• 1
    Sadler JE. A revised classification of von Willebrand disease. For the Subcommittee on von Willebrand Factor of the Scientific and Standardization Committee of the International Society on Thrombosis and Haemostasis. Thromb Haemost 1994; 10: 5205.
  • 2
    Xie L, Chesterman CN, Hogg PJ. Control of von Willebrand factor multimer size by thrombospondin-1. J Exp Med 2001; 193: 13419.
  • 3
    Zheng X, Chung D, Takayama TK et al. Structure of von Willebrand factor-cleaving protease (ADAMTS13), a metalloprotease involved in thrombotic thrombocytopenic purpura. J Biol Chem 2001; 276: 4105963.
  • 4
    Ruggeri ZM. von Willebrand factor. J Clin Invest 1997; 99: 55964.
  • 5
    Vlot AJ, Koppelman SJ, Meijers JC et al. Kinetics of factor VIII-von Willebrand factor association. Blood 1996; 87: 180916.
  • 6
    Noe DA. A mathematical model of coagulation factor VIII kinetics. Haemostasis 1996; 26: 289303.
  • 7
    Cattaneo M, Simoni L, Gringeri A, Mannucci PM. Patients with severe von Willebrand disease are insensitive to the releasing effect of DDAVP: evidence that the DDAVP-induced increase in plasma factor VIII is not secondary to the increase in plasma von Willebrand factor. Br J Haematol 1994; 86: 3337.
  • 8
    Abildgaard CF, Suzuki Z, Harrison J, Jefcoat K, Zimmerman TS. Serial studies in von Willebrand's disease: variability versus ‘variants’. Blood 1980; 56: 7126.
  • 9
    Mancuso DJ, Tuley EA, Westfield LA et al. Human von Willebrand factor gene and pseudogene: structural analysis and differentiation by polymerase chain reaction. Biochemistry 1991; 30: 25369.
  • 10
    Keightley AM, Lam YM, Brady JN, Cameron CL, Lillicrap D. Variation at the von Willebrand factor (vWF) gene locus is associated with plasma vWF:Ag levels: identification of three novel single nucleotide polymorphisms in the vWF gene promoter. Blood 1999; 93: 427783.
  • 11
    Castaman G, Eikenboom JC, Bertina RM, Rodeghiero F. Inconsistency of association between type 1 von Willebrand disease phenotype and genotype in families identified in an epidemiological investigation. Thromb Haemost 1999; 82: 106570.
  • 12
    Gill JC, EndresBrooks J, Bauer PJ, Marks WJ Jr, Montgomery RR. The effect of ABO blood group on the diagnosis of von Willebrand disease. Blood 1987; 69: 16915.
  • 13
    O'Donnell J, Boulton FE, Manning RA, Laffan MA. Amount of H antigen expressed on circulating von Willebrand factor is modified by ABO blood group genotype and is a major determinant of plasma von Willebrand factor antigen levels. Arterioscler Thromb Vasc Biol 2002; 22: 33541.
  • 14
    Sarode R, Goldstein J, Sussman II, Nagel RL, Tsai HM. Role of A and B blood group antigens in the expression of adhesive activity of von Willebrand factor. Br J Haematol 2000; 109: 85764.
  • 15
    Shima M, Fujimura Y, Nishiyama T et al. ABO blood group genotype and plasma von Willebrand factor in normal individuals. Vox Sang 1995; 68: 23640.
  • 16
    O'Donnell J, Boulton FE, Manning RA, Laffan MA. Genotype at the secretor blood group locus is a determinant of plasma von Willebrand factor level. Br J Haematol 2002; 116: 3506.
  • 17
    Souto J, Almasy L, Soria J et al. Genome-wide linkage analysis of von Willebrand factor plasma levels: results from the GAIT project. Thromb Haemost 2003; 89: 46874.
  • 18
    Kadir RA, Economides DL, Sabin CA, Owens D, Lee CA. Variations in coagulation factors in women: effects of age, ethnicity, menstrual cycle and combined oral contraceptive. Thromb Haemost 1999; 82: 145661.
  • 19
    Miller CH, Dilley A, Richardson L, Hooper WC, Evatt BL. Population differences in von Willebrand factor levels affect the diagnosis of von Willebrand disease in African-American women. Am J Hematol 2001; 67: 1259.
  • 20
    Andrew M, Paes B, Milner R et al. Development of the human coagulation system in the full-term infant. Blood 1987; 70: 16572.
  • 21
    Onundarson PT, Gudmundsdottir BR, Arnfinnsdottir AV, Kjeld M, Olafsson O. von Willebrand factor does not vary during normal menstrual cycle. Thromb Haemost 2001; 85: 1834.
  • 22
    Miller CH, Dilley AB, Drews C, Richardson L, Evatt B. Changes in von Willebrand factor and factor VIII levels during the menstrual cycle. Thromb Haemost 2002; 87: 10823.
  • 23
    David JL, Gaspard UJ, Gillain D, Raskinet R, Lepot MR. Hemostasis profile in women taking low-dose oral contraceptives. Am J Obstet Gynecol 1990; 163: 4203.
  • 24
    Bennett B, Ratnoff OD. Changes in antihemophilic factor (AHF, factor 8) procoagulant activity and AHF-like antigen in normal pregnancy, and following exercise and pneumoencephalography. J Lab Clin Med 1972; 80: 25663.
  • 25
    Stirling Y, Woolf L, North WR, Seghatchian MJ, Meade TW. Haemostasis in normal pregnancy. Thromb Haemost 1984; 52: 17682.
  • 26
    Kadir RA, Lee CA, Sabin CA, Pollard D, Economides DL. Pregnancy in women with von Willebrand's disease or factor XI deficiency. Br J Obstet Gynaecol 1998; 105: 31421.
  • 27
    Ramsahoye BH, Davies SV, Dasani H, Pearson JF. Pregnancy in von Willebrand's disease. J Clin Pathol 1994; 47: 56970.
  • 28
    Greer IA, Lowe GD, Walker JJ, Forbes CD. Haemorrhagic problems in obstetrics and gynaecology in patients with congenital coagulopathies. Br J Obstet Gynaecol 1991; 98: 90918.
  • 29
    Cushman M, Legault C, Connor EB et al. Effect of postmenopausal hormones on inflammation-sensitive proteins: the Postmenopausal Estrogen/Progestin Interventions (PEPI) Study. Circulation 1999; 100: 71722.
  • 30
    De Mitrio V, Marino R, Cicinelli E et al. Beneficial effects of postmenopausal hormone replacement therapy with transdermal estradiol on sensitivity to activated protein C. Blood Coagul Fibrinolysis 2000; 11: 17582.
  • 31
    Jern C, Eriksson E, Tengborn L et al. Changes of plasma coagulation and fibrinolysis in response to mental stress. Thromb Haemost 1989; 62: 76771.
  • 32
    Jern C, Manhem K, Eriksson E et al. Hemostatic responses to mental stress during the menstrual cycle. Thromb Haemost 1991; 66: 6148.
  • 33
    Hansen JB, Wilsgard L, Olsen JO, Osterud B. Formation and persistence of procoagulant and fibrinolytic activities in circulation after strenuous physical exercise. Thromb Haemost 1990; 64: 3859.
  • 34
    Andrew M, Carter C, O'Brodovich H, Heigenhauser G. Increases in factor VIII complex and fibrinolytic activity are dependent on exercise intensity. J Appl Physiol 1986; 60: 191722.
  • 35
    Kirkpatrick UJ, Mossa M, Blann AD, McCollum CN. Repeated exercise induces release of soluble P-selectin in patients with intermittent claudication. Thromb Haemost 1997; 78: 133842.
  • 36
    Ponjee GA, Janssen GM, van Wersch JW. Prolonged endurance exercise and blood coagulation: a 9 month prospective study. Blood Coagul Fibrinolysis 1993; 4: 215.
  • 37
    Casonato A, Pontara E, Bertomoro A et al. Demonstration that venous occlusion fails to release von Willebrand factor multimers. Blood Coagul Fibrinolysis 1995; 6: 5748.
  • 38
    Wieczorek I, Ludlam CA, MacGregor I. Venous occlusion does not release von Willebrand factor, factor VIII or PAI-1 from endothelial cells–the importance of consensus on the use of correction factors for haemoconcentration. Thromb Haemost 1993; 69: 91, 93.
  • 39
    Scharrer I, Vigh Z. Release of von Willebrand factor after venous occlusion – the importance of standardization of venous occlusion tests. Thromb Haemost 1993; 70: 8801.
  • 40
    Wieczorek I, Ludlam CA. Rebuttal to the letter to the editor by Dr Scharrer et al. Thromb Haemost 1993; 70: 881.
  • 41
    James JP, Stevens TR, Hall ND et al. Factor VIII related antigen in connective tissue disease patients and relatives. Br J Rheumatol 1990; 29: 69.
  • 42
    Lombardi R, Mannucci PM, Seghatchian MJ, Garcia VV, Coppola R. Alterations of factor VIII von Willebrand factor in clinical conditions associated with an increase in its plasma concentration. Br J Haematol 1981; 49: 618.
  • 43
    Pottinger BE, Read RC, Paleolog EM, Higgins PG, Pearson JD. Willebrand factor is an acute phase reactant in man. Thromb Res 1989; 53: 38794.
  • 44
    van Duijnhoven EM, Lustermans FA, van Wersch JW. Evaluation of the coagulation/fibrinolysis balance in patients with colorectal cancer. Haemostasis 1993; 23: 16872.
  • 45
    Sweeney JD, Killion KM, Pruet CF, Spaulding MB. Willebrand factor in head and neck cancer. Cancer 1990; 66: 23879.
  • 46
    Gadducci A, Baicchi U, Marrai R et al. Pretreatment plasma levels of fibrinopeptide-A (FPA), D-dimer (DD), and von Willebrand factor (vWF) in patients with operable cervical cancer: influence of surgical-pathological stage, tumor size, histologic type, and lymph node status. Gynecol Oncol 1993; 49: 3548.
  • 47
    Facchini V, Gadducci A, Baicchi U et al. Factor VIIIR:Ag plasma levels in patients with cervical and ovarian carcinoma. Eur J Gynaecol Oncol 1988; 9: 8793.
  • 48
    Myrup B, Bregengard C, Faber J. Primary haemostasis in thyroid disease. J Intern Med 1995; 238: 5963.
  • 49
    Rogers JS Jr, Shane SR, Jencks FS. Factor VIII activity and thyroid function. Ann Intern Med 1982; 97: 7136.
  • 50
    Sadler JE, Mannucci PM, Berntorp E et al. Impact, diagnosis and treatment of von Willebrand disease. Thromb Haemost 2000; 84: 16074.
  • 51
    Nosek Cenkowska B, Cheang MS, Pizzi NJ, Israels ED, Gerrard JM. Bleeding/bruising symptomatology in children with and without bleeding disorders. Thromb Haemost 1991; 65: 23741.
  • 52
    Dean JA, Blanchette VS, Carcao MD et al. von Willebrand disease in a pediatric-based population – comparison of type 1 diagnostic criteria and use of the PFA-100 and a von Willebrand factor/collagen-binding assay. Thromb Haemost 2000; 84: 4019.
  • 53
    Sramek A, Eikenboom JC, Briet E, Vandenbroucke JP, Rosendaal FR. Usefulness of patient interview in bleeding disorders [comment]. Arch Intern Med 1995; 155: 140915.
  • 54
    Fressinaud E, Veyradier A, Truchaud F et al. Screening for von Willebrand disease with a new analyzer using high shear stress: a study of 60 cases. Blood 1998; 91: 132531.
  • 55
    Favaloro EJ, Kershaw G, Bukuya M, Hertzberg M, Koutts J. Laboratory diagnosis of von Willebrand disorder (vWD) and monitoring of DDAVP therapy: efficacy of the PFA-100 and vWF:CBA as combined diagnostic strategies. Haemophilia 2001; 7: 1809.
  • 56
    Favaloro EJ. Utility of the PFA-100 for assessing bleeding disorders and monitoring therapy: a review of analytical variables, benefits and limitations. Haemophilia 2001; 7: 1709.
  • 57
    Burgess C, Credland P, Khair K, Hann I, Liesner R. An evaluation of the usefulness of the PFA-100 device in 100 children referred for investigation of potential haemostatic disorder. Haemophilia 2001; B247 (Abstract).
  • 58
    Scott JP, Montgomery RR, Retzinger GS. Dimeric ristocetin flocculates proteins, binds to platelets, and mediates von Willebrand factor-dependent agglutination of platelets. J Biol Chem 1991; 266: 814955.
  • 59
    Preston FE. Assays for von Willebrand factor functional activity: a UK NEQAS survey. National External Quality Assessment Scheme. Thromb Haemost 1998; 80: 863.
  • 60
    Murdock PJ, Woodhams BJ, Matthews KB, Pasi KJ, Goodall AH. Willebrand factor activity detected in a monoclonal antibody-based ELISA: an alternative to the ristocetin cofactor platelet agglutination assay for diagnostic use. Thromb Haemost 1997; 78: 12727.
  • 61
    Favaloro EJ, Henniker A, Facey D, Hertzberg M. Discrimination of von Willebrands disease (VWD) subtypes: direct comparison of von Willebrand factor:collagen binding assay (VWF:CBA) with monoclonal antibody (MAB) based VWF-capture systems. Thromb Haemost 2000; 84: 5417.
  • 62
    Riddell AF, Jenkins PV, Whalley ICN et al. Use of the collagen-binding assay for von Willebrand factor in the analysis of type 2M von Willebrand disease: a comparison with the ristocetin cofactor assay. Br J Haematol 2002; 116: 18792.
  • 63
    Favaloro EJ. Collagen binding assay for von Willebrand factor (VWF:CBA): detection of von Willebrands Disease (VWD), and discrimination of VWD subtypes, depends on collagen source. Thromb Haemost 2000; 83: 12735.
  • 64
    Casonato A, Pontara E, Bertomoro A et al. von Willebrand factor collagen binding activity in the diagnosis of von Willebrand disease: an alternative to ristocetin co-factor activity? Br J Haematol 2001; 112: 57883.
  • 65
    Ribba AS, Loisel I, Lavergne JM et al. Ser968Thr mutation within the A3 domain of von Willebrand factor (VWF) in two related patients leads to a defective binding of VWF to collagen. Thromb Haemost 2001; 86: 84854.
  • 66
    Cumming AM, Wensley RT. Analysis of von Willebrand factor multimers using a commercially available enhanced chemiluminescence kit. J Clin Pathol 1993; 46: 4703.
  • 67
    Nishino M, Girma JP, Rothschild C, Fressinaud E, Meyer D. New variant of von Willebrand disease with defective binding to factor VIII. Blood 1989; 74: 15919.
  • 68
    Ruggeri ZM, Zimmerman TS. Willebrand factor and von Willebrand disease. Blood 1987; 70: 895904.
  • 69
    Nitu Whalley IC, Lee CA, Griffioen A, Jenkins PV, Pasi KJ. Type 1 von Willebrand disease – a clinical retrospective study of the diagnosis, the influence of the ABO blood group and the role of the bleeding history. Br J Haematol 2000; 108: 25964.
  • 70
    Lippi G, Franchini M, Brocco G, Manzato F. Influence of the ABO blood type on the platelet function analyzer PFA-100. Thromb Haemost 2001; 85: 36970.
  • 71
    Federici AB. Diagnosis of von Willebrand disease. Haemophilia 1998; 4: 65460.
  • 72
    Federici AB, Canciani MT, Forza I, Cozzi G. Ristocetin cofactor and collagen binding activities normalized to antigen levels for a rapid diagnosis of type 2 von Willebrand disease – single center comparison of four different assays. Thromb Haemost 2000; 84: 11278.
  • 73
    Keeney S, Cumming AM. The molecular biology of von Willebrand disease. Clin Lab Haem 2001; 23: 20930.
  • 74
    O'Brien L, James P, Othman M et al. A founder von Willebrand factor haplotype associated with type 1 von Willebrand disease. Blood 2003; prepublished online March 20 2003 DOI 10.1182/blood-2002–12–3693:
  • 75
    Casana P, Martinez F, Haya S, Espinos C, Aznar JA. Significant linkage and non-linkage of type 1 von Willebrand disease to the von Willebrand factor gene. Br J Haematol 2001; 115: 692700.
  • 76
    Mohlke KL, Purkayastha AA, Westrick RJ et al. MVWF, a dominant modifier of murine von Willebrand factor, results from altered lineage-specific expression of a glycoyltransferase. Cell 1999; 96: 11120.
  • 77
    Ginsburg D, Konkle BA, Gill JC et al. Molecular basis of human von Willebrand disease: analysis of platelet von Willebrand factor mRNA. Proc Natl Acad Sci U S A 1989; 86: 37237.
  • 78
    Lyons SE, Bruck ME, Bowie EJ, Ginsburg D. Impaired intracellular transport produced by a subset of type IIA von Willebrand disease mutations. J Biol Chem 1992; 267: 442430.
  • 79
    Gaucher C, Dieval J, Mazurier C. Characterization of von Willebrand factor gene defects in two unrelated patients with type IIC von Willebrand disease. Blood 1994; 84: 102430.
  • 80
    Enayat MS, Guilliatt AM, Surdhar GK et al. Aberrant dimerization of von Willebrand factor as the result of mutations in the carboxy-terminal region: identification of 3 mutations in members of 3 different families with type 2A (phenotype IID) von Willebrand disease. Blood 2001; 98: 67480.
  • 81
    Rick ME, Williams SB, Sacher RA, McKeown LP. Thrombocytopenia associated with pregnancy in a patient with type IIB von Willebrand's disease. Blood 1987; 69: 7869.
  • 82
    Meyer D, Fressinaud E, Gaucher C et al. Gene defects in 150 unrelated French cases with type 2 von Willebrand disease: from the patient to the gene. INSERM Network on Molecular Abnormalities in von Willebrand Disease. Thromb Haemost 1997; 78: 4516.
  • 83
    Miller JL. Willebrand disease. Thromb Haemost 1996; 75: 8659.
  • 84
    Nitu Whalley IC, Riddell A, Lee CA et al. Identification of type 2 von Willebrand disease in previously diagnosed type 1 patients: a reappraisal using phenotypes, genotypes and molecular modelling. Thromb Haemost 2000; 84: 9981004.
  • 85
    Ribba AS, Loisel I, Lavergne JM et al. Ser968Thr mutation within the A3 domain of von Willebrand factor (VWF) in two related patients leads to a defective binding of VWF to collagen. Thromb Haemost 2001; 86: 84854.
  • 86
    Zhang ZP, Blomback M, Nyman D, Anvret M. Mutations of von Willebrand factor gene in families with von Willebrand disease in the Aland Islands. Proc Natl Acad Sci U S A 1993; 90: 793740.
  • 87
    Zhang ZP, Falk G, Blomback M, Egberg N, Anvret M. A single cytosine deletion in exon 18 of the von Willebrand factor gene is the most common mutation in Swedish vWD type III patients. Hum Mol Genet 1992; 1: 7678.
  • 88
    Federici AB, Rand JH, Bucciarelli P et al. Acquired von Willebrand syndrome: data from an international registry. Thromb Haemost 2000; 84: 3459.
  • 89
    Kumar S, Pruthi RK, Nichols WL. Willebrand disease. Mayo Clin Proc 2002; 77: 1817.
  • 90
    Sanchez-Luceros A, Meschengieser SS, Woods AI et al. Acquired von Willebrand factor abnormalities in myeloproliferative disorders and other hematologic diseases: a retrospective analysis by a single institution. Haematologica 2002; 87: 26470.