Thrombosis Research
Volume 120 , Pages S21-S25 , 2007

Treatment of urgent bleeding in von Willebrand disease

  • Joan Cox Gill

      Affiliations

    • Corresponding Author InformationTel.: +1 414 257 2424; fax: +1 414 937 6580.

References 

  1. Gill JC. Diagnosis and treatment of von Willebrand disease. Hematol Oncol Clin North Am. 2004;18:1277–1299
  2. 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;71:520–525
  3. Sadler JE. New concepts in von Willebrand disease. Annu Rev Med. 2005;56:173–191
  4. Sadler JE, Budde U, Eikenboom JC, Favaloro EJ, Hill FG, Holmberg L, et al. 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
  5. Thompson AR, Gill JC, Ewenstein BM, Mueller-Velten G, Schwarz BA Humate-P Study Group. Successful treatment for patients with von Willebrand disease undergoing urgent surgery using factor VIII/vWF concentrate (Humate-P). Haemophilia. 2004;10:42–51
  6. Gill JC, Ewenstein BM, Thompson AR, Mueller-Velten G, Schwartz BA, Humate-P Study Group . Successful treatment of urgent bleeding in von Willebrand disease with factor VIII/vWF concentrate (Humate-P): use of the ristocetin cofactor assay (vWF:RCo) to measure potency and to guide therapy. Haemophilia. 2003;9:688–695
  7. Mannucci PM, Chediak J, Hanna W, Byrnes J, Marlies L, Ewenstein BM, et al. Treatment of von Willebrand disease with a high-purity factor VIII/von Willebrand factor concentrate: a prospective, multicenter study. Blood. 2002;99:450–456
  8. Lillicrap D, Poon MC, Walker I, Xie F, Schwartz BA Association of Hemophilia Clinic Directors of Canada. Efficacy and safety of the factor VIII/von Willebrand factor concentrate, Haemate P/Humate-P: ristocetin cofactor unit dosing in patients with von Willebrand disease. Thromb Haemost. 2002;87:224–230
  9. Srivastava A, Mathews V, Bhurani D, Baidya S, Chandy M. Successful surgical hemostasis in patients with von Willebrand disease following infusion of KoateDVI. Thromb Haemost. 2002;87:541–543
  10. Mannucci PM. Desmopressin (DDAVP) in the treatment of bleeding disorders: the first 20 years. Blood. 1997;90:2515–2521
  11. Gill JC, Ottum M, Schwartz B. Evaluation of high concentration intranasal and intravenous desmopressin in pediatric patients with mild hemophilia A or mild-to-moderate type 1 von Willebrand disease. J Pediatr. 2002;140:595–599
  12. Mannucci PM, Canciani MT, Rota L, Donovan BS. Response of factor VIII/von Willebrand factor to DDAVP in healthy subjects and patients with haemophilia A and von Willebrand's disease. Br J Haematol. 1981;47:283–293
  13. Lethagen S, Harris AS, Sjorin E, Nilsson IM. Intranasal and intravenous administration of desmopressin: effect on FVIII/vWF, pharmacokinetics and reproducibility. Thromb Haemost. 1987;58:1033–1036
  14. de la Fuente B, Kasper CK, Rickles FR, Hoyer LW. Response of patients with mild and moderate hemophilia A and von Willebrand's disease to treatment with desmopressin. Ann Intern Med. 1985;103:6–14
  15. Federici AB, Mazurier C, Berntorp E, Lee CA, Scharrer I, Goudemand J, et al. Biologic response to desmopressin in patients with severe type 1 and type 2 von Willebrand disease: results of a multicenter European study. Blood. 2004;103:2032–2038
  16. Mazurier C, Gaucher C, Jorieux S, Goudemand M. Biological effect of desmopressin in eight patients with type 2N (‘Normandy’) von Willebrand disease. Collaborative Group. Br J Haematol. 1994;88:849–854
  17. Mannucci PM. Treatment of von Willebrand's disease. N Engl J Med. 2004;351:683–694
  18. Mannucci PM, Duga S, Peyvandi F. Recessively inherited coagulation disorders. Blood. 2004;104:1243–1252
  19. Franchini M, Rossetti G, Tagliaferri A, Pattacini C, Pozzoli D, Lippi G, et al. Efficacy and safety of factor VIII/von Willebrand factor concentrate (Haemate-P®) in preventing bleeding during surgery or invasive procedures in patients with von Willebrand's disease. Haematologica. 2003;88:1279–1283
  20. Mannucci PM, Cancian MT, Rota L, Donovan BS. Response of factor VIII/von Willebrand factor to DDAVP in healthy subjects and patients with haemophilia A and von Willebrand's disease. Br J Haematol. 1981;47:283–293
  21. Casonato A, Steffan A, Pontara E, Zucchetto A, Rossi C, De Marco L, et al. Post-DDAVP thrombocytopenia in type 2B von Willebrand disease is not associated with platelet consumption: failure to demonstrate glycocalicin increase or platelet activation. Thromb Haemost. 1999;81:224–228
  22. Smith TJ, Gill JC, Ambruso DR, Hathaway WE. Hyponatremia and seizures in young children given DDAVP. Am J Hematol. 1989;31:199–202
  23. Bertholini DM, Butler CS. Severe hyponatraemia secondary to desmopressin therapy in von Willebrand's disease. Anaesth Intensive Care. 2000;28:199–201
  24. Budde U, Metzner HJ, Müller H-G. Comparative analysis and classification of von Willebrand factor/factor VIII concentrates: impact on treatment of patients with von Willebrand disease. Semin Thromb Hemost. 2006;32:626–635
  25. Sindet-Pedersen S, Ramstrom G, Bernvil S, Blomback M. Hemostatic effect of tranexamic acid mouthwash in anticoagulant-treated patients undergoing oral surgery. N Engl J Med. 1989;320:840–843

☆ Conflict of interest: The author receives research support from CSL-Behring Foundation, and honoraria for speaking and consulting from CSL Behring.

☆☆ Role of the funding source: The funding source, CSL Behring, organised the symposium and supported the writing assistance for this review. CSL Behring had no role in the analysis of the medical literature and development of the presentation that provided the basis for this review.

PII: S0049-3848(07)00116-8

doi: 10.1016/j.thromres.2007.03.013

Thrombosis Research
Volume 120 , Pages S21-S25 , 2007

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