MCID: ACQ017
MIFTS: 35

Acquired Von Willebrand Syndrome malady

Categories: Rare diseases, Blood diseases

Aliases & Classifications for Acquired Von Willebrand Syndrome

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Sources:
46NIH Rare Diseases, 52Orphanet, 29ICD10 via Orphanet, 67UMLS via Orphanet
See all MalaCards sources

Aliases & Descriptions for Acquired Von Willebrand Syndrome:

Name: Acquired Von Willebrand Syndrome 46 52
Acquired Von Willebrand Disease 46 52
 
Willebrand Disease, Acquired 46

Characteristics:

Orphanet epidemiological data:

52
acquired von willebrand syndrome:
Inheritance: Not applicable; Age of onset: Adult

Classifications:



External Ids:

Orphanet52 ORPHA99147
ICD10 via Orphanet29 D68.4
UMLS via Orphanet67 C0272362

Summaries for Acquired Von Willebrand Syndrome

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NIH Rare Diseases:46 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 Integrin alphaIIb beta3 signaling and Platelet activation. Affiliated tissues include heart, skin and thyroid, and related mouse phenotypes are immune system and hematopoietic system.

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 69)
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 puberty10.3F8, VWF
4familial spastic paralysis10.3F8, VWF
5malignant type ab thymoma10.3F8, VWF
6von willibrand disease, type 310.3F8, VWF
7heart disease10.2
8congenital heart disease10.2
9tympanosclerosis10.2F8, VWF
10trismus-pseudocamptodactyly syndrome10.2GP1BA, VWF
11high anorectal malformation10.2F8, F9
12hypothyroidism10.2
13cardiomyopathy10.2
14leydig cell tumor10.2F8, VWF
15penis papillary carcinoma10.1F8, F9
16diabetic autonomic neuropathy10.1F8, F9
17systemic lupus erythematosus10.1
18multiple myeloma10.1
19lymphoma10.1
20myeloma10.1
21polycythemia10.1
22lupus erythematosus10.1
23van maldergem syndrome 210.1F8, VWF
24atrophic gastritis10.1GP1BA, VWF
25hemolytic anemia10.0
26leukemia10.0
27angiodysplasia10.0
28autoimmune hemolytic anemia10.0
29essential thrombocythemia10.0
30bernard-soulier syndrome, type c10.0GP1BA, VWF
31pregnancy loss, recurrent 210.0F8, VWF
32jumping frenchmen of maine9.9JAK2, VWF
33littre gland carcinoma9.9GP1BA, VWF
34ewing sarcoma9.9
35chronic lymphocytic leukemia9.9
36myelofibrosis9.9
37lymphoplasmacytic lymphoma9.9
38sarcoma9.9
39pancytopenia9.9
40melanoma9.9
41thrombocytosis9.9
42thyroiditis9.9
43monoclonal gammopathy of uncertain significance9.9
44amyloidosis9.9
45von willebrand disease9.9
46al amyloidosis9.9
47patent ductus arteriosus9.9
48type 2n von willebrand disease9.9F8, GP1BA, VWF
49wfs1-related disorders9.9F8, GP1BA, VWF
50infantile-onset ascending hereditary spastic paralysis9.9F8, F9, VWF

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):

idNameStatusPhaseClinical TrialsCas NumberPubChem Id
1arginineNutraceuticalPhase 4393

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:

34
Heart, Skin, Thyroid

Animal Models for Acquired Von Willebrand Syndrome or affiliated genes

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MGI Mouse Phenotypes related to Acquired Von Willebrand Syndrome:

39
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00053877.9F11, F8, F9, JAK2, VWF
2MP:00053977.1F11, F8, F9, GP1BA, JAK2, VWF
3MP:00053767.1F11, F8, F9, GP1BA, JAK2, VWF

Publications for Acquired Von Willebrand Syndrome

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Articles related to Acquired Von Willebrand Syndrome:

(show top 50)    (show all 146)
idTitleAuthorsYear
1
Severe Aortic Valve Stenosis: Sustained Cure of Acquired von Willebrand Syndrome After Surgical Valve Replacement. (27481874)
2016
2
Distinct mechanisms account for acquired von Willebrand syndrome in plasma cell dyscrasias. (27040683)
2016
3
Impact of Acquired von Willebrand Syndrome in Severe Aortic Stenosis. (26370453)
2015
4
Unexpectedly High Prevalence of Acquired von Willebrand Syndrome in Patients with Severe Aortic Stenosis as Evaluated with a Novel Large Multimer Index. (26269004)
2015
5
Acquired von Willebrand Syndrome (AVWS) as an Important Diagnostic Category of Disease. (25740338)
2015
6
Autophagy: A potential link between Acquired von Willebrand syndrome and congenital heart disease. (25804464)
2015
7
Acquired Von Willebrand Syndrome In Aortic Stenosis: Case Report And Review. (26434092)
2015
8
Bleeding diathesis associated with acquired von Willebrand Syndrome in three patients with chronic lymphocytic leukemia. (25926060)
2015
9
Continuous-flow left ventricular assist devices as a cause of acquired von Willebrand syndrome and GI bleeding. (25708771)
2015
10
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
11
Shear-stress induced acquired von Willebrand syndrome in children with congenital heart disease. (25228244)
2014
12
Acquired von Willebrand syndrome: von Willebrand factor propeptide to von Willebrand factor antigen ratio predicts remission status. (24951428)
2014
13
Acquired Von Willebrand syndrome secondary to mitral and aortic regurgitation. (24994586)
2014
14
Acquired von Willebrand Syndrome in a Child Following Berlin Heart EXCOR Pediatric Ventricular Assist Device Implantation: Case Report and Concise Literature Review. (25324261)
2014
15
Acquired von Willebrand syndrome in adult patients with congenital heart disease. (25139318)
2014
16
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
17
Current diagnostic and therapeutic approaches to patients with acquired von Willebrand syndrome: a 2013 update. (23397553)
2013
18
Rituximab effectiveness in a patient with juvenile systemic lupus erythematosus complicated with acquired Von Willebrand syndrome. (23989733)
2013
19
Maintaining hemostasis in acquired von Willebrand syndrome: a review of intravenous immunoglobulin and the importance of rituximab dose scheduling. (23252513)
2013
20
Haemostatic management of extreme challenges to haemostasis in acquired von Willebrand syndrome. (22356103)
2012
21
Acquired Von Willebrand syndrome is an early-onset problem in ventricular assist device patients. (21515067)
2011
22
Hypertrophic obstructive cardiomyopathy, bleeding history, and acquired von Willebrand syndrome: response to septal myectomy. (21364113)
2011
23
Acquired von Willebrand syndrome and mitral valve prosthesis leakage. A pilot study. (21668503)
2011
24
Acquired von Willebrand syndrome associated with hypothyroidism: a mild bleeding disorder to be further investigated. (21305800)
2011
25
Acquired von Willebrand syndrome in continuous-flow ventricular assist device recipients. (20868825)
2010
26
Bortezomib effectiveness in one patient with acquired von Willebrand syndrome associated to monoclonal gammopathy of undetermined significance. (20425807)
2010
27
Polycythemia vera-associated acquired von Willebrand syndrome despite near-normal platelet count. (20575028)
2010
28
Essential thrombocythaemia in a teenage girl resulting in acquired von Willebrand syndrome with joint haemorrhage and menorrhagia. (20352170)
2010
29
Advances in hematology. Etiology and diagnosis of acquired von Willebrand syndrome. (20351678)
2010
30
Acquired von Willebrand syndrome after exchange of the HeartMate XVE to the HeartMate II ventricular assist device. (19303790)
2009
31
Is rituximab effective in acquired von Willebrand syndrome? (19120364)
2009
32
Acquired von Willebrand syndrome and Wilms tumor: not always benign. (19006222)
2009
33
Successful imatinib mesylate therapy for acquired von Willebrand syndrome in chronic myelogenous leukemia. (19580999)
2009
34
Transient neonatal acquired von Willebrand syndrome due to transplacental transfer of maternal monoclonal antibodies. (19459202)
2009
35
Acquired von Willebrand syndrome. (18445010)
2008
36
Diagnostic workup of patients with acquired von Willebrand syndrome: a retrospective single-centre cohort study. (18208537)
2008
37
Association of acquired von Willebrand syndrome with AL amyloidosis. (17205535)
2007
38
Acquired von Willebrand syndrome: diagnostic problems and therapeutic options. (16986130)
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:00055768.3F11, 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:000759910.0GP1BA, VWF
2platelet degranulationGO:000257610.0F8, VWF
3regulation of blood coagulationGO:00301939.5F11, GP1BA
4cytokine-mediated signaling pathwayGO:00192219.5GP1BA, JAK2
5platelet activationGO:00301689.2F8, GP1BA, VWF
6blood coagulation, intrinsic pathwayGO:00075978.2F11, 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:00042528.7F11, F8, F9

Sources for Acquired Von Willebrand Syndrome

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2CDC
6CNVD
10DGIdb
15ExPASy
16FDA
17FMA
25GTR
26HGMD
27HMDB
28ICD10
29ICD10 via Orphanet
30ICD9CM
31IUPHAR
32KEGG
35MedGen
37MeSH
38MESH via Orphanet
39MGI
42NCI
43NCIt
44NDF-RT
47NINDS
48Novoseek
50OMIM
51OMIM via Orphanet
55PubMed
56QIAGEN
61SNOMED-CT via Orphanet
65Tumor Gene Family of Databases
66UMLS
67UMLS via Orphanet