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Guidelines for the evaluation of intravenous desmopressin and von Willebrand factor/factor VIII concentrate in the treatment and prophylaxis of bleedings in von Willebrand disease types 1, 2, and 3.
Semin Thromb Hemost 2006; 32(6):636-45ST

Abstract

The current standard for the diagnosis and management of patients with congenital von Willebrand disease (vWD) includes bleeding times (BTs), PFA-100 closure time (PFA-CT), factor (F) VIII:coagulant activity (C), vWF:antigen (Ag), vWF:ristocetin cofactor activity (RCo), a sensitive vWF:collagen-binding activity (CB), ristocetin-induced platelet aggregation (RIPA), analysis of vWF multimers in low- and high-resolution agarose gels, and the response to desmopressin. Guidelines and recommendations for prophylaxis and treatment of bleedings in vWD patients with vWF/FVIII concentrates should be derived from analysis of the content of these concentrates and from pharmacokinetic studies in different types of vWD patients with severe type 1, 2, or 3 vWD. The vWF/FVIII concentrates should be characterized by labeling with FVIII:C, vWF:RCo, vWF:CB, and vWF multimeric pattern, which will determine their predicted efficacy and safety in prospective management studies. Because the bleeding tendency is moderate in type 2 and severe in type 3 vWD, and because the FVIII:C levels are subnormal in type 2 and very low in type 3 vWD patients, new guidelines using vWF:RCo unit dosing for the prophylaxis and treatment of bleeding episodes are proposed. Such guidelines should be stratified for the severity of bleeding, the type of surgery (either minor or major), and also for the severity and type of vWD (i.e., either type 2 or 3 vWD).

Authors+Show Affiliations

Hemostasis Thrombosis Research, Department of Hematology, University Hospital Antwerp, Antwerp, Belgium. postbus@goodheartcenter.demon.nlNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article
Review

Language

eng

PubMed ID

16977574

Citation

Michiels, Jan J., et al. "Guidelines for the Evaluation of Intravenous Desmopressin and Von Willebrand Factor/factor VIII Concentrate in the Treatment and Prophylaxis of Bleedings in Von Willebrand Disease Types 1, 2, and 3." Seminars in Thrombosis and Hemostasis, vol. 32, no. 6, 2006, pp. 636-45.
Michiels JJ, Gadisseur A, van der Planken M, et al. Guidelines for the evaluation of intravenous desmopressin and von Willebrand factor/factor VIII concentrate in the treatment and prophylaxis of bleedings in von Willebrand disease types 1, 2, and 3. Semin Thromb Hemost. 2006;32(6):636-45.
Michiels, J. J., Gadisseur, A., van der Planken, M., Schroyens, W., van de Velden, A., & Berneman, Z. (2006). Guidelines for the evaluation of intravenous desmopressin and von Willebrand factor/factor VIII concentrate in the treatment and prophylaxis of bleedings in von Willebrand disease types 1, 2, and 3. Seminars in Thrombosis and Hemostasis, 32(6), pp. 636-45.
Michiels JJ, et al. Guidelines for the Evaluation of Intravenous Desmopressin and Von Willebrand Factor/factor VIII Concentrate in the Treatment and Prophylaxis of Bleedings in Von Willebrand Disease Types 1, 2, and 3. Semin Thromb Hemost. 2006;32(6):636-45. PubMed PMID: 16977574.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Guidelines for the evaluation of intravenous desmopressin and von Willebrand factor/factor VIII concentrate in the treatment and prophylaxis of bleedings in von Willebrand disease types 1, 2, and 3. AU - Michiels,Jan J, AU - Gadisseur,Alain, AU - van der Planken,Marc, AU - Schroyens,Wilfried, AU - van de Velden,Ann, AU - Berneman,Zwi, PY - 2006/9/16/pubmed PY - 2007/1/4/medline PY - 2006/9/16/entrez SP - 636 EP - 45 JF - Seminars in thrombosis and hemostasis JO - Semin. Thromb. Hemost. VL - 32 IS - 6 N2 - The current standard for the diagnosis and management of patients with congenital von Willebrand disease (vWD) includes bleeding times (BTs), PFA-100 closure time (PFA-CT), factor (F) VIII:coagulant activity (C), vWF:antigen (Ag), vWF:ristocetin cofactor activity (RCo), a sensitive vWF:collagen-binding activity (CB), ristocetin-induced platelet aggregation (RIPA), analysis of vWF multimers in low- and high-resolution agarose gels, and the response to desmopressin. Guidelines and recommendations for prophylaxis and treatment of bleedings in vWD patients with vWF/FVIII concentrates should be derived from analysis of the content of these concentrates and from pharmacokinetic studies in different types of vWD patients with severe type 1, 2, or 3 vWD. The vWF/FVIII concentrates should be characterized by labeling with FVIII:C, vWF:RCo, vWF:CB, and vWF multimeric pattern, which will determine their predicted efficacy and safety in prospective management studies. Because the bleeding tendency is moderate in type 2 and severe in type 3 vWD, and because the FVIII:C levels are subnormal in type 2 and very low in type 3 vWD patients, new guidelines using vWF:RCo unit dosing for the prophylaxis and treatment of bleeding episodes are proposed. Such guidelines should be stratified for the severity of bleeding, the type of surgery (either minor or major), and also for the severity and type of vWD (i.e., either type 2 or 3 vWD). SN - 0094-6176 UR - https://www.unboundmedicine.com/medline/citation/16977574/Guidelines_for_the_evaluation_of_intravenous_desmopressin_and_von_Willebrand_factor/factor_VIII_concentrate_in_the_treatment_and_prophylaxis_of_bleedings_in_von_Willebrand_disease_types_1_2_and_3_ L2 - http://www.thieme-connect.com/DOI/DOI?10.1055/s-2006-949669 DB - PRIME DP - Unbound Medicine ER -