37 lymphoplasmacytic lymphoma 10.2
38 lymphoproliferative syndrome 10.2
39 lymphocytic leukemia 10.2
40 mitral valve insufficiency 10.2
41 hemolytic anemia 10.2
42 active peptic ulcer disease 10.2
43 amyloidosis 10.2
44 hypertrophic cardiomyopathy 10.2
45 al amyloidosis 10.2
46 leukemia, b-cell, chronic 10.2
47 intracranial thrombosis 10.2 VWF F8
48 hashimoto thyroiditis 10.1
49 lymphoma, hodgkin, classic 10.1
50 lymphoma, non-hodgkin, familial 10.1

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 21)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1 Hemostatics Phase 4
2 Coagulants Phase 4
3 Natriuretic Agents Phase 4
4 Deamino Arginine Vasopressin Phase 4
5 Vasopressins Phase 4
6 arginine Phase 4
7 Arginine Vasopressin Phase 4
8 Protamines Phase 4
9
Anagrelide Approved Phase 2 68475-42-3 2182
10
Hydroxyurea Approved Phase 2 127-07-1 3657
11
Busulfan Approved, Investigational Phase 2 55-98-1 2478
12
Tyrosine Approved, Investigational, Nutraceutical Phase 2 60-18-4 6057
13 Pharmaceutical Solutions Phase 2
14 Nucleic Acid Synthesis Inhibitors Phase 2
15 Adjuvants, Immunologic Phase 2
16 interferons Phase 2
17
Octreotide Approved, Investigational Phase 1 83150-76-9 383414 6400441
18 Gastrointestinal Agents Phase 1
19 Hormones Phase 1
20 Antineoplastic Agents, Hormonal Phase 1
21 Anesthetics

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 Placebo-Controlled, Randomized, Double-Blind Trial of Prophylactic Desmopressin in Heart Valve Surgery Active, not recruiting NCT03343418 Phase 4 Desmopressin;Placebo
3 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 Recruiting NCT03613584 Phase 2 Von Willebrand Factor;Saline Solution
4 Ruxolitinib Versus Best Available Therapy in Patients With High-risk Polycythemia Vera or High-risk Essential Thrombocythemia - The Ruxo-BEAT Trial Recruiting NCT02577926 Phase 2 Ruxolitinib;BAT
5 Safety and Efficacy of Octreotide LAR Depot in Left Ventricular Assist Device (LVAD) Associate Gastrointestinal (GI) Completed NCT01707225 Phase 1 Octreotide LAR Depot
6 Does an Acquired Von Willebrand Syndrome Influence Perioperative Blood Loss in Patients With Severe Aortic Stenosis Undergoing Aortic Valve Replacement? Completed NCT00805051
7 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
8 Von Willebrand Antigen and Activity as Novel Biomarkers of Hemostasis in Inflammatory Bowel Disease Not yet recruiting NCT03715673
9 Perioperative Modification of Hemostasis During Ventricular Assist Device Implantation Not yet recruiting NCT03966313
10 Treatment of Ex-vivo Small Bowel Mucosa With a Dedicated Radiofrequency Ablation (SB-RFA) Catheter. Withdrawn NCT03320395

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, Thyroid, Testes, Breast, Myeloid, Colon, Kidney

Publications for Acquired Von Willebrand Syndrome

Articles related to Acquired Von Willebrand Syndrome:

(show top 50) (show all 497)
# Title Authors PMID Year
1
Turbulent Flow Promotes Cleavage of VWF (von Willebrand Factor) by ADAMTS13 (A Disintegrin and Metalloproteinase With a Thrombospondin Type-1 Motif, Member 13). 38
31291760 2019
2
Acquired von Willebrand factor deficiency is reduced in HeartMate 3 patients†. 38
30815698 2019
3
The Predicament of Gastrointestinal Bleeding in Patients With a Continuous-Flow Left Ventricular Assist Device: Pathophysiology, Evaluation, and Management. 38
30365405 2019
4
Factors defining occurrence of ischemic and hemorrhagic strokes during continuous flow left ventricular assist device support. 38
31435873 2019
5
Benchtop von Willebrand Factor Testing: Comparison of Commercially Available Ventricular Assist Devices and Evaluation of Variables for a Standardized Test Method. 38
30004942 2019
6
Reversal of acquired von Willebrand syndrome with allogeneic stem cell transplant for chronic lymphocytic leukemia. 38
31029024 2019
7
Acquired Von Willebrand Syndrome (AVWS) in cardiovascular disease: a state of the art review for clinicians. 38
31004311 2019
8
Acquired von Willebrand syndrome in a patient with small lymphocytic lymphoma and Sjögren's syndrome: which associated condition should be prioritized? 38
31157683 2019
9
Varying test results: A clue to pediatric acquired von Willebrand syndrome. 38
30723987 2019
10
Acquired von Willebrand Syndrome in Pediatric Extracorporeal Membrane Oxygenation Patients: A Single Institution's Experience. 38
31246745 2019
11
Acquired von Willebrand syndrome in paediatric patients during mechanical circulatory support. 38
30590475 2019
12
Diagnosis and immediate treatment of acquired von Willebrand syndrome revealed by recurrent cerebral hemorrhage. 38
31262628 2019
13
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. 38
31177280 2019
14
Generation and Application of Rat Monoclonal Antibodies Specific for a Human Blood Coagulation Protein: von Willebrand Factor. 38
31192778 2019
15
[Blood Coagulation Disorders During ECMO and LVAD Therapy]. 38
31212331 2019
16
Valproic Acid-Induced Coagulopathy. 38
31201069 2019
17
Remission of acquired von Willebrand syndrome in a patient with chronic lymphocytic leukemia treated with venetoclax. 38
31106622 2019
18
Acquired von Willebrand Syndrome Associated with Cardiovascular Diseases. 38
30867356 2019
19
Fulminant Essential Thrombocythemia Associated with Acquired Von Willebrand Syndrome and Bleeding Episodes in a 14-year-old Girl. 38
30895567 2019
20
Evaluation of the Utility of von Willebrand Factor Propeptide in the Differential Diagnosis of von Willebrand Disease and Acquired von Willebrand Syndrome. 38
29913537 2019
21
Stress and Exposure Time on von Willebrand Factor Degradation. 38
30374981 2019
22
Gastrointestinal Bleeding During Continuous-Flow Left Ventricular Assist Device Support: State of the Field. 38
30520778 2019
23
Acquired von Willebrand syndrome in patients treated with veno-arterial extracorporeal membrane oxygenation. 38
30656612 2019
24
Acquired von Willebrand Syndrome and Chronic Anaemia: A Different Diagnostic Approach. 38
31293998 2019
25
Acquired von Willebrand Syndrome in Patients With Ventricular Assist Device. 38
30805339 2019
26
Intravenous immunoglobulin in patients with acquired Von Willebrand syndrome: A single referral centre experience. 38
30461133 2019
27
Severe acquired von Willebrand syndrome secondary to systemic lupus erythematosus. 38
30408840 2019
28
Coexistence of Hermansky-Pudlak syndrome and JAK2V617F-positive essential thrombocythemia. 38
30932722 2019
29
Bleeding Disorders Associated with Cancer. 38
31317489 2019
30
Acquired Von Willebrand Syndrome and Blood Pump Design. 38
30370640 2018
31
Management of acquired von Willebrand syndrome. 38
30401518 2018
32
Combined Coagulopathy Can Induce Both Hemorrhagic and Thrombotic Complications in Multiple Myeloma. 38
29984746 2018
33
Acquired von Willebrand syndrome in congenital heart disease surgery: results from an observational case-series. 38
29908036 2018
34
Association between the severity of acquired von Willebrand syndrome and gastrointestinal bleeding after continuous-flow left ventricular assist device implantation. 38
29741685 2018
35
Diagnosis and Management of Acquired von Willebrand Disease in Heart Disease: A Review of the Literature. 38
30458570 2018
36
Adult and pediatric mechanical circulation: a guide for the hematologist. 38
30504351 2018
37
Changes in Von Willebrand factor profile predicts clinical outcomes in patients on mechanical circulatory support. 38
30199919 2018
38
Evaluation of a semi-automated von Willebrand factor multimer assay, the Hydragel 5 von Willebrand multimer, by two European Centers. 38
30349898 2018
39
Differences in von Willebrand factor function in type 2A von Willebrand disease and left ventricular assist device-induced acquired von Willebrand syndrome. 38
30397685 2018
40
The course of acquired von Willebrand syndrome during pregnancy among patients with essential thrombocytosis. 38
29654448 2018
41
Idiopathic pulmonary arterial hypertension - a unrecognized cause of high-shear high-flow haemostatic defects (otherwise referred to as acquired von Willebrand syndrome) in children. 38
30141279 2018
42
Hemostasis and Thrombosis in the Oldest Old. 38
29920621 2018
43
Administration of plasma-derived coagulation factor VIII during the perioperative period of mastectomy for breast cancer with acquired von Willebrand syndrome. 38
30225530 2018
44
Speed Reduction Does Not Restore High Molecular Weight von Willebrand Multimers During HeartMate II Support: An In Vivo Study. 38
29863626 2018
45
Management of epistaxis in patients with ventricular assist device: a retrospective review. 38
30068378 2018
46
Acquired von Willebrand syndrome during systemic mastocytosis: an analysis of 21 cases. 38
30057374 2018
47
Acquired von Willebrand syndrome and impaired platelet function during venovenous extracorporeal membrane oxygenation: Rapid onset and fast recovery. 38
29650295 2018
48
Waldenstrom macroglobulinaemia manifesting as acquired von Willebrand syndrome. 38
29984508 2018
49
Gastrointestinal Bleeding in Left Ventricular Assist Device: Octreotide and Other Treatment Modalities. 38
29406356 2018
50
von Willebrand factor regulation of blood vessel formation. 38
29866817 2018

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 VWF JAK2 GP1BA F9 F8 F11
2
Show member pathways
12.31 F9 F8 F11
3
Show member pathways
11.61 VWF JAK2
4 11.6 VWF F9 F8 F11
5 11.55 VWF GP1BA
6
Show member pathways
11.42 VWF GP1BA
7
Show member pathways
11.22 VWF GP1BA F9 F8 F11
8 11.05 JAK2 F11
9 10.44 VWF GP1BA
10 10.26 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 space GO:0005615 9.26 GP1BA F9 F8 F11
2 platelet alpha granule lumen GO:0031093 8.62 VWF F8

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 VWF GP1BA F9 F8 F11
2 platelet activation GO:0030168 9.43 VWF GP1BA F8
3 blood coagulation GO:0007596 9.43 VWF JAK2 GP1BA F9 F8 F11
4 platelet degranulation GO:0002576 9.37 VWF F8
5 regulation of blood coagulation GO:0030193 9.32 GP1BA F11
6 blood coagulation, intrinsic pathway GO:0007597 9.02 VWF GP1BA F9 F8 F11

Sources for Acquired Von Willebrand Syndrome

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
28 GO
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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 TGDB
71 Tocris
72 UMLS
73 UMLS via Orphanet
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