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:
30ICD10 via Orphanet, 47NIH Rare Diseases, 53Orphanet, 68UMLS via Orphanet
See all MalaCards sources

Aliases & Descriptions for Acquired Von Willebrand Syndrome:

Name: Acquired Von Willebrand Syndrome 47 53
Acquired Von Willebrand Disease 47 53
 
Willebrand Disease, Acquired 47

Characteristics:

Orphanet epidemiological data:

53
acquired von willebrand syndrome:
Inheritance: Not applicable; Age of onset: Adult

Classifications:



External Ids:

Orphanet53 ORPHA99147
UMLS via Orphanet68 C0272362
ICD10 via Orphanet30 D68.4

Summaries for Acquired Von Willebrand Syndrome

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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 for Acquired Von Willebrand Syndrome

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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:

35
Heart, Prostate, Thyroid, Skin

Animal Models for Acquired Von Willebrand Syndrome or affiliated genes

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MGI Mouse Phenotypes related to Acquired Von Willebrand Syndrome:

40
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

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. (28028990)
2016
2
Acquired von Willebrand Syndrome: An Under-Recognized Cause of Major Bleeding in the Cardiac Intensive Care Unit. (27834763)
2016
3
Severe Aortic Valve Stenosis: Sustained Cure of Acquired von Willebrand Syndrome After Surgical Valve Replacement. (27481874)
2016
4
Distinct mechanisms account for acquired von Willebrand syndrome in plasma cell dyscrasias. (27040683)
2016
5
Acquired von Willebrand syndrome in children with aortic and pulmonary stenosis. (27841910)
2016
6
Impact of Acquired von Willebrand Syndrome in Severe Aortic Stenosis. (26370453)
2015
7
Unexpectedly High Prevalence of Acquired von Willebrand Syndrome in Patients with Severe Aortic Stenosis as Evaluated with a Novel Large Multimer Index. (26269004)
2015
8
Acquired von Willebrand Syndrome (AVWS) as an Important Diagnostic Category of Disease. (25740338)
2015
9
Autophagy: A potential link between Acquired von Willebrand syndrome and congenital heart disease. (25804464)
2015
10
Acquired Von Willebrand Syndrome In Aortic Stenosis: Case Report And Review. (26434092)
2015
11
Bleeding diathesis associated with acquired von Willebrand Syndrome in three patients with chronic lymphocytic leukemia. (25926060)
2015
12
Continuous-flow left ventricular assist devices as a cause of acquired von Willebrand syndrome and GI bleeding. (25708771)
2015
13
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
14
Shear-stress induced acquired von Willebrand syndrome in children with congenital heart disease. (25228244)
2014
15
Acquired von Willebrand syndrome: von Willebrand factor propeptide to von Willebrand factor antigen ratio predicts remission status. (24951428)
2014
16
Acquired Von Willebrand syndrome secondary to mitral and aortic regurgitation. (24994586)
2014
17
Acquired von Willebrand Syndrome in a Child Following Berlin Heart EXCOR Pediatric Ventricular Assist Device Implantation: Case Report and Concise Literature Review. (25324261)
2014
18
Acquired von Willebrand syndrome in adult patients with congenital heart disease. (25139318)
2014
19
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
20
Current diagnostic and therapeutic approaches to patients with acquired von Willebrand syndrome: a 2013 update. (23397553)
2013
21
Rituximab effectiveness in a patient with juvenile systemic lupus erythematosus complicated with acquired Von Willebrand syndrome. (23989733)
2013
22
Maintaining hemostasis in acquired von Willebrand syndrome: a review of intravenous immunoglobulin and the importance of rituximab dose scheduling. (23252513)
2013
23
Haemostatic management of extreme challenges to haemostasis in acquired von Willebrand syndrome. (22356103)
2012
24
Acquired Von Willebrand syndrome is an early-onset problem in ventricular assist device patients. (21515067)
2011
25
Hypertrophic obstructive cardiomyopathy, bleeding history, and acquired von Willebrand syndrome: response to septal myectomy. (21364113)
2011
26
Acquired von Willebrand syndrome and mitral valve prosthesis leakage. A pilot study. (21668503)
2011
27
Acquired von Willebrand syndrome in continuous-flow ventricular assist device recipients. (20868825)
2010
28
Bortezomib effectiveness in one patient with acquired von Willebrand syndrome associated to monoclonal gammopathy of undetermined significance. (20425807)
2010
29
Polycythemia vera-associated acquired von Willebrand syndrome despite near-normal platelet count. (20575028)
2010
30
Essential thrombocythaemia in a teenage girl resulting in acquired von Willebrand syndrome with joint haemorrhage and menorrhagia. (20352170)
2010
31
Advances in hematology. Etiology and diagnosis of acquired von Willebrand syndrome. (20351678)
2010
32
Acquired von Willebrand syndrome after exchange of the HeartMate XVE to the HeartMate II ventricular assist device. (19303790)
2009
33
Is rituximab effective in acquired von Willebrand syndrome? (19120364)
2009
34
Acquired von Willebrand syndrome and Wilms tumor: not always benign. (19006222)
2009
35
Successful imatinib mesylate therapy for acquired von Willebrand syndrome in chronic myelogenous leukemia. (19580999)
2009
36
Transient neonatal acquired von Willebrand syndrome due to transplacental transfer of maternal monoclonal antibodies. (19459202)
2009
37
Acquired von Willebrand syndrome. (18445010)
2008
38
Association of acquired von Willebrand syndrome with AL amyloidosis. (17205535)
2007
39
Cefotaxime as the potential cause of transient acquired von Willebrand syndrome. (16529605)
2006
40
Immune-mediated etiology of acquired von Willebrand syndrome in systemic lupus erythematosus and in benign monoclonal gammopathy: therapeutic implications. (16977568)
2006
41
Acquired von Willebrand syndrome 2004: International Registry--diagnosis and management from online to bedside. (15029273)
2004
42
Acquired von Willebrand syndrome with autoimmune hemolytic anemia]. (15103936)
2004
43
Acquired von Willebrand syndrome: its pathophysiology, laboratory features and management. (14739623)
2003
44
Lack of multimer organization of von Willebrand factor in an acquired von Willebrand syndrome. (11886398)
2002
45
Acquired von Willebrand syndromes: clinical features, aetiology, pathophysiology, classification and management. (11686107)
2001
46
Acquired von Willebrand syndrome type 1 in hypothyroidism: reversal after treatment with thyroxine. (11292187)
2001
47
Pharmacokinetic studies with FVIII/von Willebrand factor concentrate can be a diagnostic tool to distinguish between subgroups of patients with acquired von Willebrand syndrome. (11372672)
2001
48
Treatment of acquired von Willebrand syndrome with intravenous immunoglobulin. (7878645)
1994
49
The use of high-dose intravenous gamma-globulin in acquired von Willebrand syndrome. (2447856)
1988
50
Comparison of inhibitory and binding characteristics of an antibody causing acquired von Willebrand syndrome: an assay for von Willebrand factor binding by antibody. (3927998)
1985

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:003109310.2F8, VWF
2extracellular regionGO:00055768.5F11, F8, F9, VWF

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

(show all 7)
idNameGO IDScoreTop Affiliating Genes
1platelet degranulationGO:000257610.0F8, VWF
2hemostasisGO:00075999.9GP1BA, VWF
3regulation of blood coagulationGO:00301939.8F11, GP1BA
4cytokine-mediated signaling pathwayGO:00192219.4GP1BA, JAK2
5platelet activationGO:00301689.2F8, GP1BA, VWF
6blood coagulation, intrinsic pathwayGO:00075978.4F11, F8, F9, GP1BA, VWF
7blood coagulationGO:00075967.1F11, 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
26GTR
27HGMD
28HMDB
29ICD10
30ICD10 via Orphanet
31ICD9CM
32IUPHAR
33KEGG
36MedGen
38MeSH
39MESH via Orphanet
40MGI
43NCI
44NCIt
45NDF-RT
48NINDS
49Novoseek
51OMIM
52OMIM via Orphanet
56PubMed
57QIAGEN
62SNOMED-CT via Orphanet
66Tumor Gene Family of Databases
67UMLS
68UMLS via Orphanet