The Role of the von Willebrand Factor Collagen-Binding Assay (VWF:CB) in the Diagnosis and Treatment of von Willebrand Disease (VWD) and Way Beyond: A Comprehensive 36-Year History^[*]

Abstract

The von Willebrand factor (VWF) collagen binding (VWF:CB) assay was first reported for use in von Willebrand diagnostics in 1986, by Brown and Bosak. Since then, the VWF:CB has continued to be used to help diagnose von Willebrand disease (VWD) (correctly) and also to help assign the correct subtype, as well as to assist in the monitoring of VWD therapy, especially desmopressin (DDAVP). However, it is important to recognize that the specific value of any VWF:CB is predicated on the use of an optimized VWF:CB, and that not all VWF:CB assays are so optimized. There are some good commercial assays available, but there are also some “not-so-good” commercial assays available, and these may continue to give the VWF:CB “a bad reputation.” In addition to VWD diagnosis and management, the VWF:CB found purpose in a variety of other applications, from assessing ADAMTS13 activity, to investigation into acquired von Willebrand syndrome (especially as associated with use of mechanical circulatory support or cardiac assist devices), to assessment of VWF activity in disease states in where an excess of high-molecular-weight VWF may accumulate, and lead to increased (micro)thrombosis risk (e.g., coronavirus disease 2019, thrombotic thrombocytopenic purpura). The VWF:CB turns 37 in 2023. This review is a celebration of the utility of the VWF:CB over this nearly 40-year history.

Keywords

von Willebrand factor - von Willebrand disease - VWF:CB - VWF:RCo - VWF:GPIbR - VWF:GPIbM - VWF:Ag - diagnosis - management - history

^* Dedicated to the memories of J. Evan Sadler (1951-2018), Elizabeth (Betsy) van Cott (1964-2021), and William L. Nichols (1940-2022).

Publication History

Article published online:
20 February 2023

• References

• 1 Colonne CK, Reardon B, Curnow J, Favaloro EJ. Why is misdiagnosis of von Willebrand disease still prevalent and how can we overcome it? A focus on clinical considerations and recommendations. J Blood Med 2021; 12: 755-768
• 2 Favaloro EJ. Von Willebrand disease: local diagnosis and management of a globally distributed bleeding disorder. Semin Thromb Hemost 2011; 37 (05) 440-455
• 3 Favaloro EJ. Navigating the myriad of von Willebrand factor assays. Hamostaseologie 2020; 40 (04) 431-442
• 4 Favaloro EJ. Classification of von Willebrand disease in the context of modern contemporary von Willebrand factor testing methodologies. Res Pract Thromb Haemost 2020; 4 (06) 952-957
• 5 Sadler JE, Budde U, Eikenboom JCJ. et al; Working Party on von Willebrand Disease Classification. Update on the pathophysiology and classification of von Willebrand disease: a report of the Subcommittee on von Willebrand Factor. J Thromb Haemost 2006; 4 (10) 2103-2114
• 6 Curnow J, Pasalic L, Favaloro EJ. Treatment of von Willebrand disease. Semin Thromb Hemost 2016; 42 (02) 133-146
• 7 Duncan E, Rodgers S. One-stage factor VIII assays. Methods Mol Biol 2017; 1646: 247-263
• 8 Rodgers S, Duncan E. Chromogenic factor VIII assays for improved diagnosis of hemophilia A. Methods Mol Biol 2017; 1646: 265-276
• 9 Favaloro EJ, Mohammed S, Patzke J. Laboratory testing for von Willebrand factor antigen (VWF:Ag). Methods Mol Biol 2017; 1646: 403-416
• 10 Favaloro EJ. Diagnosing von Willebrand disease: a short history of laboratory milestones and innovations, plus current status, challenges, and solutions. Semin Thromb Hemost 2014; 40 (05) 551-570
• 11 Mohammed S, Favaloro EJ. Laboratory testing for von Willebrand factor Ristocetin cofactor (VWF:RCo). Methods Mol Biol 2017; 1646: 435-451
• 12 Patzke J, Favaloro EJ. Laboratory testing for von Willebrand factor activity by glycoprotein Ib binding assays (VWF:GPIb). Methods Mol Biol 2017; 1646: 453-460
• 13 Flood VH, Gill JC, Morateck PA. et al. Gain-of-function GPIb ELISA assay for VWF activity in the Zimmerman Program for the Molecular and Clinical Biology of VWD. Blood 2011; 117 (06) e67-e74
• 14 Favaloro EJ, Mohammed S. Towards improved diagnosis of von Willebrand disease: comparative evaluations of several automated von Willebrand factor antigen and activity assays. Thromb Res 2014; 134 (06) 1292-1300
• 15 Favaloro EJ, Dean E, Arunachalam S, Vong R, Mohammed S. Evaluating errors in the laboratory identification of von Willebrand disease using contemporary von Willebrand factor assays. Pathology 2022; 54 (03) 308-317
• 16 Favaloro EJ, Dean E, Arunachalam S. Evaluating performance of contemporary and historical von Willebrand factor (VWF) assays in the laboratory identification of von Willebrand disease (VWD): the Australasian experience. Semin Thromb Hemost 2022; 48 (06) 711-731
• 17 Favaloro EJ. Commentary on the ASH ISTH NHF WFH 2021 guidelines on the diagnosis of VWD: reflections based on recent contemporary test data. Blood Adv 2022; 6 (02) 416-419
• 18 James PD, Connell NT, Ameer B. et al. ASH ISTH NHF WFH 2021 guidelines on the diagnosis of von Willebrand disease. Blood Adv 2021; 5 (01) 280-300
• 19 Flood VH, Gill JC, Morateck PA. et al. Common VWF exon 28 polymorphisms in African Americans affecting the VWF activity assay by ristocetin cofactor. Blood 2010; 116 (02) 280-286
• 20 Favaloro EJ, Mohammed S. Laboratory testing for von Willebrand factor collagen binding (VWF:CB). Methods Mol Biol 2017; 1646: 417-433
• 21 Favaloro EJ. Collagen binding assay for von Willebrand factor (VWF:CBA): detection of von Willebrand's Disease (VWD), and discrimination of VWD subtypes, depends on collagen source. Thromb Haemost 2000; 83 (01) 127-135
• 22 Favaloro EJ. Evaluation of commercial von Willebrand factor collagen binding assays to assist the discrimination of types 1 and 2 von Willebrand disease. Thromb Haemost 2010; 104 (05) 1009-1021
• 23 Rodgers SE, Lerda NV, Favaloro EJ. et al. Identification of von Willebrand disease type 2N (Normandy) in Australia: a cross-laboratory investigation using different methods. Am J Clin Pathol 2002; 118 (02) 269-276
• 24 Mohammed S, Favaloro EJ. Laboratory testing for von Willebrand factor: factor VIII binding (for 2N VWD). Methods Mol Biol 2017; 1646: 461-472
• 25 Frontroth JP, Favaloro EJ. Ristocetin-induced platelet aggregation (RIPA) and RIPA mixing studies. Methods Mol Biol 2017; 1646: 473-494
• 26 Favaloro EJ, Mohammed S, Vong R. et al. How we diagnose 2M von Willebrand disease (VWD): use of a strategic algorithmic approach to distinguish 2M VWD from other VWD types. Haemophilia 2021; 27 (01) 137-148
• 27 Chandler WL, Peerschke EI, Castellone DD, Meijer P. NASCOLA Proficiency Testing Committee. Von Willebrand factor assay proficiency testing. The North American Specialized Coagulation Laboratory Association experience. Am J Clin Pathol 2011; 135 (06) 862-869
• 28 Meijer P, Haverkate F. An external quality assessment program for von Willebrand factor laboratory analysis: an overview from the European concerted action on thrombosis and disabilities foundation. Semin Thromb Hemost 2006; 32 (05) 485-491
• 29 Favaloro EJ, Oliver S. Evaluation of a new commercial von Willebrand factor multimer assay. Haemophilia 2017; 23 (04) e373-e377
• 30 Oliver S, Lau KKE, Chapman K, Favaloro EJ. Laboratory testing for von Willebrand factor multimers. Methods Mol Biol 2017; 1646: 495-511
• 31 Oliver S, Vanniasinkam T, Mohammed S, Vong R, Favaloro EJ. Semi-automated von Willebrand factor multimer assay for von Willebrand disease: further validation, benefits and limitations. Int J Lab Hematol 2019; 41 (06) 762-771
• 32 Favaloro EJ, Oliver S, Mohammed S, Vong R. Comparative assessment of von Willebrand factor multimers vs activity for von Willebrand disease using modern contemporary methodologies. Haemophilia 2020; 26 (03) 503-512
• 33 Favaloro EJ, Bonar RA, Meiring M. et al. Evaluating errors in the laboratory identification of von Willebrand disease in the real world. Thromb Res 2014; 134 (02) 393-403
• 34 Favaloro EJ, Bonar RA, Mohammed S. et al. Type 2M von Willebrand disease - more often misidentified than correctly identified. Haemophilia 2016; 22 (03) e145-e155
• 35 Brown JE, Bosak JO. An ELISA test for the binding of von Willebrand antigen to collagen. Thromb Res 1986; 43 (03) 303-311
• 36 Favaloro EJ, Grispo L, Exner T, Koutts J. Development of a simple collagen based ELISA assay aids in the diagnosis of, and permits sensitive discrimination between type I and type II, von Willebrand's disease. Blood Coagul Fibrinolysis 1991; 2 (02) 285-291
• 37 Perret BA, Furlan M, Jenö P, Beck EA. Von Willebrand factor-dependent agglutination of washed fixed human platelets by insoluble collagen isolated from bovine aorta. J Lab Clin Med 1986; 107 (03) 244-252
• 38 Duggan MJ, DiMichele DM, Christian MJ, Fink LM, Hathaway WE. Collagen-binding of von Willebrand's factor antigen in the classification of von Willebrand's disease. Am J Clin Pathol 1987; 88 (01) 97-102
• 39 Lawrie AS, Harrison P, Armstrong AL, Wilbourn BR, Dalton RG, Savidge GF. Comparison of the in vitro characteristics of von Willebrand factor in British and commercial factor VIII concentrates. Br J Haematol 1989; 73 (01) 100-104
• 40 Niesvizky R, Calandri C, Patel ND, Zhou SL, Potter BJ, Rand JH. von Willebrand factor binding to collagen in patients with end-stage renal disease. J Lab Clin Med 1994; 123 (01) 137-142
• 41 Favaloro EJ, Dean M, Grispo L, Exner T, Koutts J. von Willebrand's disease: use of collagen binding assay provides potential improvement to laboratory monitoring of desmopressin (DDAVP) therapy. Am J Hematol 1994; 45 (03) 205-211
• 42 van Genderen PJ, Vink T, Michiels JJ, van 't Veer MB, Sixma JJ, van Vliet HH. Acquired von Willebrand disease caused by an autoantibody selectively inhibiting the binding of von Willebrand factor to collagen. Blood 1994; 84 (10) 3378-3384
• 43 Rasko JE, North KN, Favaloro EJ, Grispo L, Berndt MC. Attenuated platelet sensitivity to collagen in patients with neurofibromatosis type 1. Br J Haematol 1995; 89 (03) 582-588
• 44 Thomas KB, Sutor AH, Altinkaya N, Grohmann A, Zehenter A, Leititis JU. von Willebrand factor-collagen binding activity is increased in newborns and infants. Acta Paediatr 1995; 84 (06) 697-699
• 45 van Genderen PJ, Budde U, Michiels JJ, van Strik R, van Vliet HH. The reduction of large von Willebrand factor multimers in plasma in essential thrombocythaemia is related to the platelet count. Br J Haematol 1996; 93 (04) 962-965
• 46 Fischer BE, Kramer G, Mitterer A. et al. Effect of multimerization of human and recombinant von Willebrand factor on platelet aggregation, binding to collagen and binding of coagulation factor VIII. Thromb Res 1996; 84 (01) 55-66
• 47 Zieger B, Budde U, Jessat U. et al. New families with von Willebrand disease type 2M (Vicenza). Thromb Res 1997; 87 (01) 57-64
• 48 Chang P, Aronson DL. Plasma derived von Willebrand factor preparations: collagen binding and ristocetin cofactor activities. Thromb Haemost 1997; 78 (02) 930-933
• 49 van Genderen PJ, Prins FJ, Lucas IS. et al. Decreased half-life time of plasma von Willebrand factor collagen binding activity in essential thrombocythaemia: normalization after cytoreduction of the increased platelet count. Br J Haematol 1997; 99 (04) 832-836
• 50 Favaloro EJ, Koutts J. Laboratory assays for von Willebrand factor: relative contribution to the diagnosis of von Willebrand's disease. Pathology 1997; 29 (04) 385-391
• 51 Mohri H, Hisanaga S, Mishima A, Fujimoto S, Uezono S, Okubo T. Autoantibody inhibits binding of von Willebrand factor to glycoprotein Ib and collagen in multiple myeloma: recognition sites present on the A1 loop and A3 domains of von Willebrand factor. Blood Coagul Fibrinolysis 1998; 9 (01) 91-97
• 52 Siekmann J, Turecek PL, Schwarz HP. The determination of von Willebrand factor activity by collagen binding assay. Haemophilia 1998; 4 (Suppl. 03) 15-24
• 53 Fischer BE, Thomas KB, Dorner F. Collagen covalently immobilized onto plastic surfaces simplifies measurement of von Willebrand factor-collagen binding activity. Ann Hematol 1998; 76 (3-4): 159-166
• 54 Ramasamy I, Farrugia A, Tran E, Anastasius V, Charnock A. Biological activity of von Willebrand factor during the manufacture of therapeutic factor VIII concentrates as determined by the collagen-binding assay. Biologicals 1998; 26 (02) 155-166
• 55 Fischer BE, Thomas KB, Dorner F. von Willebrand factor: measuring its antigen or function? Correlation between the level of antigen, activity, and multimer size using various detection systems. Thromb Res 1998; 91 (01) 39-43
• 56 Bodó I, Eikenboom J, Montgomery R, Patzke J, Schneppenheim R, Di Paola J. von Willebrand factor Subcommittee of the Standardization and Scientific Committee of the International Society for Thrombosis and Haemostasis. Platelet-dependent von Willebrand factor activity. Nomenclature and methodology: communication from the SSC of the ISTH. J Thromb Haemost 2015; 13 (07) 1345-1350
• 57 Barington KA, Kaersgaard P. A very-high-purity von Willebrand factor preparation containing high-molecular-weight multimers. Vox Sang 1999; 76 (02) 85-89
• 58 Fischer BE. Recombinant von Willebrand factor: potential therapeutic use. J Thromb Thrombolysis 1999; 8 (03) 197-205
• 59 Johnstone IB. Plasma von Willebrand factor-collagen binding activity in normal dogs and in dogs with von Willebrand's disease. J Vet Diagn Invest 1999; 11 (04) 308-313
• 60 Johnstone IB. Desmopressin enhances the binding of plasma von Willebrand factor to collagen in plasmas from normal dogs and dogs with type I von Willebrand's disease. Can Vet J 1999; 40 (09) 645-648
• 61 Favaloro EJ, Smith J, Petinos P, Hertzberg M, Koutts J. RCPA Quality Assurance Program (QAP) in Haematology Haemostasis Scientific Advisory Panel. Laboratory testing for von Willebrand's disease: an assessment of current diagnostic practice and efficacy by means of a multi-laboratory survey. Thromb Haemost 1999; 82 (04) 1276-1282
• 62 Gerritsen HE, Turecek PL, Schwarz HP, Lämmle B, Furlan M. Assay of von Willebrand factor (vWF)-cleaving protease based on decreased collagen binding affinity of degraded vWF: a tool for the diagnosis of thrombotic thrombocytopenic purpura (TTP). Thromb Haemost 1999; 82 (05) 1386-1389
• 63 Mauz-Körholz C, Budde U, Körholz D, Göbel U. DDAVP treatment in a child with von Willebrand disease type 2M. Eur J Pediatr 1999; 158 (Suppl. 03) S174-S176
• 64 Kertzscher F, Müller AE, Lenk H. Analysis of von Willebrand factor in platelets of patients with various forms of von Willebrand disease: is there a clinical relevance?. Eur J Pediatr 1999; 158 (Suppl. 03) S177-S179
• 65 Favaloro EJ. Laboratory assessment as a critical component of the appropriate diagnosis and sub-classification of von Willebrand's disease. Blood Rev 1999; 13 (04) 185-204
• 66 Favaloro EJ. Sulfatide-binding assay for von Willebrand factor. Detection of von Willebrand's disease without discrimination of vWD subtypes. Thromb Res 2000; 98 (02) 213-219
• 67 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 (03) 401-409
• 68 Favaloro EJ. Detection of von Willebrand disorder and identification of qualitative von Willebrand factor defects. Direct comparison of commercial ELISA-based von Willebrand factor activity options. Am J Clin Pathol 2000; 114 (04) 608-618
• 69 Favaloro EJ, Henniker A, Facey D, Hertzberg M. Discrimination of von Willebrand's 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 (04) 541-547
• 70 Favaloro EJ, Thom J, Baker R. Australasian Society for Thrombosis and Haemostasis (ASTH) Emerging Technologies Group. Assessment of current diagnostic practice and efficacy in testing for von Willebrand's disorder: results from the second Australasian multi-laboratory survey. Blood Coagul Fibrinolysis 2000; 11 (08) 729-737
• 71 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 (06) 1127-1128
• 72 Casonato A, Pontara E, Bertomoro A, Sartorello F, Cattini MG, Girolami A. 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 (03) 578-583
• 73 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 (02) 180-189
• 74 Casonato A, Pontara E, Vertolli UP. et al. Plasma and platelet von Willebrand factor abnormalities in patients with uremia: lack of correlation with uremic bleeding. Clin Appl Thromb Hemost 2001; 7 (02) 81-86
• 75 Hubbard AR, Rigsby P, Barrowcliffe TW. Standardisation of factor VIII and von Willebrand factor in plasma: calibration of the 4th International Standard (97/586). Thromb Haemost 2001; 85 (04) 634-638
• 76 Sutor AH, Thomas KB, Prüfer FH, Grohmann A, Brandis M, Zimmerhackl LB. Function of von Willebrand factor in children with diarrhea-associated hemolytic-uremic syndrome (D+ HUS). Semin Thromb Hemost 2001; 27 (03) 287-292
• 77 Kallas A, Talpsep T. The von Willebrand factor collagen-binding activity assay: clinical application. Ann Hematol 2001; 80 (08) 466-471
• 78 Mannucci PM, Canciani MT, Forza I, Lussana F, Lattuada A, Rossi E. Changes in health and disease of the metalloprotease that cleaves von Willebrand factor. Blood 2001; 98 (09) 2730-2735
• 79 Váradi K, Turecek PL, Mitterer A, Dorner F, Schwarz HP. Thrombin-mediated in vitro processing of pro-von Willebrand factor. Thromb Haemost 2001; 86 (06) 1449-1458
• 80 Riddell AF, Jenkins PV, Nitu-Whalley IC, McCraw AH, Lee CA, Brown SA. 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 (01) 187-192
• 81 Turecek PL, Váradi K, Schlokat U, Pichler L, Dorner F, Schwarz HP. In vivo and in vitro processing of recombinant pro-von Willebrand factor. Histochem Cell Biol 2002; 117 (02) 123-129
• 82 Linder N, Shenkman B, Levin E. et al. Deposition of whole blood platelets on extracellular matrix under flow conditions in preterm infants. Arch Dis Child Fetal Neonatal Ed 2002; 86 (02) F127-F130
• 83 Saenko E, Kannicht C, Loster K. et al. Development and applications of surface plasmon resonance-based von Willebrand factor-collagen binding assay. Anal Biochem 2002; 302 (02) 252-262
• 84 Michiels JJ, van de Velde A, van Vliet HH, van der Planken M, Schroyens W, Berneman Z. Response of von Willebrand factor parameters to desmopressin in patients with type 1 and type 2 congenital von Willebrand disease: diagnostic and therapeutic implications. Semin Thromb Hemost 2002; 28 (02) 111-132
• 85 Neugebauer BM, Goy C, Budek I, Seitz R. Comparison of two von Willebrand factor collagen-binding assays with different binding affinities for low, medium, and high multimers of von Willebrand factor. Semin Thromb Hemost 2002; 28 (02) 139-148
• 86 Turecek PL, Siekmann J, Schwarz HP. Comparative study on collagen-binding enzyme-linked immunosorbent assay and ristocetin cofactor activity assays for detection of functional activity of von Willebrand factor. Semin Thromb Hemost 2002; 28 (02) 149-160
• 87 Budde U, Drewke E, Mainusch K, Schneppenheim R. Laboratory diagnosis of congenital von Willebrand disease. Semin Thromb Hemost 2002; 28 (02) 173-190
• 88 Favaloro EJ. Von Willebrand factor collagen-binding (activity) assay in the diagnosis of von Willebrand disease: a 15-year journey. Semin Thromb Hemost 2002; 28 (02) 191-202
• 89 Finkelstein Y, Shenkman B, Sirota L. et al. Whole blood platelet deposition on extracellular matrix under flow conditions in preterm neonatal sepsis. Eur J Pediatr 2002; 161 (05) 270-274
• 90 Remuzzi G, Galbusera M, Noris M. et al; Italian Registry of Recurrent and Familial HUS/TTP. Thrombotic Thrombocytopenic Purpura/Hemolytic Uremic Syndrome. von Willebrand factor cleaving protease (ADAMTS13) is deficient in recurrent and familial thrombotic thrombocytopenic purpura and hemolytic uremic syndrome. Blood 2002; 100 (03) 778-785
• 91 Hubbard AR, Sands D, Chang AC, Mazurier C. Standardisation of von Willebrand factor in therapeutic concentrates: calibration of the 1st International Standard for von Willebrand Factor concentrate (00/514). Thromb Haemost 2002; 88 (03) 380-386
• 92 Rick ME, Moll S, Taylor MA, Krizek DM, White II GC, Aronson DL. Clinical use of a rapid collagen binding assay for von Willebrand factor cleaving protease in patients with thrombotic thrombocytopenic purpura. Thromb Haemost 2002; 88 (04) 598-604
• 93 Paczuski R. Determination of von Willebrand factor activity with collagen-binding assay and diagnosis of von Willebrand disease: effect of collagen source and coating conditions. J Lab Clin Med 2002; 140 (04) 250-254
• 94 Sciahbasi A, Andreotti F, De Cristofaro R. et al. Prothrombotic response to coronary angioplasty in patients with unstable angina and raised C-reactive protein. J Thromb Thrombolysis 2002; 14 (02) 131-138
• 95 Haley E, Babar N, Ritter C. et al. Effect of ABO blood group on the collagen-binding assay for von Willebrand factor. Am J Hematol 2002; 71 (03) 229-231
• 96 Neugebauer BM, Goy C, Seitz R. A collagen binding assay: an additional method for von Willebrand factor activity in therapeutic concentrates. Thromb Haemost 2002; 88 (05) 871-872
• 97 Amaral MM, Kempfer AC, Farías CE, Carballo GA, Silaf MR, Lazzari MA. Von Willebrand factor cleaving protease activity in the physiopathology of microangiopathic disorders. Medicina (B Aires) 2003; 63 (02) 130-136
• 98 Bowen DJ. An influence of ABO blood group on the rate of proteolysis of von Willebrand factor by ADAMTS13. J Thromb Haemost 2003; 1 (01) 33-40
• 99 Knovich MA, Case D, Owen J. Inappropriate use of positive predictive value in describing the rapid collagen binding assay for von Willebrand factor cleaving protease. Thromb Haemost 2003; 89 (04) 768-769
• 100 Vincentelli A, Susen S, Le Tourneau T. et al. Acquired von Willebrand syndrome in aortic stenosis. N Engl J Med 2003; 349 (04) 343-349
• 101 Studt JD, Böhm M, Budde U, Girma JP, Varadi K, Lämmle B. Measurement of von Willebrand factor-cleaving protease (ADAMTS-13) activity in plasma: a multicenter comparison of different assay methods. J Thromb Haemost 2003; 1 (09) 1882-1887
• 102 Miller CH, Haff E, Platt SJ. et al. Measurement of von Willebrand factor activity: relative effects of ABO blood type and race. J Thromb Haemost 2003; 1 (10) 2191-2197
• 103 Zhou J, Jia YQ, Jiang H, Yang YM, Deng CQ. [Application of collagen-binding assay for von Willebrand disease]. Sichuan Da Xue Xue Bao Yi Xue Ban 2004; 35 (01) 126-129
• 104 Sakai M, Shima M, Izumi Y, Shirahata A. [Acquired von Willebrand syndrome with autoimmune hemolytic anemia]. Rinsho Ketsueki 2004; 45 (03) 223-227
• 105 Lethagen S, Carlson M, Hillarp A. A comparative in vitro evaluation of six von Willebrand factor concentrates. Haemophilia 2004; 10 (03) 243-249
• 106 Gao WQ, Su J, Bai X, Wang ZY, Ruan CG. Evaluation of von Willebrand factor-cleaving protease activity in patients with thrombotic thrombocytopenic purpura. Chin Med J (Engl) 2004; 117 (06) 818-822
• 107 Gao WQ, Su J, Xing XP. et al. [Residual collagen binding assay for von Willebrand factor-cleaving protease activity and its clinical application]. Zhongguo Shi Yan Xue Ye Xue Za Zhi 2004; 12 (06) 726-729
• 108 Burnouf T, Caron C, Burkhardt T, Goudemand J. Content and functional activity of von Willebrand factor in apheresis plasma. Vox Sang 2004; 87 (01) 27-33
• 109 Hubbard AR, Heath AB. Standardization of factor VIII and von Willebrand factor in plasma: calibration of the WHO 5th International Standard (02/150). J Thromb Haemost 2004; 2 (08) 1380-1384
• 110 Tripodi A, Chantarangkul V, Böhm M. et al. Measurement of von Willebrand factor cleaving protease (ADAMTS-13): results of an international collaborative study involving 11 methods testing the same set of coded plasmas. J Thromb Haemost 2004; 2 (09) 1601-1609
• 111 Casonato A, Cattini MG, Soldera C. et al. A new L1446P mutation is responsible for impaired von Willebrand factor synthesis, structure, and function. J Lab Clin Med 2004; 144 (05) 254-259
• 112 Chng WJ, Yip CY, Baliwag MB, Liu TC. Differential effect of the ABO blood group on von Willebrand factor collagen binding activity and ristocetin cofactor assay. Blood Coagul Fibrinolysis 2005; 16 (01) 75-78
• 113 Favaloro EJ, Bonar R, Kershaw G. et al; Royal College Of Pathologists Of Australasia Quality Assurance Program In Haematology. Laboratory diagnosis of von Willebrand disorder: use of multiple functional assays reduces diagnostic error rates. Lab Hematol 2005; 11 (02) 91-97
• 114 Mühlhausen C, Schneppenheim R, Budde U. et al. Decreased plasma concentration of von Willebrand factor antigen (VWF:Ag) in patients with glycogen storage disease type Ia. J Inherit Metab Dis 2005; 28 (06) 945-950
• 115 Hellström-Westas L, Ley D, Berg AC, Kristoffersson AC, Holmberg L. VWF-cleaving protease (ADAMTS13) in premature infants. Acta Paediatr 2005; 94 (02) 205-210
• 116 Reiter RA, Varadi K, Turecek PL, Jilma B, Knöbl P. Changes in ADAMTS13 (von-Willebrand-factor-cleaving protease) activity after induced release of von Willebrand factor during acute systemic inflammation. Thromb Haemost 2005; 93 (03) 554-558
• 117 Trasi S, Shetty S, Ghosh K, Mohanty D. Prevalence and spectrum of von Willebrand disease from western India. Indian J Med Res 2005; 121 (05) 653-658
• 118 Callan MB, Giger U, Catalfamo JL. Effect of desmopressin on von Willebrand factor multimers in Doberman Pinschers with type 1 von Willebrand disease. Am J Vet Res 2005; 66 (05) 861-867
• 119 Heseltine JC, Panciera DL, Troy GC, Monroe WE, Brooks MB, Feldman BF. Effect of levothyroxine administration on hemostatic analytes in Doberman Pinschers with von Willebrand disease. J Vet Intern Med 2005; 19 (04) 523-527
• 120 Rojnuckarin P, Akkawat B, Intragumtornchai T. Von Willebrand factor (vWF) antigen levels and function in healthy Thais. Southeast Asian J Trop Med Public Health 2005; 36 (05) 1292-1297
• 121 O'Donnell JS, McKinnon TA, Crawley JT, Lane DA, Laffan MA. Bombay phenotype is associated with reduced plasma-VWF levels and an increased susceptibility to ADAMTS13 proteolysis. Blood 2005; 106 (06) 1988-1991
• 122 Penas N, Pérez-Rodríguez A, Torea JH. et al. von Willebrand disease R1374C: type 2A or 2M? A challenge to the revised classification. High frequency in the northwest of Spain (Galicia). Am J Hematol 2005; 80 (03) 188-196
• 123 Liu F, Lu GY, Dong NZ, Bai X, Su J, Ruan CG. [A study on the significance of plasma thrombospondin1 in thrombotic thrombocytopenic purpura and the relationship between thrombospondin1 and von Willebrand factor cleaving protease (ADAMTS13)]. Zhonghua Yi Xue Za Zhi 2005; 85 (45) 3190-3193
• 124 Favaloro EJ, Soltani S, McDonald J, Grezchnik E, Easton L, Favaloro JW. Reassessment of ABO blood group, sex, and age on laboratory parameters used to diagnose von Willebrand disorder: potential influence on the diagnosis vs the potential association with risk of thrombosis. Am J Clin Pathol 2005; 124 (06) 910-917 [ Erratum in: Am J Clin Pathol. 2006 May;125(5):796]
• 125 Sabino EP, Erb HN, Catalfamo JL. Development of a collagen-binding activity assay as a screening test for type II von Willebrand disease in dogs. Am J Vet Res 2006; 67 (02) 242-249
• 126 Couto CG, Lara A, Iazbik MC, Brooks MB. Evaluation of platelet aggregation using a point-of-care instrument in retired racing Greyhounds. J Vet Intern Med 2006; 20 (02) 365-370
• 127 Lu GY, Guo XF, Wang ZY, Bai X, Su J, Ruan CG. [The association of von Willebrand factor and von Willebrand factor-cleaving protease in systemic lupus erythematosus]. Zhonghua Nei Ke Za Zhi 2006; 45 (01) 42-44
• 128 Liu F, Feys HB, Dong NZ. et al. [Determination of the ADAMTS13 antigen and its activity in TTP patients and carriers]. Zhonghua Xue Ye Xue Za Zhi 2006; 27 (03) 154-157
• 129 Iwaki T, Sandoval-Cooper MJ, Brechmann M, Ploplis VA, Castellino FJ. A fibrinogen deficiency accelerates the initiation of LDL cholesterol-driven atherosclerosis via thrombin generation and platelet activation in genetically predisposed mice. Blood 2006; 107 (10) 3883-3891
• 130 Lisman T, Bongers TN, Adelmeijer J. et al. Elevated levels of von Willebrand Factor in cirrhosis support platelet adhesion despite reduced functional capacity. Hepatology 2006; 44 (01) 53-61
• 131 Casonato A, Fabris F, Pontara E. et al. Diagnosis and follow-up of thrombotic thrombocytopenic purpura by means of von Willebrand factor collagen binding assay. Clin Appl Thromb Hemost 2006; 12 (03) 296-304
• 132 Sucker C, Senft B, Scharf RE, Zotz RB. Determination of von Willebrand factor activity: evaluation of the HaemosIL assay in comparison with established procedures. Clin Appl Thromb Hemost 2006; 12 (03) 305-310
• 133 Shelat SG, Smith P, Ai J, Zheng XL. Inhibitory autoantibodies against ADAMTS-13 in patients with thrombotic thrombocytopenic purpura bind ADAMTS-13 protease and may accelerate its clearance in vivo. J Thromb Haemost 2006; 4 (08) 1707-1717
• 134 Shenkman B, Budde U, Angerhaus D. et al. ADAMTS-13 regulates platelet adhesion under flow. A new method for differentiation between inherited and acquired thrombotic thrombocytopenic purpura. Thromb Haemost 2006; 96 (02) 160-166
• 135 Baronciani L, Federici AB, Cozzi G, Canciani MT, Mannucci PM. von Willebrand factor collagen binding assay in von Willebrand disease type 2A, 2B, and 2M. J Thromb Haemost 2006; 4 (09) 2088-2090
• 136 Favaloro EJ. Laboratory monitoring of therapy in von Willebrand disease: efficacy of the PFA-100 and von Willebrand factor:collagen-binding activity as coupled strategies. Semin Thromb Hemost 2006; 32 (06) 566-576
• 137 Budde U, Metzner HJ, Müller HG. Comparative analysis and classification of von Willebrand factor/factor VIII concentrates: impact on treatment of patients with von Willebrand disease. Semin Thromb Hemost 2006; 32 (06) 626-635
• 138 Popov J, Zhukov O, Ruden S, Zeschmann T, Sferruzza A, Sahud M. Performance and clinical utility of a commercial von Willebrand factor collagen binding assay for laboratory diagnosis of von Willebrand disease. Clin Chem 2006; 52 (10) 1965-1967
• 139 Coleman R, Favaloro EJ, Soltani S, Keng TB. Acquired von Willebrand disease: potential contribution of the VWF:CB to the identification of functionally inhibiting auto-antibodies to von Willebrand factor. J Thromb Haemost 2006; 4 (09) 2085-2088
• 140 Lo B, Nierich AP, Kalkman CJ, Fijnheer R. Relatively increased von Willebrand factor activity after off-pump coronary artery bypass graft surgery. Thromb Haemost 2007; 97 (01) 21-26
• 141 Meiring M, Badenhorst PN, Kelderman M. Performance and utility of a cost-effective collagen-binding assay for the laboratory diagnosis of Von Willebrand disease. Clin Chem Lab Med 2007; 45 (08) 1068-1072
• 142 Gudmundsdottir BR, Marder VJ, Onundarson PT. Risk of excessive bleeding associated with marginally low von Willebrand factor and mild platelet dysfunction. J Thromb Haemost 2007; 5 (02) 274-281
• 143 Starke R, Machin S, Scully M, Purdy G, Mackie I. The clinical utility of ADAMTS13 activity, antigen and autoantibody assays in thrombotic thrombocytopenic purpura. Br J Haematol 2007; 136 (04) 649-655
• 144 Davies JA, Collins PW, Hathaway LS, Bowen DJ. Effect of von Willebrand factor Y/C1584 on in vivo protein level and function and interaction with ABO blood group. Blood 2007; 109 (07) 2840-2846
• 145 Veyradier A, Trossaert M, Lefrancois A, Fressinaud E, Meyer D. von Willebrand factor collagen binding assay with a commercial kit using type III collagen in von Willebrand disease type 2. J Thromb Haemost 2007; 5 (04) 868-870
• 146 Favaloro EJ, Lloyd J, Rowell J. et al. Comparison of the pharmacokinetics of two von Willebrand factor concentrates [Biostate and AHF (High Purity)] in people with von Willebrand disorder. A randomised cross-over, multi-centre study. Thromb Haemost 2007; 97 (06) 922-930
• 147 Feys HB, Canciani MT, Peyvandi F, Deckmyn H, Vanhoorelbeke K, Mannucci PM. ADAMTS13 activity to antigen ratio in physiological and pathological conditions associated with an increased risk of thrombosis. Br J Haematol 2007; 138 (04) 534-540
• 148 Wadanoli M, Sako D, Shaw GD. et al. The von Willebrand factor antagonist (GPG-290) prevents coronary thrombosis without prolongation of bleeding time. Thromb Haemost 2007; 98 (02) 397-405
• 149 Gunther K, Garizio D, Nesara P. ADAMTS13 activity and the presence of acquired inhibitors in human immunodeficiency virus-related thrombotic thrombocytopenic purpura. Transfusion 2007; 47 (09) 1710-1716
• 150 Favaloro EJ. An update on the von Willebrand factor collagen binding assay: 21 years of age and beyond adolescence but not yet a mature adult. Semin Thromb Hemost 2007; 33 (08) 727-744
• 151 Favaloro EJ, Kershaw G, McLachlan AJ, Lloyd J. Time to think outside the box? Proposals for a new approach to future pharmacokinetic studies of von Willebrand factor concentrates in people with von Willebrand disease. Semin Thromb Hemost 2007; 33 (08) 745-758
• 152 Marcucci R, Cesari F, Cinotti S. et al. ADAMTS-13 activity in the presence of elevated von Willebrand factor levels as a novel mechanism of residual platelet reactivity in high risk coronary patients on antiplatelet treatment. Thromb Res 2008; 123 (01) 130-136
• 153 Lu GY, Shen L, Wang ZY. et al. Significance of plasma von Willebrand factor level and von Willebrand factor-cleaving protease activity in patients with chronic renal diseases. Chin Med J (Engl) 2008; 121 (02) 133-136
• 154 Tiede A, Priesack J, Werwitzke S. et al. Diagnostic workup of patients with acquired von Willebrand syndrome: a retrospective single-centre cohort study. J Thromb Haemost 2008; 6 (04) 569-576
• 155 Gallinaro L, Cattini MG, Sztukowska M. et al. A shorter von Willebrand factor survival in O blood group subjects explains how ABO determinants influence plasma von Willebrand factor. Blood 2008; 111 (07) 3540-3545
• 156 Geisen U, Heilmann C, Beyersdorf F. et al. Non-surgical bleeding in patients with ventricular assist devices could be explained by acquired von Willebrand disease. Eur J Cardiothorac Surg 2008; 33 (04) 679-684
• 157 Chung MC, Popova TG, Jorgensen SC. et al. Degradation of circulating von Willebrand factor and its regulator ADAMTS13 implicates secreted Bacillus anthracis metalloproteases in anthrax consumptive coagulopathy. J Biol Chem 2008; 283 (15) 9531-9542
• 158 Hanebutt FL, Rolf N, Loesel A, Kuhlisch E, Siegert G, Knoefler R. Evaluation of desmopressin effects on haemostasis in children with congenital bleeding disorders. Haemophilia 2008; 14 (03) 524-530
• 159 Lara-García A, Couto CG, Iazbik MC, Brooks MB. Postoperative bleeding in retired racing greyhounds. J Vet Intern Med 2008; 22 (03) 525-533
• 160 Guerin V, Ryman A, Velez F. Acquired von Willebrand disease: potential contribution of the von Willebrand factor collagen-binding to the identification of functionally inhibiting auto-antibodies to von Willebrand factor: a rebuttal. J Thromb Haemost 2008; 6 (06) 1051-1052
• 161 Burnouf T, Caron C, Radosevich M, Goubran HA, Goudemand J, El-Ekiaby M. Properties of a concentrated minipool solvent-detergent treated cryoprecipitate processed in single-use bag systems. Haemophilia 2008; 14 (05) 956-962
• 162 Prohaska W, Zittermann A, Lüth JU. et al. Prevalent platelet dysfunction in patients with aortic valve disease. J Heart Valve Dis 2008; 17 (05) 542-547
• 163 Song J, Lee KA, Park TS, Park R, Choi JR. Linear relationship between ADAMTS13 activity and platelet dynamics even before severe thrombocytopenia. Ann Clin Lab Sci 2008; 38 (04) 368-375
• 164 Burgess H, Wood D. Validation of a von Willebrand factor antigen enzyme-linked immunosorbent assay and newly developed collagen-binding assay. Can J Vet Res 2008; 72 (05) 420-427
• 165 Le Tourneau T, Susen S, Caron C. et al. Functional impairment of von Willebrand factor in hypertrophic cardiomyopathy: relation to rest and exercise obstruction. Circulation 2008; 118 (15) 1550-1557
• 166 Hermans C, Batlle J. Autosomal dominant von Willebrand disease type 2M. Acta Haematol 2009; 121 (2-3): 139-144
• 167 Gadisseur A, van der Planken M, Schroyens W, Berneman Z, Michiels JJ. Dominant von Willebrand disease type 2M and 2U are variable expressions of one distinct disease entity caused by loss-of-function mutations in the A1 domain of the von Willebrand factor gene. Acta Haematol 2009; 121 (2-3): 145-153
• 168 Michiels JJ, van Vliet HH. Dominant von Willebrand disease type 2A groups I and II due to missense mutations in the A2 domain of the von Willebrand factor gene: diagnosis and management. Acta Haematol 2009; 121 (2-3): 154-166
• 169 Feys HB, Deckmyn H, Vanhoorelbeke K. ADAMTS13 in health and disease. Acta Haematol 2009; 121 (2-3): 183-185
• 170 Favaloro EJ. Toward a new paradigm for the identification and functional characterization of von Willebrand disease. Semin Thromb Hemost 2009; 35 (01) 60-75
• 171 Larkin D, de Laat B, Jenkins PV. et al. Severe Plasmodium falciparum malaria is associated with circulating ultra-large von Willebrand multimers and ADAMTS13 inhibition. PLoS Pathog 2009; 5 (03) e1000349
• 172 Favaloro EJ, Thom J, Patterson D. et al. Desmopressin therapy to assist the functional identification and characterisation of von Willebrand disease: differential utility from combining two (VWF:CB and VWF:RCo) von Willebrand factor activity assays?. Thromb Res 2009; 123 (06) 862-868
• 173 Caudill JS, Nichols WL, Plumhoff EA. et al. Comparison of coagulation factor XIII content and concentration in cryoprecipitate and fresh-frozen plasma. Transfusion 2009; 49 (04) 765-770
• 174 Pérez-Rodríguez A, García-Rivero A, Lourés E, López-Fernández MF, Rodríguez-Trillo A, Batlle J. Autosomal dominant C1149R von Willebrand disease: phenotypic findings and their implications. Haematologica 2009; 94 (05) 679-686
• 175 Varadi K, Rottensteiner H, Vejda S. et al. Species-dependent variability of ADAMTS13-mediated proteolysis of human recombinant von Willebrand factor. J Thromb Haemost 2009; 7 (07) 1134-1142
• 176 Udvardy ML, Szekeres-Csiki K, Hársfalvi J. Novel evaluation method for densitometric curves of von Willebrand factor multimers and a new parameter (M(MW)) to describe the degree of multimerisation. Thromb Haemost 2009; 102 (02) 412-417
• 177 Favaloro EJ, Thom J, Patterson D. et al. Potential supplementary utility of combined PFA-100 and functional von Willebrand factor testing for the laboratory assessment of desmopressin and factor concentrate therapy in von Willebrand disease. Blood Coagul Fibrinolysis 2009; 20 (06) 475-483
• 178 Burgess HJ, Woods JP, Abrams-Ogg AC, Wood RD. Use of a questionnaire to predict von Willebrand disease status and characterize hemorrhagic signs in a population of dogs and evaluation of a diagnostic profile to predict risk of bleeding. Can J Vet Res 2009; 73 (04) 241-251
• 179 Burgess HJ, Woods JP, Abrams-Ogg AC, Wood RD. Evaluation of laboratory methods to improve characterization of dogs with von Willebrand disease. Can J Vet Res 2009; 73 (04) 252-259
• 180 Riddell AF, Gomez K, Millar CM. et al. Characterization of W1745C and S1783A: 2 novel mutations causing defective collagen binding in the A3 domain of von Willebrand factor. Blood 2009; 114 (16) 3489-3496
• 181 Fuchs B, Budde U, Schulz A, Kessler CM, Fisseau C, Kannicht C. Flow-based measurements of von Willebrand factor (VWF) function: binding to collagen and platelet adhesion under physiological shear rate. Thromb Res 2010; 125 (03) 239-245
• 182 Mina A, Favaloro EJ, Mohammed S, Koutts J. A laboratory evaluation into the short activated partial thromboplastin time. Blood Coagul Fibrinolysis 2010; 21 (02) 152-157
• 183 Mori F, Rossi P, Nardini I, Gambelli D, Farina C. Evaluation of von Willebrand factor activity in factor VIII/von Willebrand factor concentrates with the automated von Willebrand factor: activity IL test. Blood Coagul Fibrinolysis 2010; 21 (03) 221-228
• 184 Bongers TN, Emonts M, de Maat MP. et al. Reduced ADAMTS13 in children with severe meningococcal sepsis is associated with severity and outcome. Thromb Haemost 2010; 103 (06) 1181-1187
• 185 Wang AY, Dong NZ, Ma ZN, Zhang JY, Su J, Ruan CG. Evaluation and clinical application of a new method for detecting ADAMTS13 activity. Chin Med J (Engl) 2010; 123 (14) 1859-1863
• 186 Casonato A, Gallinaro L, Cattini MG. et al. Reduced survival of type 2B von Willebrand factor, irrespective of large multimer representation or thrombocytopenia. Haematologica 2010; 95 (08) 1366-1372
• 187 Wang A, Liu F, Dong N. et al. Thrombospondin-1 and ADAMTS13 competitively bind to VWF A2 and A3 domains in vitro. Thromb Res 2010; 126 (04) e260-e265
• 188 Crow S, Chen D, Milano C. et al. Acquired von Willebrand syndrome in continuous-flow ventricular assist device recipients. Ann Thorac Surg 2010; 90 (04) 1263-1269 , discussion 1269
• 189 Meyer AL, Malehsa D, Bara C. et al. Acquired von Willebrand syndrome in patients with an axial flow left ventricular assist device. Circ Heart Fail 2010; 3 (06) 675-681
• 190 Frontroth JP, Hepner M, Sciuccati G, Feliú Torres A, Pieroni G, Bonduel M. Prospective study of low-dose ristocetin-induced platelet aggregation to identify type 2B von Willebrand disease (VWD) and platelet-type VWD in children. Thromb Haemost 2010; 104 (06) 1158-1165
• 191 van Loon JE, Leebeek FW, Deckers JW. et al. Effect of genetic variations in syntaxin-binding protein-5 and syntaxin-2 on von Willebrand factor concentration and cardiovascular risk. Circ Cardiovasc Genet 2010; 3 (06) 507-512
• 192 Shapovalov KG, Vitkovskiĭ IuA. [Collagen-binding activity of Willebrand factor and thrombocyte aggregation in patients with frostbite]. Voen Med Zh 2010; 331 (12) 18-22
• 193 van Schie MC, de Maat MP, Isaacs A. et al. Variation in the von Willebrand factor gene is associated with von Willebrand factor levels and with the risk for cardiovascular disease. Blood 2011; 117 (04) 1393-1399
• 194 Chen J, Reheman A, Gushiken FC. et al. N-acetylcysteine reduces the size and activity of von Willebrand factor in human plasma and mice. J Clin Invest 2011; 121 (02) 593-603
• 195 Qin HH, Wang XF, Ding QL. et al. [Phenotype and genotype analysis of three Chinese pedigrees with von Willebrand disease]. Zhonghua Xue Ye Xue Za Zhi 2011; 32 (02) 99-102
• 196 Karger R, Weippert-Kretschmer M, Budde U, Kretschmer V. Diagnosis and therapeutic management in a patient with type 2B-like acquired von Willebrand syndrome. Blood Coagul Fibrinolysis 2011; 22 (02) 144-147
• 197 Yango J, Alexopoulou O, Eeckhoudt S, Hermans C, Daumerie C. Evaluation of the respective influence of thyroid hormones and TSH on blood coagulation parameters after total thyroidectomy. Eur J Endocrinol 2011; 164 (04) 599-603
• 198 Solomon C, Budde U, Schneppenheim S. et al. Acquired type 2A von Willebrand syndrome caused by aortic valve disease corrects during valve surgery. Br J Anaesth 2011; 106 (04) 494-500
• 199 Steinlechner B, Zeidler P, Base E. et al. Patients with severe aortic valve stenosis and impaired platelet function benefit from preoperative desmopressin infusion. Ann Thorac Surg 2011; 91 (05) 1420-1426
• 200 Tauer JT, Gneuss A, Lohse JE, Jürgens T, Knöfler R. Evaluation of desmopressin effect on primary haemostasis in pediatric patients with aspirin-like defect as hereditary thrombocytopathy. Klin Padiatr 2011; 223 (03) 169-172
• 201 Topf HG, Weiss D, Lischetzki G, Strasser E, Rascher W, Rauh M. Evaluation of a modified thromboelastography assay for the screening of von Willebrand disease. Thromb Haemost 2011; 105 (06) 1091-1099
• 202 Sosothikul D, Kittikalayawong Y, Aungbamnet P, Buphachat C, Seksarn P. Reference values for thrombotic markers in children. Blood Coagul Fibrinolysis 2012; 23 (03) 208-211
• 203 Lotta LA, Lombardi R, Mariani M. et al. Platelet reactive conformation and multimeric pattern of von Willebrand factor in acquired thrombotic thrombocytopenic purpura during acute disease and remission. J Thromb Haemost 2011; 9 (09) 1744-1751
• 204 Pérez-Rodríguez A, Pinto JC, Lourés E. et al. Acquired von Willebrand syndrome and mitral valve prosthesis leakage. A pilot study. Eur J Haematol 2011; 87 (05) 448-456
• 205 Heilmann C, Geisen U, Beyersdorf F. et al. Acquired Von Willebrand syndrome is an early-onset problem in ventricular assist device patients. Eur J Cardiothorac Surg 2011; 40 (06) 1328-1333 , discussion 1233
• 206 Dieckmann KP, Struss WJ, Budde U. Evidence for acute vascular toxicity of cisplatin-based chemotherapy in patients with germ cell tumour. Anticancer Res 2011; 31 (12) 4501-4505
• 207 Heilmann C, Geisen U, Beyersdorf F. et al. Acquired von Willebrand syndrome in patients with extracorporeal life support (ECLS). Intensive Care Med 2012; 38 (01) 62-68
• 208 Choi SJ, Lee EY, Kim HJ. et al. A Gly1609Arg missense mutation in the vWF gene in a Korean patient with von Willebrand disease type 2A. Ann Clin Lab Sci 2012; 42 (01) 98-102
• 209 Weiss DR, Strasser EF, Ringwald J, Zimmermann R, Eckstein R. High resolution multimer analysis and the PFA-100 platelet function analyser can detect von Willebrand disease type 2A without a pathological ratio of ristocetin cofactor activity and von Willebrand antigen level. Clin Lab 2012; 58 (11-12): 1203-1209
• 210 Czúcz J, Schaffer G, Csuka D. et al. Endothelial cell function in patients with hereditary angioedema: elevated soluble E-selectin level during inter-attack periods. J Clin Immunol 2012; 32 (01) 61-69
• 211 Favaloro EJ, Bonar R, Chapman K, Meiring M, Funk Adcock D. Differential sensitivity of von Willebrand factor (VWF) ‘activity’ assays to large and small VWF molecular weight forms: a cross-laboratory study comparing ristocetin cofactor, collagen-binding and mAb-based assays. J Thromb Haemost 2012; 10 (06) 1043-1054
• 212 Flood VH, Gill JC, Christopherson PA. et al. Critical von Willebrand factor A1 domain residues influence type VI collagen binding. J Thromb Haemost 2012; 10 (07) 1417-1424
• 213 Flood VH, Gill JC, Christopherson PA. et al. Comparison of type I, type III and type VI collagen binding assays in diagnosis of von Willebrand disease. J Thromb Haemost 2012; 10 (07) 1425-1432
• 214 Koyama N, Matsumoto M, Tamaki S, Yoshikawa M, Fujimura Y, Kimura H. Reduced larger von Willebrand factor multimers at dawn in OSA plasmas reflect severity of apnoeic episodes. Eur Respir J 2012; 40 (03) 657-664
• 215 Hassan R, Yusof WA, Hussain NH, Abdullah WZ. Single-center experience of von Willebrand disease (vWD) among patients with menorrhagia: a diagnosis which could be missed. Indian J Hematol Blood Transfus 2012; 28 (03) 157-161
• 216 Jiang LL, Wang XF, Ding QL. et al. [Phenotype and genotype analysis of two Chinese pedigrees with type 3 von Willebrand diseases]. Zhonghua Yi Xue Yi Chuan Xue Za Zhi 2012; 29 (05) 524-528
• 217 Jiang LL, Cao YN, Wang XF. et al. [The phenotypic and genotypic diagnosis of three Chinese patients with von Willebrand disease]. Zhonghua Nei Ke Za Zhi 2012; 51 (10) 788-792
• 218 Wiegand G, Hofbeck M, Zenker M, Budde U, Rauch R. Bleeding diathesis in Noonan syndrome: is acquired von Willebrand syndrome the clue?. Thromb Res 2012; 130 (05) e251-e254
• 219 Montilla M, Hernández-Ruiz L, García-Cozar FJ, Alvarez-Laderas I, Rodríguez-Martorell J, Ruiz FA. Polyphosphate binds to human von Willebrand factor in vivo and modulates its interaction with glycoprotein Ib. J Thromb Haemost 2012; 10 (11) 2315-2323
• 220 Mina A, Favaloro EJ, Koutts J. A novel flow cytometry single tube bead assay for quantitation of von Willebrand factor antigen and collagen-binding. Thromb Haemost 2012; 108 (05) 999-1005
• 221 Al-Awadhi AM, Jadaon MM, Alsayegh FA, Al-Sharrah SK. Smoking, von Willebrand factor and ADAMTS-13 in healthy males. Scand J Clin Lab Invest 2012; 72 (08) 614-618
• 222 Legendre P, Navarrete AM, Rayes J. et al. Mutations in the A3 domain of von Willebrand factor inducing combined qualitative and quantitative defects in the protein. Blood 2013; 121 (11) 2135-2143
• 223 Ni Y, Nesrallah J, Agnew M, Geske FJ, Favaloro EJ. Establishment and characterization of a new and stable collagen-binding assay for the assessment of von Willebrand factor activity. Int J Lab Hematol 2013; 35 (02) 170-176
• 224 Soares RP, Bydlowski SP, Nascimento NM, Thomaz AM, Bastos EN, Lopes AA. Plasmatic ADAMTS-13 metalloprotease and von Willebrand factor in children with cyanotic congenital heart disease. Braz J Med Biol Res 2013; 46 (04) 375-381
• 225 Soares RP, Bydlowski SP, Jatene MB, Hironaka JF, Lopes AA. Decreased plasma ADAMTS-13 activity as a predictor of postoperative bleeding in cyanotic congenital heart disease. Clinics (São Paulo) 2013; 68 (04) 531-536
• 226 Hugenholtz GC, Adelmeijer J, Meijers JC, Porte RJ, Stravitz RT, Lisman T. An unbalance between von Willebrand factor and ADAMTS13 in acute liver failure: implications for hemostasis and clinical outcome. Hepatology 2013; 58 (02) 752-761
• 227 Colombatti R, De Bon E, Bertomoro A. et al. Coagulation activation in children with sickle cell disease is associated with cerebral small vessel vasculopathy. PLoS One 2013; 8 (10) e78801
• 228 Hu J, Mondal NK, Sorensen EN. et al. Platelet glycoprotein Ibα ectodomain shedding and non-surgical bleeding in heart failure patients supported by continuous-flow left ventricular assist devices. J Heart Lung Transplant 2014; 33 (01) 71-79
• 229 Kraisin S, Palasuwan A, Popruk S, Nantakomol D. Reduced ADAMTS13 activity is associated with an ADAMTS13 SNP, fever and microparticles in a malaria-like model. Malar J 2014; 13: 3
• 230 Mazur P, Plicner D, Zdziarska J, Sadowski J, Undas A. Decreased von Willebrand factor ristocetin cofactor activity and increased ADAMTS13 antigen increase postoperative drainage after coronary artery bypass grafting. Eur J Cardiothorac Surg 2014; 45 (02) e26-e32
• 231 van Loon JE, Sonneveld MA, Praet SF, de Maat MP, Leebeek FW. Performance related factors are the main determinants of the von Willebrand factor response to exhaustive physical exercise. PLoS One 2014; 9 (03) e91687
• 232 Rau J, Rosenthal C, Langer E. et al. A calcium-containing electrolyte-balanced hydroxyethyl starch (HES) solution is associated with higher factor VIII activity than is a non-balanced HES solution, but does not affect von Willebrand factor function or thromboelastometric measurements–results of a model of in vitro haemodilution. Blood Transfus 2014; 12 (02) 260-268
• 233 Kajdácsi E, Jani PK, Csuka D. et al. Endothelial cell activation during edematous attacks of hereditary angioedema types I and II. J Allergy Clin Immunol 2014; 133 (06) 1686-1691
• 234 Goel A, Alagammai PL, Nair SC. et al. ADAMTS13 deficiency, despite well-compensated liver functions in patients with noncirrhotic portal hypertension. Indian J Gastroenterol 2014; 33 (04) 355-363
• 235 Mancini I, Valsecchi C, Lotta LA. et al. FRETS-VWF73 rather than CBA assay reflects ADAMTS13 proteolytic activity in acquired thrombotic thrombocytopenic purpura patients. Thromb Haemost 2014; 112 (02) 297-303
• 236 Quiroga T, Goycoolea M, Belmont S. et al. Quantitative impact of using different criteria for the laboratory diagnosis of type 1 von Willebrand disease. J Thromb Haemost 2014; 12 (08) 1238-1243
• 237 Waldow HC, Westhoff-Bleck M, Widera C, Templin C, von Depka M. Acquired von Willebrand syndrome in adult patients with congenital heart disease. Int J Cardiol 2014; 176 (03) 739-745
• 238 Loeffelbein F, Funk D, Nakamura L. et al. Shear-stress induced acquired von Willebrand syndrome in children with congenital heart disease. Interact Cardiovasc Thorac Surg 2014; 19 (06) 926-932
• 239 Kalbhenn J, Schmidt R, Nakamura L, Schelling J, Rosenfelder S, Zieger B. Early diagnosis of acquired von Willebrand syndrome (AVWS) is elementary for clinical practice in patients treated with ECMO therapy. J Atheroscler Thromb 2015; 22 (03) 265-271
• 240 Bürgin-Maunder CS, Brooks PR, Hitchen-Holmes D, Russell FD. Moderate dietary supplementation with omega-3 fatty acids does not impact plasma von Willebrand factor profile in mildly hypertensive subjects. BioMed Res Int 2015; 2015: 394871
• 241 Caspar T, Jesel L, Desprez D. et al. Effects of transcutaneous aortic valve implantation on aortic valve disease-related hemostatic disorders involving von Willebrand factor. Can J Cardiol 2015; 31 (06) 738-743
• 242 Hugenholtz GC, Ruitenbeek K, Adelmeijer J. et al. Development of a hyperactive primary hemostatic system during off-pump lung transplantation resulting from an unbalance between von Willebrand factor and its cleaving protease ADAMTS13. Am J Transplant 2015; 15 (07) 1958-1966
• 243 Ferhat-Hamida MY, Boukerb H, Hariti G. Contribution of the collagen binding activity (VWF:CB) in the range of tests for the diagnosis and classification of von Willebrand disease. Ann Biol Clin (Paris) 2015; 73 (04) 461-468
• 244 van Meegeren ME, Mancini TL, Schoormans SC. et al. Clinical phenotype in genetically confirmed von Willebrand disease type 2N patients reflects a haemophilia A phenotype. Haemophilia 2015; 21 (05) e375-e383
• 245 Bartoli CR, Kang J, Restle DJ. et al. Inhibition of ADAMTS-13 by doxycycline reduces von Willebrand factor degradation during supraphysiological shear stress: therapeutic implications for left ventricular assist device-associated bleeding. JACC Heart Fail 2015; 3 (11) 860-869
• 246 Periayah MH, Halim AS, Saad AZ, Yaacob NS, Karim FA. Report on von Willebrand disease in Malaysia. Open Access Maced J Med Sci 2016; 4 (01) 112-117
• 247 Qu L, Jiang M, Qiu W. et al. Assessment of the diagnostic value of plasma levels, activities, and their ratios of von Willebrand factor and ADAMTS13 in patients with cerebral infarction. Clin Appl Thromb Hemost 2016; 22 (03) 252-259
• 248 Feys HB, Van Aelst B, Devloo R, Vandekerckhove P, Compernolle V. The contribution of von Willebrand factor-GPIbα interactions to persistent aggregate formation in apheresis platelet concentrates. Vox Sang 2016; 110 (04) 344-351
• 249 Favaloro EJ, Mohammed S. Evaluation of a von Willebrand factor three test panel and chemiluminescent-based assay system for identification of, and therapy monitoring in, von Willebrand disease. Thromb Res 2016; 141: 202-211
• 250 Nagy EE, Varga-Fekete T, Puskas A. et al. High circulating osteoprotegerin levels are associated with non-zero blood groups. BMC Cardiovasc Disord 2016; 16 (01) 106
• 251 Muthiah K, Connor D, Ly K. et al. Longitudinal changes in hemostatic parameters and reduced pulsatility contribute to non-surgical bleeding in patients with centrifugal continuous-flow left ventricular assist devices. J Heart Lung Transplant 2016; 35 (06) 743-751
• 252 Sun CF, Liu GQ, Zhao X. et al. [ADAMTS13 level in prothrombotic status and its related factor analysis]. Zhongguo Shi Yan Xue Ye Xue Za Zhi 2016; 24 (04) 1125-1131
• 253 Sun CF, Zhao X, Han F. et al. [Changes of ADAMTS13 activity and TSP1 level in patients with hematologic malignancies]. Zhongguo Shi Yan Xue Ye Xue Za Zhi 2016; 24 (05) 1294-1298
• 254 de Maat MP, van Schie M, Kluft C, Leebeek FW, Meijer P. Biological variation of hemostasis variables in thrombosis and bleeding: consequences for performance specifications. Clin Chem 2016; 62 (12) 1639-1646
• 255 Favaloro EJ. Utility of the von Willebrand factor collagen binding assay in the diagnosis of von Willebrand disease. Am J Hematol 2017; 92 (01) 114-118
• 256 Ezigbo ED, Ukaejiofo EO, Nwagha TU. Molecular characterization of exon 28 of von Willebrand's factor gene in Nigerian population. Niger J Clin Pract 2017; 20 (02) 235-238
• 257 Zhang P, Yu ZQ, Zhang XH. et al. [Detection and analysis of plasma VWF levels in healthy Chinese by automatic standardized assay]. Zhonghua Xue Ye Xue Za Zhi 2017; 38 (02) 146-152
• 258 Heilmann C, Trummer G, Beyersdorf F. et al. Acquired Von Willebrand syndrome in patients on long-term support with HeartMate II. Eur J Cardiothorac Surg 2017; 51 (03) 587-590
• 259 Wang Z, Dou M, Du X. et al. Influences of ABO blood group, age and gender on plasma coagulation factor VIII, fibrinogen, von Willebrand factor and ADAMTS13 levels in a Chinese population. PeerJ 2017; 5: e3156
• 260 Frank RD, Lanzmich R, Haager PK, Budde U. Severe aortic valve stenosis. Clin Appl Thromb Hemost 2017; 23 (03) 229-234
• 261 Kumar M, Cao W, McDaniel JK. et al. Plasma ADAMTS13 activity and von Willebrand factor antigen and activity in patients with subarachnoid haemorrhage. Thromb Haemost 2017; 117 (04) 691-699
• 262 Cibor D, Owczarek D, Butenas S, Salapa K, Mach T, Undas A. Levels and activities of von Willebrand factor and metalloproteinase with thrombospondin type-1 motif, number 13 in inflammatory bowel diseases. World J Gastroenterol 2017; 23 (26) 4796-4805
• 263 Doruelo AL, Haberichter SL, Christopherson PA. et al. Clinical and laboratory phenotype variability in type 2M von Willebrand disease. J Thromb Haemost 2017; 15 (08) 1559-1566
• 264 Michiels JJ, Smejkal P, Penka M. et al. Diagnostic differentiation of von Willebrand disease types 1 and 2 by von Willebrand factor multimer analysis and DDAVP challenge test. Clin Appl Thromb Hemost 2017; 23 (06) 518-531
• 265 Chan CHH, Pieper IL, Robinson CR, Friedmann Y, Kanamarlapudi V, Thornton CA. Shear stress-induced total blood trauma in multiple species. Artif Organs 2017; 41 (10) 934-947
• 266 Lavin M, Aguila S, Schneppenheim S. et al. Novel insights into the clinical phenotype and pathophysiology underlying low VWF levels. Blood 2017; 130 (21) 2344-2353
• 267 Stufano F, Baronciani L, Mane-Padros D, Cozzi G, Faraudo S, Peyvandi F. A comparative evaluation of a new fully automated assay for von Willebrand factor collagen binding activity to an established method. Haemophilia 2018; 24 (01) 156-161
• 268 Geisen U, Brehm K, Trummer G. et al. Platelet secretion defects and acquired von Willebrand syndrome in patients with ventricular assist devices. J Am Heart Assoc 2018; 7 (02) e006519
• 269 Jousselme E, Jourdy Y, Rugeri L, Négrier C, Nougier C. Comparison of an automated chemiluminescent assay to a manual ELISA assay for determination of von Willebrand Factor collagen binding activity on VWD plasma patients previously diagnosed through molecular analysis of VWF. Int J Lab Hematol 2018; 40 (01) 77-83
• 270 Tan Y, Luan ZQ, Hao JB, Song D, Yu F, Zhao MH. Plasma ADAMTS-13 activity in proliferative lupus nephritis: a large cohort study from China. Lupus 2018; 27 (03) 389-398
• 271 Alharbi A, Hassan SB, Al-Momen AK. et al. Influence of ABO blood group on von Willebrand factor tests in healthy Saudi blood donors. Blood Coagul Fibrinolysis 2018; 29 (02) 211-215
• 272 Russell RT, McDaniel JK, Cao W. et al. Low plasma ADAMTS13 activity is associated with coagulopathy, endothelial cell damage and mortality after severe paediatric trauma. Thromb Haemost 2018; 118 (04) 676-687
• 273 Zayat R, Khattab MA, Grottke O. et al. Survival of HeartMate II patients despite cessation of anticoagulation - outcomes and hemostatic analysis. Circ J 2018; 82 (05) 1309-1318
• 274 Sherazi S, Kouides P, Francis C. et al. Prospective analysis of bleeding events in left ventricular assist device patients. Int J Artif Organs 2018; 41 (05) 269-276
• 275 Favaloro EJ, Bonar R, Hollestelle MJ. et al. Differential sensitivity of von Willebrand factor activity assays to reduced VWF molecular weight forms: a large international cross-laboratory study. Thromb Res 2018; 166: 96-105
• 276 Atiq F, Meijer K, Eikenboom J. et al; WiN Study Group. Comorbidities associated with higher von Willebrand factor (VWF) levels may explain the age-related increase of VWF in von Willebrand disease. Br J Haematol 2018; 182 (01) 93-105
• 277 Torkildsen L, Bishop MA, Barr JW, Pashmakova MB. Comparison of multiple thawing techniques on thaw time and stability of hemostatic proteins in canine plasma products. J Small Anim Pract 2018;
• 278 Lasom S, Komanasin N, Settasatian N, Settasatian C, Kukongviriyapan U, Intharapetch P. Association of a disintegrin and metalloproteinase with a thrombospondin type 1 motif member 13 polymorphisms with severity of coronary stenosis in type 2 diabetes mellitus. J Res Med Sci 2018; 23: 59
• 279 Palyu E, Harsfalvi J, Tornai T. et al. Major changes of von Willebrand factor multimer distribution in cirrhotic patients with stable disease or acute decompensation. Thromb Haemost 2018; 118 (08) 1397-1408
• 280 Oliveira LMM, Amorim MVA, Corsini CA, Neto CCA, Chaves DG. Standardization and comparison of nonautomated assays to measure the collagen binding activity of von Willebrand factor. Int J Lab Hematol 2018; 40 (05) 597-603
• 281 Pelland-Marcotte MC, Humpl T, James PD. et al. Idiopathic pulmonary arterial hypertension - a unrecognized cause of high-shear high-flow haemostatic defects (otherwise referred to as acquired von Willebrand syndrome) in children. Br J Haematol 2018; 183 (02) 267-275
• 282 Icheva V, Nowak-Machen M, Budde U. et al. Acquired von Willebrand syndrome in congenital heart disease surgery: results from an observational case-series. J Thromb Haemost 2018; 16 (11) 2150-2158
• 283 Staley EM, Cao W, Pham HP. et al. Clinical factors and biomarkers predict outcome in patients with immune-mediated thrombotic thrombocytopenic purpura. Haematologica 2019; 104 (01) 166-175
• 284 Slobodianuk TL, Kochelek C, Foeckler J, Kalloway S, Weiler H, Flood VH. Defective collagen binding and increased bleeding in a murine model of von Willebrand disease affecting collagen IV binding. J Thromb Haemost 2019; 17 (01) 63-71
• 285 Moonla C, Akkawat B, Kittikalayawong Y. et al. Bleeding symptoms and von Willebrand factor levels: 30-year experience in a tertiary care center. Clin Appl Thromb Hemost 2019; 25: 1076029619866916
• 286 Colling ME, Friedman KD, Dzik WH. In vitro assessment of von Willebrand factor in cryoprecipitate, antihemophilic factor/VWF complex (human), and recombinant von Willebrand factor. Clin Appl Thromb Hemost 2019; 25: 1076029619873976
• 287 Pechmann A, Wellmann S, Stoecklin B, Krüger M, Zieger B. Increased von Willebrand factor parameters in children with febrile seizures. PLoS One 2019; 14 (01) e0210004
• 288 Kumar MA, Cao W, Pham HP. et al. Relative deficiency of plasma a disintegrin and metalloprotease with thrombospondin type 1 repeats 13 activity and elevation of human neutrophil peptides in patients with traumatic brain injury. J Neurotrauma 2019; 36 (02) 222-229
• 289 Yan B, He Y, Lu SQ. et al. [Establishment of flow cytometric immunobead array for detecting plasma von Willebrand factor activity and its clinical application in the prognosis of ischemic stroke]. Zhongguo Shi Yan Xue Ye Xue Za Zhi 2019; 27 (01) 208-214
• 290 Kubicki R, Stiller B, Kroll J. et al. Acquired von Willebrand syndrome in paediatric patients during mechanical circulatory support. Eur J Cardiothorac Surg 2019; 55 (06) 1194-1201
• 291 Coghill PA, Kanchi S, Azartash-Namin ZJ, Long JW, Snyder TA. Benchtop von Willebrand factor testing: comparison of commercially available ventricular assist devices and evaluation of variables for a standardized test method. ASAIO J 2019; 65 (05) 481-488
• 292 Klaeske K, Dieterlen MT, Scholz U. et al. Acquired von Willebrand factor deficiency is reduced in HeartMate 3 patients†. Eur J Cardiothorac Surg 2019; 56 (03) 444-450
• 293 McBride D, Jepson RE, Cortellini S, Chan DL. Primary hemostatic function in dogs with acute kidney injury. J Vet Intern Med 2019; 33 (05) 2029-2036
• 294 Radley G, Pieper IL, Robinson CR. et al. In vitro benchmarking study of ventricular assist devices in current clinical use. J Card Fail 2020; 26 (01) 70-79
• 295 Jakob A, Schachinger E, Klau S. et al. Von Willebrand factor parameters as potential biomarkers for disease activity and coronary artery lesion in patients with Kawasaki disease. Eur J Pediatr 2020; 179 (03) 377-384
• 296 Stufano F, Baronciani L, Bucciarelli P. et al. Evaluation of a fully automated von Willebrand factor assay panel for the diagnosis of von Willebrand disease. Haemophilia 2020; 26 (02) 298-305
• 297 Harsfalvi J, Molnar Z, Csanyi MC. et al. Long-lasting prothrombotic state implied by changes of plasma von Willebrand factor parameters after radical prostatectomy for prostate malignancy. Urol Oncol 2020; 38 (04) 191-197
• 298 Schlagenhauf A, Kalbhenn J, Geisen U, Beyersdorf F, Zieger B. Acquired von Willebrand syndrome and platelet function defects during extracorporeal life support (mechanical circulatory support). Hamostaseologie 2020; 40 (02) 221-225
• 299 Sacco M, Lancellotti S, Ferrarese M. et al. Noncanonical type 2B von Willebrand disease associated with mutations in the VWF D'D3 and D4 domains. Blood Adv 2020; 4 (14) 3405-3415
• 300 Ladeira VS, Barbosa AR, Oliveira MM. et al. ADAMTS-13-VWF axis in sickle cell disease patients. Ann Hematol 2021; 100 (02) 375-382
• 301 Ranger A, Gaspar M, Elkhatteb A. et al. The heparin-von Willebrand factor interaction and conventional tests of haemostasis - the challenges in predicting bleeding in cardiopulmonary bypass. Br J Haematol 2021; 192 (06) 1073-1081
• 302 Favaloro EJ, Henry BM, Lippi G. Increased VWF and decreased ADAMTS-13 in COVID-19: creating a milieu for (micro)thrombosis. Semin Thromb Hemost 2021; 47 (04) 400-418
• 303 Philippe A, Gendron N, Bory O. et al. Von Willebrand factor collagen-binding capacity predicts in-hospital mortality in COVID-19 patients: insight from VWF/ADAMTS13 ratio imbalance. Angiogenesis 2021; 24 (03) 407-411
• 304 Abdulrehman J, Ziemba YC, Hsu P. et al. Diagnosis of von Willebrand disease: an assessment of the quality of testing in North American laboratories. Haemophilia 2021; 27 (06) e713-e720
• 305 Thangaraju K, Katneni U, Akpan IJ. et al. The impact of age and BMI on the VWF/ADAMTS13 axis and simultaneous thrombin and plasmin generation in hospitalized COVID-19 patients. Front Med (Lausanne) 2022; 8: 817305
• 306 Sinkovits G, Réti M, Müller V. et al. Associations between the von Willebrand factor-ADAMTS13 axis, complement activation, and COVID-19 severity and mortality. Thromb Haemost 2022; 122 (02) 240-256
• 307 Fels S, Boeckelmann D, Glonnegger H, Büchsel M, Zieger B. Novel likely pathogenic variant in the A3 domain of von Willebrand factor leading to a collagen-binding defect. Hamostaseologie 2023; 43 (02) 122-125
• 308 Lapić I, Radić Antolic M, Dejanović Bekić S. et al. Reevaluation of von Willebrand disease diagnosis in a Croatian paediatric cohort combining bleeding scores, phenotypic laboratory assays and next generation sequencing: a pilot study. Biochem Med (Zagreb) 2022; 32 (01) 010707
• 309 Nagy EE, Puskás A, Kelemen P. et al. Elevated serum cystatin C and decreased cathepsin S/cystatin C ratio are associated with severe peripheral arterial disease and polyvascular involvement. Diagnostics (Basel) 2022; 12 (04) 833
• 310 Lapić I, Radić Antolic M, Boban A, Coen Herak D, Rogić D, Zadro R. Next-generation sequencing of von Willebrand factor and coagulation factor VIII genes: a cross-sectional study in Croatian adult patients diagnosed with von Willebrand disease. Croat Med J 2022; 63 (02) 166-175
• 311 Bowyer A, Brown P, Hopkins B. et al. Von Willebrand factor assays in patients with acquired immune thrombotic thrombocytopenia purpura treated with caplacizumab. Br J Haematol 2022; 197 (03) 349-358
• 312 Gowani F, Phillips B, Leveque C. et al. Recurrent gastrointestinal bleeding in a middle-aged man. Lab Med 2022; 53 (04) e91-e94
• 313 Ziemba YC, Abdulrehman J, Hollestelle MJ. et al. Diagnostic testing for von Willebrand disease: trends and insights from North American laboratories over the last decade. Semin Thromb Hemost 2022; 48 (06) 700-710
• 314 Laporte P, Tuffigo M, Ryman A. et al. HemosIL VWF:GPIbR assay has a greater sensitivity than VWF:RCo technique to detect acquired von Willebrand syndrome in myeloproliferative neoplasms. Thromb Haemost 2022; 122 (10) 1673-1682
• 315 Gritsch H, Schrenk G, Weinhappl N, Mellgård B, Ewenstein B, Turecek PL. Structure and function of recombinant versus plasma-derived von Willebrand factor and impact on multimer pharmacokinetics in von Willebrand disease. J Blood Med 2022; 13: 649-662
• 316 Lapić I, Radić Antolic M, Rogić D. et al. Type 1 von Willebrand disease in a pediatric patient caused by a novel heterozygous deletion of exons 1 to 6 of the von Willebrand factor gene: a case report. Lab Med 2022; lmac138
• 317 Icheva V, Budde U, Magunia H. et al. Acquired von Willebrand syndrome is common in infants with systemic-to-pulmonary shunts: retrospective case-series. Front Pediatr 2022; 10: 1040128
• 318 Kalbhenn J, Pooth JS, Trummer G, Kranzhöfer D, Schlagenhauf A, Zieger B. Pervasive platelet secretion defects in patients with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2). Cells 2023; 12 (01) 193
• 319 Icheva V, Ebert J, Budde U. et al. Perioperative diagnosis and impact of acquired von Willebrand syndrome in infants with congenital heart disease. Blood 2023; 141 (01) 102-110
• 320 Van Den Helm S, Letunica N, Barton R. et al. Changes in von Willebrand factor multimers, concentration, and function during pediatric extracorporeal membrane oxygenation. Pediatr Crit Care Med 2023; 24 (04) 268-276
• 321 Scott DM, Griffin B, Pepper DS, Barnes MJ. The binding of purified factor VIII/von Willebrand factor to collagens of differing type and form. Thromb Res 1981; 24 (5-6): 467-472
• 322 Santoro SA. Preferential binding of high molecular weight forms of von Willebrand factor to fibrillar collagen. Biochim Biophys Acta 1983; 756 (01) 123-126
• 323 Sixma JJ, Sakariassen KS, Stel HV. et al. Functional domains on von Willebrand factor. Recognition of discrete tryptic fragments by monoclonal antibodies that inhibit interaction of von Willebrand factor with platelets and with collagen. J Clin Invest 1984; 74 (03) 736-744
• 324 Laffan MA, Lester W, O'Donnell JS. et al. The diagnosis and management of von Willebrand disease: a United Kingdom Haemophilia Centre Doctors Organization guideline approved by the British Committee for Standards in Haematology. Br J Haematol 2014; 167 (04) 453-465
• 325 Favaloro EJ, Mohammed S, Chapman K. et al. A multicenter laboratory assessment of a new automated chemiluminescent assay for ADAMTS13 activity. J Thromb Haemost 2021; 19 (02) 417-428
• 326 Favaloro EJ, Pasalic L, Henry B, Lippi G. Laboratory testing for ADAMTS13: utility for TTP diagnosis/exclusion and beyond. Am J Hematol 2021; 96 (08) 1049-1055
• 327 Nichols WL, Hultin MB, James AH. et al. von Willebrand disease (VWD): evidence-based diagnosis and management guidelines, the National Heart, Lung, and Blood Institute (NHLBI) Expert Panel report (USA). Haemophilia 2008; 14 (02) 171-232