AVWS
MCID: ACQ017
MIFTS: 49

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 52 58 15
Acquired Von Willebrand Disease 52 58
Willebrand Disease, Acquired 52
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

NIH Rare Diseases : 52 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 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 Hemostatics and Coagulants have been mentioned in the context of this disorder. Affiliated tissues include heart, thyroid and myeloid, and related phenotypes are reduced factor viii activity and reduced von willebrand factor activity

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 254)
# Related Disease Score Top Affiliating Genes
1 von willebrand's disease 30.9 VWF GP1BA F9 F8 F3 F11
2 al amyloidosis 30.7 VWF ADAMTS13
3 myeloproliferative neoplasm 30.6 VWF U2AF1 JAK2
4 hemophilia 30.4 F9 F8 F7
5 thrombasthenia 30.0 GP1BA F3
6 hypothyroidism 30.0 VWF F9 F8 F3
7 thrombocytopenic purpura, autoimmune 29.9 GP1BA F8 ADAMTS13
8 deficiency anemia 29.9 U2AF1 JAK2 GP1BA ADAMTS13
9 active peptic ulcer disease 29.9 VWF F7 F3
10 thrombocytosis 29.9 VWF U2AF1 JAK2 F3
11 mitral valve stenosis 29.8 VWF F3
12 portal vein thrombosis 29.8 JAK2 F7
13 budd-chiari syndrome 29.6 JAK2 F7 F3
14 essential thrombocythemia 29.5 VWF U2AF1 JAK2 GP1BA F3
15 hemolytic anemia 29.3 VWF F3 ADAMTS13
16 angiodysplasia 29.2 VWF F8 F3 ATAT1 ADAMTS13
17 acquired hemophilia 29.2 F9 F8 F3 F11
18 acquired hemophilia a 29.2 F9 F8 F3 F11
19 portal hypertension 29.1 VWF JAK2 F3 ADAMTS13
20 hemophilia a 29.1 VWF F9 F8 F7 F3
21 purpura 29.0 VWF GP1BA F3 ADAMTS13
22 glanzmann thrombasthenia 28.9 VWF GP1BA F9 F8 F3
23 von willebrand disease, type 1 28.9 VWF GP1BA F8 F3 ATAT1
24 factor xiii deficiency 28.7 VWF F9 F8 F7 F3
25 factor viii deficiency 28.6 VWF F9 F8 F7 F3 F11
26 thrombocytopenia 28.1 VWF U2AF1 JAK2 GP1BA F9 F3
27 hemorrhagic disease 27.3 VWF GP1BA F9 F8 F7 F3
28 rare hemorrhagic disorder 10.7
29 monoclonal gammopathy of uncertain significance 10.6
30 polycythemia vera 10.5
31 polycythemia 10.5
32 autoimmune disease 10.4
33 myeloma, multiple 10.4
34 fainting 10.4 VWF F8
35 pediatric angiosarcoma 10.4 VWF F8
36 breast hemangioma 10.4 VWF F8
37 systemic lupus erythematosus 10.4
38 aortic valve disease 2 10.4
39 lupus erythematosus 10.4
40 lymphangiosarcoma 10.4 VWF F8
41 thyroid angiosarcoma 10.4 VWF F8
42 atrial standstill 1 10.3
43 wilms tumor 5 10.3
44 heart disease 10.3
45 macroglobulinemia 10.3
46 sneddon syndrome 10.3 F9 F8
47 cardiovascular cancer 10.3 VWF F8
48 thrombotic microangiopathy 10.2 VWF ADAMTS13
49 leukemia, chronic lymphocytic 10.2
50 thrombophilia due to thrombin defect 10.2

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 reduced factor viii activity 58 31 frequent (33%) Frequent (79-30%) HP:0003125
2 reduced von willebrand factor activity 58 31 frequent (33%) Frequent (79-30%) HP:0008330
3 abnormality of cardiovascular system morphology 58 31 frequent (33%) Frequent (79-30%) HP:0030680
4 hematological neoplasm 58 31 frequent (33%) Frequent (79-30%) HP:0004377
5 prolonged prothrombin time 58 31 frequent (33%) Frequent (79-30%) HP:0008151
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 hematuria 58 31 occasional (7.5%) Occasional (29-5%) HP:0000790
9 gastrointestinal angiodysplasia 58 31 occasional (7.5%) Occasional (29-5%) HP:0000471
10 mitral regurgitation 58 31 occasional (7.5%) Occasional (29-5%) HP:0001653
11 hypochromic anemia 58 31 occasional (7.5%) Occasional (29-5%) HP:0001931
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 pulmonic stenosis 58 31 occasional (7.5%) Occasional (29-5%) HP:0001642
15 persistent bleeding after trauma 58 31 occasional (7.5%) Occasional (29-5%) HP:0001934
16 joint hemorrhage 58 31 occasional (7.5%) Occasional (29-5%) HP:0005261
17 menorrhagia 58 31 occasional (7.5%) Occasional (29-5%) HP:0000132
18 metrorrhagia 58 31 occasional (7.5%) Occasional (29-5%) HP:0100608
19 aortic valve stenosis 58 31 occasional (7.5%) Occasional (29-5%) HP:0001650
20 aortic regurgitation 58 31 occasional (7.5%) Occasional (29-5%) HP:0001659
21 normocytic anemia 58 31 occasional (7.5%) Occasional (29-5%) HP:0001897
22 asthenia 58 31 occasional (7.5%) Occasional (29-5%) HP:0025406
23 refractory anemia 58 31 occasional (7.5%) Occasional (29-5%) HP:0005505
24 melena 58 31 occasional (7.5%) Occasional (29-5%) HP:0002249
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:

45
# Description MGI Source Accession Score Top Affiliating Genes
1 hematopoietic system MP:0005397 9.81 ADAMTS13 ATAT1 F11 F3 F8 F9
2 homeostasis/metabolism MP:0005376 9.61 ADAMTS13 F11 F3 F7 F8 F9
3 mortality/aging MP:0010768 9.28 ADAMTS13 ATAT1 F11 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 20)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1 Hemostatics Phase 4
2 Coagulants Phase 4
3 Protamines Phase 4
4 Arginine Vasopressin Phase 4
5 Deamino Arginine Vasopressin Phase 4
6 Natriuretic Agents Phase 4
7 Vasopressins Phase 4
8
Arginine Investigational, Nutraceutical Phase 4 74-79-3 6322
9
Hydroxyurea Approved Phase 2 127-07-1 3657
10
Anagrelide Approved Phase 2 68475-42-3 2182
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 Adjuvants, Immunologic Phase 2
15 interferons Phase 2
16
Octreotide Approved, Investigational Phase 1 83150-76-9 383414 6400441
17 Gastrointestinal Agents Phase 1
18 Hormones Phase 1
19 Antineoplastic Agents, Hormonal Phase 1
20 Anesthetics

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 Placebo-Controlled, Randomized, Double-Blind Trial of Prophylactic Desmopressin in Heart Valve Surgery Unknown status NCT03343418 Phase 4 Desmopressin;Placebo
2 Severe Aortic Stenosis and Acquired Von Willebrand´s Disease: The Impact of Desmopressin in Valve-Replacement Surgery Completed NCT01994330 Phase 4 desmopressin
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:

40
Heart, Thyroid, Myeloid, Breast, Kidney, T Cells, B Cells

Publications for Acquired Von Willebrand Syndrome

Articles related to Acquired Von Willebrand Syndrome:

(show top 50) (show all 519)
# Title Authors PMID Year
1
Hemostatic Management of Extracorporeal Circuits Including Cardiopulmonary Bypass and Extracorporeal Membrane Oxygenation. 61
31858515 2020
2
Reduced Activity of von Willebrand Factor after Flow-Diverting Stent Implantation for Intracranial Aneurysms: A Link to Acquired von Willebrand Disease? 61
31896567 2020
3
Disease progression and defects in primary hemostasis as major cause of bleeding in multiple myeloma. 61
31541609 2020
4
Acquired Von Willebrand Syndrome Secondary to Langerhans Cell Histiocytosis. 61
31900848 2020
5
Acquired von Willebrand syndrome and left ventricular assist devices. 61
31669166 2020
6
Reply to Geisen et al, Acquired von Willebrand syndrome and left ventricular assist devices. 61
31668522 2020
7
Review of von Willebrand Disease and Acquired von Willebrand Syndrome for Patients Undergoing Cardiac Surgery. 61
31570241 2019
8
Acquired von Willebrand syndrome in patients on long-term left ventricular assist device support: Results of a Belgian center. 61
31710861 2019
9
From Budd-Chiari syndrome to acquired von Willebrand syndrome: thrombosis and bleeding complications in the myeloproliferative neoplasms. 61
31808903 2019
10
Features of Marfan syndrome not listed in the Ghent nosology - the dark side of the disease. 61
31829751 2019
11
Characteristics of Acquired Inhibitors to Factor VIII and Von Willebrand Factor Secondary to Systemic Lupus Erythematosus: Experiences From a Chinese Tertiary Medical Center. 61
31815811 2019
12
Management of rare acquired bleeding disorders. 61
31808848 2019
13
Remission of acquired von Willebrand syndrome in a patient with chronic lymphocytic leukemia treated with venetoclax. 61
31106622 2019
14
Bleeding in critical care associated with left ventricular assist devices: pathophysiology, symptoms, and management. 61
31808855 2019
15
Patients with aortic stenosis have von Willebrand factor abnormalities and increased proliferation of endothelial colony forming cells. 61
31860769 2019
16
From Budd-Chiari syndrome to acquired von Willebrand syndrome: thrombosis and bleeding complications in the myeloproliferative neoplasms. 61
31778549 2019
17
Fulminant Essential Thrombocythemia Associated with Acquired Von Willebrand Syndrome and Bleeding Episodes in a 14-year-old Girl. 61
30895567 2019
18
A Prolonged Treatment Response in Acquired Von Willebrand Syndrome. 61
31546261 2019
19
Diagnosis and immediate treatment of acquired von Willebrand syndrome revealed by recurrent cerebral hemorrhage. 61
31262628 2019
20
Acquired von Willebrand syndrome in patients treated with veno-arterial extracorporeal membrane oxygenation. 61
30656612 2019
21
Acquired von Willebrand Syndrome in Pediatric Extracorporeal Membrane Oxygenation Patients: A Single Institution's Experience. 61
31246745 2019
22
Bleeding signs due to acquired von Willebrand syndrome at diagnosis of chronic myeloid leukaemia in children. 61
31617211 2019
23
Rituximab to the rescue: novel therapy for chronic gastrointestinal bleeding due to angiodysplasia and acquired von Willebrand syndrome. 61
31656571 2019
24
Acquired von Willebrand Syndrome in Pediatric Extracorporeal Membrane Oxygenation: Sheared and Cleaved Everywhere, but What to Do About It? 61
31580279 2019
25
Hemostatic complications associated with ventricular assist devices. 61
31624778 2019
26
Valproic Acid-Induced Coagulopathy. 61
31201069 2019
27
The Predicament of Gastrointestinal Bleeding in Patients With a Continuous-Flow Left Ventricular Assist Device: Pathophysiology, Evaluation, and Management. 61
30365405 2019
28
Acquired von Willebrand factor deficiency is reduced in HeartMate 3 patients†. 61
30815698 2019
29
Turbulent Flow Promotes Cleavage of VWF (von Willebrand Factor) by ADAMTS13 (A Disintegrin and Metalloproteinase With a Thrombospondin Type-1 Motif, Member 13). 61
31291760 2019
30
Factors defining occurrence of ischemic and hemorrhagic strokes during continuous flow left ventricular assist device support. 61
31435873 2019
31
Benchtop von Willebrand Factor Testing: Comparison of Commercially Available Ventricular Assist Devices and Evaluation of Variables for a Standardized Test Method. 61
30004942 2019
32
Acquired von Willebrand syndrome in a patient with small lymphocytic lymphoma and Sjögren's syndrome: which associated condition should be prioritized? 61
31157683 2019
33
Acquired Von Willebrand Syndrome (AVWS) in cardiovascular disease: a state of the art review for clinicians. 61
31004311 2019
34
Reversal of acquired von Willebrand syndrome with allogeneic stem cell transplant for chronic lymphocytic leukemia. 61
31029024 2019
35
Varying test results: A clue to pediatric acquired von Willebrand syndrome. 61
30723987 2019
36
Acquired von Willebrand syndrome in paediatric patients during mechanical circulatory support. 61
30590475 2019
37
Generation and Application of Rat Monoclonal Antibodies Specific for a Human Blood Coagulation Protein: von Willebrand Factor. 61
31192778 2019
38
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 2019
39
[Blood Coagulation Disorders During ECMO and LVAD Therapy]. 61
31212331 2019
40
Acquired von Willebrand Syndrome Associated with Cardiovascular Diseases. 61
30867356 2019
41
Evaluation of the Utility of von Willebrand Factor Propeptide in the Differential Diagnosis of von Willebrand Disease and Acquired von Willebrand Syndrome. 61
29913537 2019
42
Stress and Exposure Time on von Willebrand Factor Degradation. 61
30374981 2019
43
Gastrointestinal Bleeding During Continuous-Flow Left Ventricular Assist Device Support: State of the Field. 61
30520778 2019
44
Acquired von Willebrand Syndrome in Patients With Ventricular Assist Device. 61
30805339 2019
45
Intravenous immunoglobulin in patients with acquired Von Willebrand syndrome: A single referral centre experience. 61
30461133 2019
46
Two Rare Complications in One Patient: Acquired von Willebrand Syndrome Associated with Intracranial Plasmacytoma. 61
31534805 2019
47
Acquired von Willebrand Syndrome and Chronic Anaemia: A Different Diagnostic Approach. 61
31293998 2019
48
Severe acquired von Willebrand syndrome secondary to systemic lupus erythematosus. 61
30408840 2019
49
Coexistence of Hermansky-Pudlak syndrome and JAK2V617F-positive essential thrombocythemia. 61
30932722 2019
50
Coincidental Central Precocious Puberty and Wilms Tumor in a 5-Year-Old Girl. 61
31583153 2019

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

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 F9 F8 F7 F3 F11
2 collagen-containing extracellular matrix GO:0062023 9.56 VWF F9 F7 F3
3 extracellular space GO:0005615 9.5 GP1BA F9 F8 F7 F3 F11
4 endoplasmic reticulum lumen GO:0005788 9.46 F9 F8 F7 ADAMTS13
5 serine-type peptidase complex GO:1905286 8.62 F7 F3

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

(show all 13)
# Name GO ID Score Top Affiliating Genes
1 proteolysis GO:0006508 9.81 F9 F7 F11 ADAMTS13
2 positive regulation of cell migration GO:0030335 9.7 JAK2 F7 F3
3 ER to Golgi vesicle-mediated transport GO:0006888 9.65 F9 F8 F7
4 protein processing GO:0016485 9.58 F7 F3 ADAMTS13
5 platelet activation GO:0030168 9.56 VWF GP1BA F8 ADAMTS13
6 hemostasis GO:0007599 9.56 VWF GP1BA F9 F8 F7 F3
7 blood coagulation, intrinsic pathway GO:0007597 9.55 VWF GP1BA F9 F8 F11
8 response to tumor necrosis factor GO:0034612 9.49 JAK2 ADAMTS13
9 positive regulation of positive chemotaxis GO:0050927 9.46 F7 F3
10 regulation of blood coagulation GO:0030193 9.43 GP1BA F11
11 positive regulation of platelet-derived growth factor receptor signaling pathway GO:0010641 9.37 F7 F3
12 blood coagulation, extrinsic pathway GO:0007598 9.32 F7 F3
13 blood coagulation GO:0007596 9.28 VWF JAK2 GP1BA F9 F8 F7

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

# Name GO ID Score Top Affiliating Genes
1 peptidase activity GO:0008233 9.46 F9 F7 F11 ADAMTS13
2 serine-type peptidase activity GO:0008236 9.13 F9 F7 F11
3 serine-type endopeptidase activity GO:0004252 8.92 F9 F7 F3 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
29 GTR
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 LOVD
41 MedGen
43 MeSH
44 MESH via Orphanet
45 MGI
48 NCI
49 NCIt
50 NDF-RT
53 NINDS
54 Novoseek
56 OMIM
57 OMIM via Orphanet
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 TGDB
70 Tocris
71 UMLS
72 UMLS via Orphanet
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