MCID: ACQ017
MIFTS: 36

Acquired Von Willebrand Syndrome malady

Categories: Rare diseases, Blood diseases

Aliases & Classifications for Acquired Von Willebrand Syndrome

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Sources:
31ICD10 via Orphanet, 48NIH Rare Diseases, 54Orphanet, 69UMLS via Orphanet
See all MalaCards sources

Aliases & Descriptions for Acquired Von Willebrand Syndrome:

Name: Acquired Von Willebrand Syndrome 48 54
Acquired Von Willebrand Disease 48 54
 
Willebrand Disease, Acquired 48

Characteristics:

Orphanet epidemiological data:

54
acquired von willebrand syndrome:
Inheritance: Not applicable; Age of onset: Adult

Classifications:



External Ids:

Orphanet54 ORPHA99147
UMLS via Orphanet69 C0272362
ICD10 via Orphanet31 D68.4

Summaries for Acquired Von Willebrand Syndrome

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NIH Rare Diseases:48 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. Last updated: 9/15/2011

MalaCards based summary: Acquired Von Willebrand Syndrome, also known as acquired von willebrand disease, is related to tyrobp-related polycystic lipomembranous osteodysplasia with sclerosing leukoencephalopathy and tyrosine hydroxylase-deficient dopa-responsive dystonia. An important gene associated with Acquired Von Willebrand Syndrome is VWF (Von Willebrand Factor), and among its related pathways are Platelet activation and Integrin alphaIIb beta3 signaling. Affiliated tissues include heart, prostate and thyroid, and related mouse phenotypes are hematopoietic system and homeostasis/metabolism.

Related Diseases for Acquired Von Willebrand Syndrome

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

(show top 50)    (show all 65)
idRelated DiseaseScoreTop Affiliating Genes
1tyrobp-related polycystic lipomembranous osteodysplasia with sclerosing leukoencephalopathy10.3F8, VWF
2tyrosine hydroxylase-deficient dopa-responsive dystonia10.3F8, VWF
3secondary central precocious puberty10.2F8, VWF
4familial spastic paralysis10.2F8, VWF
5malignant type ab thymoma10.2F8, VWF
6von willibrand disease, type 310.2F8, VWF
7tympanosclerosis10.2F8, VWF
8trismus-pseudocamptodactyly syndrome10.2GP1BA, VWF
9high anorectal malformation10.2F8, F9
10heart disease10.1
11penis papillary carcinoma10.1F8, F9
12leydig cell tumor10.1F8, VWF
13diabetic autonomic neuropathy10.1F8, F9
14hypothyroidism10.1
15polycythemia10.1
16cardiomyopathy10.1
17van maldergem syndrome 210.1F8, VWF
18atrophic gastritis10.1GP1BA, VWF
19systemic lupus erythematosus10.0
20multiple myeloma10.0
21lymphoma10.0
22essential thrombocythemia10.0
23lupus erythematosus10.0
24prostatic hypertrophy10.0F11, F8
25hemolytic anemia9.9
26leukemia9.9
27prostatitis9.9
28angiodysplasia9.9
29autoimmune hemolytic anemia9.9
30transient cerebral ischemia9.9F11, F9
31bernard-soulier syndrome, type c9.9GP1BA, VWF
32littre gland carcinoma9.9GP1BA, VWF
33pregnancy loss, recurrent 29.9F8, VWF
34type 2n von willebrand disease9.9F8, GP1BA, VWF
35wfs1-related disorders9.9F8, GP1BA, VWF
36infantile-onset ascending hereditary spastic paralysis9.9F8, F9, VWF
37otopalatodigital syndrome, type i9.8F8, F9, VWF
38thrombophilia due to activated protein c resistance9.8F8, F9, VWF
39factor v deficiency9.8F8, F9, VWF
40ewing sarcoma9.8
41chronic lymphocytic leukemia9.8
42myelofibrosis9.8
43sarcoma9.8
44pancytopenia9.8
45patent ductus arteriosus9.8
46thrombocytosis9.8
47thyroiditis9.8
48monoclonal gammopathy of uncertain significance9.8
49amyloidosis9.8
50al amyloidosis9.8

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

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MGI Mouse Phenotypes related to Acquired Von Willebrand Syndrome according to GeneCards Suite gene sharing:

41
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00053977.9F11, F8, F9, GP1BA, JAK2, VWF
2MP:00053767.9F11, F8, F9, GP1BA, JAK2, VWF
3MP:00053877.6F11, F8, F9, JAK2, VWF

Drugs & Therapeutics for Acquired Von Willebrand Syndrome

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Drugs for Acquired Von Willebrand Syndrome (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show all 7)
idNameStatusPhaseClinical TrialsCas NumberPubChem Id
1Natriuretic AgentsPhase 41645
2VasopressinsPhase 4286
3HemostaticsPhase 41359
4Deamino Arginine VasopressinPhase 472
5Arginine VasopressinPhase 4290
6CoagulantsPhase 41428
7arginineNutraceuticalPhase 4408

Interventional clinical trials:

idNameStatusNCT IDPhase
1Severe Aortic Stenosis and Acquired Von Willebrand´s Disease: The Impact of Desmopressin in Valve-Replacement SurgeryCompletedNCT01994330Phase 4
2Acquired Von Willebrand Syndrome in Severe Aortic StenosisCompletedNCT00805051
3ADAMTS13 in Thrombotic Thrombocytopenic PurpuraCompletedNCT00426686

Search NIH Clinical Center for Acquired Von Willebrand Syndrome

Genetic Tests for Acquired Von Willebrand Syndrome

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Anatomical Context for Acquired Von Willebrand Syndrome

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MalaCards organs/tissues related to Acquired Von Willebrand Syndrome:

36
Heart, Prostate, Thyroid, Skin

Publications for Acquired Von Willebrand Syndrome

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Articles related to Acquired Von Willebrand Syndrome:

(show top 50)    (show all 153)
idTitleAuthorsYear
1
Acquired Von Willebrand syndrome in patients on long-term support with HeartMate II. (28082469)
2017
2
Pediatric Acquired von Willebrand Syndrome in Cardiopulmonary Disorders: Do Laboratory Abnormalities Predict Bleeding Risk? (28060126)
2017
3
Acquired von Willebrand Syndrome. (28028990)
2016
4
Acquired von Willebrand Syndrome: An Under-Recognized Cause of Major Bleeding in the Cardiac Intensive Care Unit. (27834763)
2016
5
Severe Aortic Valve Stenosis: Sustained Cure of Acquired von Willebrand Syndrome After Surgical Valve Replacement. (27481874)
2016
6
Distinct mechanisms account for acquired von Willebrand syndrome in plasma cell dyscrasias. (27040683)
2016
7
Acquired von Willebrand syndrome in children with aortic and pulmonary stenosis. (27841910)
2016
8
Long-term remission of acquired von Willebrand syndrome associated with multiple myeloma using bortezomib and dexamethasone therapy. (27641423)
2016
9
Intractable bleeding tendency due to acquired von Willebrand syndrome after Jarvik 2000 implant. (27021290)
2016
10
An 11-year experience of acquired von Willebrand syndrome in children diagnosed with Wilms tumour in a tertiary referral centre. (27616321)
2016
11
Factors related to the development of acquired von Willebrand syndrome in patients with essential thrombocythemia and polycythemia vera. (27919526)
2016
12
Lenalidomide as a novel treatment for refractory Acquired von Willebrand Syndrome associated with monoclonal gammopathy. (26991062)
2016
13
Diagnostic Challenges in Acquired von Willebrand Syndrome: A Complex Case of Prostate Carcinoma Associated-Acquired von Willebrand Syndrome. (27806384)
2016
14
Acquired von Willebrand syndrome associated with left ventricular assist device. (27143258)
2016
15
Impact of Acquired von Willebrand Syndrome in Severe Aortic Stenosis. (26370453)
2015
16
Unexpectedly High Prevalence of Acquired von Willebrand Syndrome in Patients with Severe Aortic Stenosis as Evaluated with a Novel Large Multimer Index. (26269004)
2015
17
Acquired von Willebrand Syndrome (AVWS) as an Important Diagnostic Category of Disease. (25740338)
2015
18
Autophagy: A potential link between Acquired von Willebrand syndrome and congenital heart disease. (25804464)
2015
19
Acquired Von Willebrand Syndrome In Aortic Stenosis: Case Report And Review. (26434092)
2015
20
Bleeding diathesis associated with acquired von Willebrand Syndrome in three patients with chronic lymphocytic leukemia. (25926060)
2015
21
Continuous-flow left ventricular assist devices as a cause of acquired von Willebrand syndrome and GI bleeding. (25708771)
2015
22
Acquired von Willebrand Syndrome During the Course of Myelofibrosis: Analysis of 32 Cases. (26771972)
2015
23
Treatment of Acquired von Willebrand Syndrome and Prevention of Bleeding Postautologous Stem Cell Transplant during Severe Pancytopenia with IVIG. (25922770)
2015
24
Thalidomide for treatment of gastrointestinal bleedings due to angiodysplasia: a case report in acquired von Willebrand syndrome and review of the literature. (25929157)
2015
25
Treatment of acquired von Willebrand syndrome in aortic stenosis with transcatheter aortic valve replacement. (25946442)
2015
26
Bleeding complications after arthroscopy in a JAK2V617F-positive patient with essential thrombocythemia and acquired von Willebrand syndrome (AVWS). (25432436)
2015
27
Acquired von Willebrand syndrome in paediatric patients with congenital heart disease: challenges in the diagnosis and management of this rare condition. (25495773)
2015
28
Factors predisposing to acquired von Willebrand syndrome during the course of polycythemia vera - retrospective analysis of 142 consecutive cases. (26269222)
2015
29
Treatment of the acquired von Willebrand syndrome. (26577336)
2015
30
Prevalence of acquired von Willebrand syndrome during essential thrombocythemia: a retrospective analysis of 170 consecutive patients. (26658493)
2015
31
Acquired von Willebrand syndrome in a case of polycythemia vera resulting in recurrent and massive bleeding events in the pleural and abdominal cavity. (25215616)
2014
32
Shear-stress induced acquired von Willebrand syndrome in children with congenital heart disease. (25228244)
2014
33
Acquired von Willebrand syndrome: von Willebrand factor propeptide to von Willebrand factor antigen ratio predicts remission status. (24951428)
2014
34
Acquired Von Willebrand syndrome secondary to mitral and aortic regurgitation. (24994586)
2014
35
Acquired von Willebrand Syndrome in a Child Following Berlin Heart EXCOR Pediatric Ventricular Assist Device Implantation: Case Report and Concise Literature Review. (25324261)
2014
36
Acquired von Willebrand syndrome in adult patients with congenital heart disease. (25139318)
2014
37
A two-step approach (Enzyme-linked immunosorbent assay and confirmation assay) to detect antibodies against von Willebrand factor in patients with Acquired von Willebrand Syndrome. (25300810)
2014
38
Shear stress-associated acquired von Willebrand syndrome in patients with mitral regurgitation. (25251907)
2014
39
Resolution of acquired von Willebrand syndrome after transcatheter aortic valve implantation through a left transcarotid approach. (25129274)
2014
40
Acquired von Willebrand syndrome in patients with a centrifugal or axial continuous flow left ventricular assist device. (24720921)
2014
41
Correction of acquired von Willebrand syndrome by transcatheter aortic valve implantation. (25480995)
2014
42
An atypical manifestation of acquired von Willebrand syndrome (AVWS) associated with systemic lupus erythematosus (SLE). (23625134)
2014
43
Development of acquired von Willebrand syndrome during short-term micro axial pump support: implications for bleeding in a patient bridged to a long-term continuous-flow left ventricular assist device. (24614358)
2014
44
Platelet dysfunction and acquired von Willebrand syndrome in patients with left ventricular assist devicesa8 (25543177)
2014
45
Early Diagnosis of Acquired von Willebrand Syndrome (AVWS) is Elementary for Clinical Practice in Patients Treated with ECMO Therapy. (25186021)
2014
46
Remission of recurrent gastrointestinal bleeding after septal reduction therapy in patients with hypertrophic obstructive cardiomyopathy-associated acquired von Willebrand syndrome. (25387993)
2014
47
Immediate Recovery of Acquired von Willebrand Syndrome after Left Ventricular Assist Device Explantation: Implications for Heart Transplantation. (25251586)
2014
48
Continuous-flow Left Ventricular Assist Device, Valvular Disease, and Acquired von Willebrand Syndrome. (25476464)
2014
49
Lymphoplasmacytic lymphoma exposed by haemoptysis and acquired von Willebrand syndrome. (24469392)
2014
50
Salvage therapy with high dose Intravenous Immunoglobulins in acquired Von Willebrand Syndrome and unresponsive severe intestinal bleeding. (24926417)
2014

Variations for Acquired Von Willebrand Syndrome

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Expression for genes affiliated with Acquired Von Willebrand Syndrome

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Search GEO for disease gene expression data for Acquired Von Willebrand Syndrome.

Pathways for genes affiliated with Acquired Von Willebrand Syndrome

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GO Terms for genes affiliated with Acquired Von Willebrand Syndrome

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Cellular components related to Acquired Von Willebrand Syndrome according to GeneCards Suite gene sharing:

idNameGO IDScoreTop Affiliating Genes
1platelet alpha granule lumenGO:00310939.6F8, VWF
2extracellular regionGO:00055768.9F11, F8, F9, VWF

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

(show all 7)
idNameGO IDScoreTop Affiliating Genes
1hemostasisGO:000759910.1GP1BA, VWF
2platelet degranulationGO:000257610.0F8, VWF
3cytokine-mediated signaling pathwayGO:00192219.7GP1BA, JAK2
4platelet activationGO:00301689.6F8, GP1BA, VWF
5regulation of blood coagulationGO:00301939.3F11, GP1BA
6blood coagulation, intrinsic pathwayGO:00075978.6F11, F8, F9, GP1BA, VWF
7blood coagulationGO:00075967.8F11, F8, F9, GP1BA, JAK2, VWF

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

idNameGO IDScoreTop Affiliating Genes
1serine-type endopeptidase activityGO:00042528.9F11, F8, F9

Sources for Acquired Von Willebrand Syndrome

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2CDC
6CNVD
10DGIdb
15ExPASy
16FDA
17FMA
27GTR
28HGMD
29HMDB
30ICD10
31ICD10 via Orphanet
32ICD9CM
33IUPHAR
34KEGG
37MedGen
39MeSH
40MESH via Orphanet
41MGI
44NCI
45NCIt
46NDF-RT
49NINDS
50Novoseek
52OMIM
53OMIM via Orphanet
57PubMed
58QIAGEN
63SNOMED-CT via Orphanet
67Tumor Gene Family of Databases
68UMLS
69UMLS via Orphanet