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Managing patients with von Willebrand disease type 1, 2 and 3 with desmopressin and von Willebrand factor-factor VIII concentrate in surgical settings.
Acta Haematol. 2009; 121(2-3):167-76.AH

Abstract

Guidelines and recommendations for the acute and prophylactic treatment of bleeding in von Willebrand disease (VWD) patients with von Willebrand factor (VWF)/factor VIII (FVIII) concentrates should be based on the analysis of the content of VWF/FVIII concentrates and on pharmacokinetic studies in patients with different severity of VWD (type 1, type 2 or type 3). The VW/FVIII concentrates should be assessed using the parameters FVIII:coagulant activity (C), VWF:ristocetin cofactor activity (RCo), VWF:collagen binding and VWF multimeric patterns for the presence of large multimers to determine their predicted efficacy and safety in prospective management studies. As the bleeding tendency is moderate in VWD type 2 and severe in type 3 and because the FVIII:C levels are subnormal in type 2 but very low in type 3 VWD patients, new guidelines using VWF:RCo unit dosing for the acute and prophylactic treatment of bleeding episodes are proposed. Such guidelines should be stratified for the severity of bleeding, the type of surgery (minor or major) and also for the bleeding score in either VWD type 1, 2 or 3.

Authors+Show Affiliations

Hemostasis and Thrombosis Research Center, Antwerp University Hospital, Wilrijkstraat 10, Edegem, Belgium. postbus@goodheartcenter.demon.nlNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article
Review

Language

eng

PubMed ID

19506363

Citation

Michiels, Jan Jacques, et al. "Managing Patients With Von Willebrand Disease Type 1, 2 and 3 With Desmopressin and Von Willebrand Factor-factor VIII Concentrate in Surgical Settings." Acta Haematologica, vol. 121, no. 2-3, 2009, pp. 167-76.
Michiels JJ, van Vliet HH, Berneman Z, et al. Managing patients with von Willebrand disease type 1, 2 and 3 with desmopressin and von Willebrand factor-factor VIII concentrate in surgical settings. Acta Haematol. 2009;121(2-3):167-76.
Michiels, J. J., van Vliet, H. H., Berneman, Z., Schroyens, W., & Gadisseur, A. (2009). Managing patients with von Willebrand disease type 1, 2 and 3 with desmopressin and von Willebrand factor-factor VIII concentrate in surgical settings. Acta Haematologica, 121(2-3), 167-76. https://doi.org/10.1159/000214857
Michiels JJ, et al. Managing Patients With Von Willebrand Disease Type 1, 2 and 3 With Desmopressin and Von Willebrand Factor-factor VIII Concentrate in Surgical Settings. Acta Haematol. 2009;121(2-3):167-76. PubMed PMID: 19506363.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Managing patients with von Willebrand disease type 1, 2 and 3 with desmopressin and von Willebrand factor-factor VIII concentrate in surgical settings. AU - Michiels,Jan Jacques, AU - van Vliet,Huub H D M, AU - Berneman,Zwi, AU - Schroyens,Wilfried, AU - Gadisseur,Alain, Y1 - 2009/06/08/ PY - 2009/6/10/entrez PY - 2009/6/10/pubmed PY - 2009/7/7/medline SP - 167 EP - 76 JF - Acta haematologica JO - Acta Haematol. VL - 121 IS - 2-3 N2 - Guidelines and recommendations for the acute and prophylactic treatment of bleeding in von Willebrand disease (VWD) patients with von Willebrand factor (VWF)/factor VIII (FVIII) concentrates should be based on the analysis of the content of VWF/FVIII concentrates and on pharmacokinetic studies in patients with different severity of VWD (type 1, type 2 or type 3). The VW/FVIII concentrates should be assessed using the parameters FVIII:coagulant activity (C), VWF:ristocetin cofactor activity (RCo), VWF:collagen binding and VWF multimeric patterns for the presence of large multimers to determine their predicted efficacy and safety in prospective management studies. As the bleeding tendency is moderate in VWD type 2 and severe in type 3 and because the FVIII:C levels are subnormal in type 2 but very low in type 3 VWD patients, new guidelines using VWF:RCo unit dosing for the acute and prophylactic treatment of bleeding episodes are proposed. Such guidelines should be stratified for the severity of bleeding, the type of surgery (minor or major) and also for the bleeding score in either VWD type 1, 2 or 3. SN - 1421-9662 UR - https://www.unboundmedicine.com/medline/citation/19506363/Managing_patients_with_von_Willebrand_disease_type_1_2_and_3_with_desmopressin_and_von_Willebrand_factor_factor_VIII_concentrate_in_surgical_settings_ L2 - https://www.karger.com?DOI=10.1159/000214857 DB - PRIME DP - Unbound Medicine ER -