Von Willebrand disease – comparison of two methods of vWF multimer analysis
Authors:
I. Škorňová; J. Staško; P. Hollý; M. Dobrotová; T. Šimurda; J. Sokol; J. Žolková; I. Plameňová; P. Kubisz
Authors‘ workplace:
Jesseniova lekárska fakulta v Martine a Univerzitná nemocnica v Martine, Slovenská republika
; Národné centrum hemostázy a trombózy, Klinika hematológie a transfúziológie, Univerzita Komenského v Bratislave
Published in:
Transfuze Hematol. dnes,27, 2021, No. 1, p. 42-50.
Category:
Original Papers
doi:
https://doi.org/10.48095/cctahd202142
Overview
Von Willebrand factor (vWF) is a multimeric protein that plays a principal role in two key functions in hemostasis. It participates in the interaction between the platelets and the subendothelium at the site of the vascular damage and if is also important in the interaction between the platelets. vWF is complexed with factor VIII (1: 1) to protect it from proteolysis by activated Protein C. vWF is a protein that is assembled from identical subunits into linear strings of varying size referred to as multimers. These multimers can be > 20 million daltons in mass and > 2 micrometers in length. Clinically significant and most effective in interacting with collagen and thrombocyte receptors are high molecular weight multimers. In our article we present comparing of two available methods of von Willebrand factor multimer analysis – the classical manual method and the new semi-automatic method of the Hydrasys instrument. The new method uses a prepared 2% agarose gel, the results are available within 6 h, the method is standardized and reproducible. However, abnormalities of multimers must be further investigated in more detail using the classical-manual method with different concentrations of agarose gels.
Keywords:
von Willebrand factor – von Willebrand factor multimers – von Willebrand disease – electorforesis – ADAMTS13
Sources
1. Nichols WL Jr, Hultin MB, James AH, et al. The diagnosis, evaluation, and management of vWD, the National heart, lung, and blood institute (NHLBI), NIH Publication No. 08-5832, 2007; 1–126.
2. Sadler JE, Budde U, Eikenboom JC, 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: 2103–2114.
3. Montgomery RR, Haberichter SL. von Willebrand factor structure and function. In: Federici AB et al. von Willebrand disease. 1st Ed. Wiley-Blackwell, Oxford, UK 2011; 30–48.
4. Enayat MS, Hill FG. Analysis of the complexity of the multimeric structure of FVIII related antigen/von Willebrand protein using a modified electrophoretic technique. J Clin Pathol. 1983; 36: 915–919.
5. Ruggeri ZM, Zimmerman TS. Classification of variant vWD subtypes by analysis of functional characteristics and multimeric composition of FVIII/von Willebrand factor. Ann NY Acad Sci. 1981; 370: 205–209.
6. Veyradier A, Jenkins ChSP, Fressinaud E, et al. Acquired von Willebrand syndrome: from pathophysiology to management. Thromb Haemost. 2000; 84: 175–182.
7. Eikenboom JC, Van der Meer FJM, Briet E. Acquired von Willebrands disease due to excessive fibrinolysis. Br J Haematol. 1992; 81: 618–620.
8. Curnow J, Pasalic L, Favaloro EJ. Treatment of von Willebrand disease. Semin Thromb Hemost. 2016; 42: 133–146.
9. Favaloro EJ. Von Willebrand disease: local diagnosis and management of a globally distributed bleeding disorder. Semin Thromb Hemost. 2011; 37: 440–455.
10. Flood VH. Perils, problems, and progress in laboratory diagnosis of von Willebrand disease. Semin Thromb Hemost. 2014; 40: 41–48.
11. Favaloro EJ. Rethinking the diagnosis of von Willebrand disease. Thromb Res. 2011; 127 (2): 17–21.
12. Federici AB, Castaman G, Mannucci PM, et al. Guidelines for the diagnosis and management of von Willebrand disease in Italy. Haemophilia. 2002; 8: 607–621.
13. Federici AB, Mannucci PM. Diagnosis and management of von Willebrand disease. Haemophilia. 1999; 5 (2): 28–37.
14. Škorňová I, Staško J, Hollý P, et al. Analýza multimérov von Willebrandovho faktora pri von Willebrandovej chorobe. Vask Med. 2014; 6 (1): 32–34.
15. Oliver S, Vanniasinkam T, Mohammed S, et al. Semi-automated von Willebrand factor multimer assay for von Willebrand disease: Further validation, benefits and limitations. Int J Lab Hematol. 2019; 41 (6): 762–771.
16. Crist RA, Heikal NM, Rodgers GM et al. Evaluation of a new commercial method for von Willebrand factor multimeric analysis. Int J Lab Hem. 2018; 40: 586–591.
17. Bowyer AE, Goodfellow KJ, Seidel H, et al. Evaluation of a semi-automated von Willebrand factor multimer assay, the Hydragel 5 von Willebrand multimer, by two European centers. Res Pract Thromb Haemost. 2018; 2 (4): 790–799.
18. Seidel H, Westhofen P, Bautista H, et al. Clinical evaluation of the sebia hydragel von Willebrand factor assay in comparison to electrophoresis and blotting based multimer analysis, Res Pract Thromb Haemost 2017; 1: 717–718.
19. Bowyer AE, Goodfellow KJ, Nouadje G, et al. Performances of the Hydragel 5 von Willebrand multimers – a new within-day von Willebrand factor (VWF) multimer screening method evaluation of a new commercial method for von Willebrand factor multimeric analysis. Res Pract Thromb Haemost. 2017; 1: 494.
20. Vasse M, Francois D, Ligneel T, et al. Interest of the new rapid test “Hydragel 5 von Willebrand multimers” for the analysis of von Willebrand multimers. Int J Lab Hem. 2016; 38: 113.
21. Vasse M, Francois D, Bironien R, et al. Clinical interest of same-day resluts assay,,Hydragel 5 von Willebrand multimers“ for therapeutic decision. Clin Chem Lab Med. 2017; 55: 721.
22. Lemaitre A, Nouadje G, Beaulieu Bautista H, et al. Clinical evaluation of the Hydragel 5 von Willebrand multimers of Sebia. Clin Chem Lab Med. 2017; 55: 706.
23. Goodfellow KJ, Bowyer AE, Nouadje G, et al. Sebia Hydragel 5 von Willebrand multimers – a new and rapid von Willebrand factor (VWF) multimer screening method to aid subtyping of type 2 von Willebrand disease (VWD). Clin Chem Lab Med. 2017; 55: 721.
Labels
Haematology Internal medicine Clinical oncologyArticle was published in
Transfusion and Haematology Today
2021 Issue 1
Most read in this issue
- Heparin induced thrombocytopenia
- Blood cell analysers – signifi cance of a dimorphic red blood cell population
- Recommendations for chronic lymphocytic leukaemia diagnosis and therapy 2021
- Novel molecules used in the targeted treatment of acute myeloid leukaemia – adverse reactions, treatment complications and signifi cant drug interactions