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Abnormal von Willebrand factor multimer distribution

MedGen UID:
868225
•Concept ID:
C4022617
•
Anatomical Abnormality
HPO: HP:0030131

Definition

Deviation from the normal von Willebrand factor multimer pattern. [from HPO]

Professional guidelines

PubMed

Laboratory aspects of von Willebrand disease: test repertoire and options for activity assays and genetic analysis.
Castaman G, Hillarp A, Goodeve A
Haemophilia 2014 May;20 Suppl 4(0 4):65-70. doi: 10.1111/hae.12410. PMID: 24762278Free PMC Article

Recent clinical studies

Etiology

Collagen binding provides a sensitive screen for variant von Willebrand disease.
Flood VH, Gill JC, Friedman KD, Christopherson PA, Jacobi PM, Hoffmann RG, Montgomery RR, Haberichter SL; Zimmerman Program Investigators
Clin Chem 2013 Apr;59(4):684-91. Epub 2013 Jan 22 doi: 10.1373/clinchem.2012.199000. PMID: 23340442Free PMC Article
Shear stress and von Willebrand factor in health and disease.
Tsai HM
Semin Thromb Hemost 2003 Oct;29(5):479-88. doi: 10.1055/s-2003-44556. PMID: 14631548
Acquired von Willebrand's syndrome: a single institution experience.
Kumar S, Pruthi RK, Nichols WL
Am J Hematol 2003 Apr;72(4):243-7. doi: 10.1002/ajh.10298. PMID: 12666134
Defective von willebrand factor activity detected by the filterometer in three clinical conditions.
O'Brien JR, Tsai HM, Etherington MD
Platelets 2000 Nov;11(7):388-94. doi: 10.1080/09537100020008339. PMID: 11132105

Diagnosis

Laboratory aspects of von Willebrand disease: test repertoire and options for activity assays and genetic analysis.
Castaman G, Hillarp A, Goodeve A
Haemophilia 2014 May;20 Suppl 4(0 4):65-70. doi: 10.1111/hae.12410. PMID: 24762278Free PMC Article
Collagen binding provides a sensitive screen for variant von Willebrand disease.
Flood VH, Gill JC, Friedman KD, Christopherson PA, Jacobi PM, Hoffmann RG, Montgomery RR, Haberichter SL; Zimmerman Program Investigators
Clin Chem 2013 Apr;59(4):684-91. Epub 2013 Jan 22 doi: 10.1373/clinchem.2012.199000. PMID: 23340442Free PMC Article
Clinical measurement of von Willebrand factor by fluorescence correlation spectroscopy.
Torres R, Genzen JR, Levene MJ
Clin Chem 2012 Jun;58(6):1010-8. Epub 2012 Mar 26 doi: 10.1373/clinchem.2011.179200. PMID: 22451621Free PMC Article
Plasma von Willebrand factor multimer quantitative analysis by in-gel immunostaining and infrared fluorescent imaging.
Pruthi RK, Daniels TM, Heit JA, Chen D, Owen WG, Nichols WL
Thromb Res 2010 Dec;126(6):543-9. doi: 10.1016/j.thromres.2010.09.015. PMID: 20889192
Acquired von Willebrand's syndrome: a single institution experience.
Kumar S, Pruthi RK, Nichols WL
Am J Hematol 2003 Apr;72(4):243-7. doi: 10.1002/ajh.10298. PMID: 12666134

Therapy

Acquired von Willebrand's syndrome: a single institution experience.
Kumar S, Pruthi RK, Nichols WL
Am J Hematol 2003 Apr;72(4):243-7. doi: 10.1002/ajh.10298. PMID: 12666134
Defective von willebrand factor activity detected by the filterometer in three clinical conditions.
O'Brien JR, Tsai HM, Etherington MD
Platelets 2000 Nov;11(7):388-94. doi: 10.1080/09537100020008339. PMID: 11132105

Prognosis

Type IB von Willebrand's disease and pregnancy: comparison of analytical methods of von Willebrand factor for classification of von Willebrand's disease subtypes.
Takahashi H, Hayashi N, Shibata A
Thromb Res 1988 May 1;50(3):409-18. doi: 10.1016/0049-3848(88)90270-8. PMID: 3260697

Clinical prediction guides

ADAMTS13 regulation of VWF multimer distribution in severe COVID-19.
Ward SE, Fogarty H, Karampini E, Lavin M, Schneppenheim S, Dittmer R, Morrin H, Glavey S, Ni Cheallaigh C, Bergin C, Martin-Loeches I, Mallon PW, Curley GF, Baker RI, Budde U, O'Sullivan JM, O'Donnell JS; Irish COVID-19 Vasculopathy Study (iCVS) investigators
J Thromb Haemost 2021 Aug;19(8):1914-1921. Epub 2021 Jun 20 doi: 10.1111/jth.15409. PMID: 34053187Free PMC Article
Idiopathic pulmonary arterial hypertension - a unrecognized cause of high-shear high-flow haemostatic defects (otherwise referred to as acquired von Willebrand syndrome) in children.
Pelland-Marcotte MC, Humpl T, James PD, Rand ML, Bouskill V, Reyes JT, Bowman ML, Carcao MD
Br J Haematol 2018 Oct;183(2):267-275. Epub 2018 Aug 23 doi: 10.1111/bjh.15530. PMID: 30141279
Impaired dimerization of von Willebrand factor subunit due to mutation A2801D in the CK domain results in a recessive type 2A subtype IID von Willebrand disease.
Hommais A, Stépanian A, Fressinaud E, Mazurier C, Pouymayou K, Meyer D, Girma JP, Ribba AS
Thromb Haemost 2006 May;95(5):776-81. PMID: 16676067
Shear stress and von Willebrand factor in health and disease.
Tsai HM
Semin Thromb Hemost 2003 Oct;29(5):479-88. doi: 10.1055/s-2003-44556. PMID: 14631548
Multimeric structure of factor VIII/von Willebrand factor in von Willebrand's disease.
Meyer D, Obert B, Pietu G, Lavergne JM, Zimmerman TS
J Lab Clin Med 1980 Apr;95(4):590-602. PMID: 6965697

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