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PFA-100 monitoring of von Willebrand factor (VWF) responses to desmopressin (DDAVP) and factor VIII/VWF concentrate substitution in von Willebrand disease type 1 and 2.
Thromb Haemost 2008; 100(3):462-8TH

Abstract

Dose-response relationship was studied between PFA-100 closure times (PFA CTs) and factor (F)VIII-von Willebrand factor (VWF) parameters in patients with von Willebrand disease (VWD) type 1 and type 2 before and after treatment with DDAVP (n=84) or FVIII/VWF concentrate (n=38). DDAVP treatment of patients with VWD type 1 normalised the PFA CTs by increasing VWF levels to normal. Of the 14 patients with VWD type 2, PFA CTs did not normalize in eight. Haemate-P substitution in patients with VWD type 1 induced a less favourable response as compared to DDAVP, because PFA CTs did not correct in all patients. Of 12 patients with VWD type 2 treated with Haemate-P, six showed a correction of PFA CTs (<250 sec), which correlated with the normalisation of the VWF CB/Ag ratio. In-vitro studies were performed by using whole blood of patients with VWD and adding various amounts of FVIII/VWF concentrate. Addition of Haemate-P induced an increase of the VWF CB/Ag ratio from 0.30 to 0.70 in blood of patients with VWD type 2 with correction of the PFA CTs. Immunate did not result in an increase of VWF CB/Ag ratio in blood of VWD type 2 patients, and the PFA CTs remained prolonged. We conclude that PFA-100 might be an adequate instrument not only for diagnosis but also for monitoring of DDAVP responses and FVIII/VWF substitution of patients with VWD type 1 and 2, but this is dependent upon the type of VWD and the concentrate used.

Authors+Show Affiliations

Department of Haematology, Erasmus MC, PO Box 2040, 3000 CA Rotterdam, The Netherlands.No affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article

Language

eng

PubMed ID

18766263

Citation

van Vliet, Huub H D M., et al. "PFA-100 Monitoring of Von Willebrand Factor (VWF) Responses to Desmopressin (DDAVP) and Factor VIII/VWF Concentrate Substitution in Von Willebrand Disease Type 1 and 2." Thrombosis and Haemostasis, vol. 100, no. 3, 2008, pp. 462-8.
van Vliet HH, Kappers-Klunne MC, Leebeek FW, et al. PFA-100 monitoring of von Willebrand factor (VWF) responses to desmopressin (DDAVP) and factor VIII/VWF concentrate substitution in von Willebrand disease type 1 and 2. Thromb Haemost. 2008;100(3):462-8.
van Vliet, H. H., Kappers-Klunne, M. C., Leebeek, F. W., & Michiels, J. J. (2008). PFA-100 monitoring of von Willebrand factor (VWF) responses to desmopressin (DDAVP) and factor VIII/VWF concentrate substitution in von Willebrand disease type 1 and 2. Thrombosis and Haemostasis, 100(3), pp. 462-8.
van Vliet HH, et al. PFA-100 Monitoring of Von Willebrand Factor (VWF) Responses to Desmopressin (DDAVP) and Factor VIII/VWF Concentrate Substitution in Von Willebrand Disease Type 1 and 2. Thromb Haemost. 2008;100(3):462-8. PubMed PMID: 18766263.
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TY - JOUR T1 - PFA-100 monitoring of von Willebrand factor (VWF) responses to desmopressin (DDAVP) and factor VIII/VWF concentrate substitution in von Willebrand disease type 1 and 2. AU - van Vliet,Huub H D M, AU - Kappers-Klunne,Mies C, AU - Leebeek,Frank W G, AU - Michiels,Jan J, PY - 2008/9/4/pubmed PY - 2008/10/18/medline PY - 2008/9/4/entrez SP - 462 EP - 8 JF - Thrombosis and haemostasis JO - Thromb. Haemost. VL - 100 IS - 3 N2 - Dose-response relationship was studied between PFA-100 closure times (PFA CTs) and factor (F)VIII-von Willebrand factor (VWF) parameters in patients with von Willebrand disease (VWD) type 1 and type 2 before and after treatment with DDAVP (n=84) or FVIII/VWF concentrate (n=38). DDAVP treatment of patients with VWD type 1 normalised the PFA CTs by increasing VWF levels to normal. Of the 14 patients with VWD type 2, PFA CTs did not normalize in eight. Haemate-P substitution in patients with VWD type 1 induced a less favourable response as compared to DDAVP, because PFA CTs did not correct in all patients. Of 12 patients with VWD type 2 treated with Haemate-P, six showed a correction of PFA CTs (<250 sec), which correlated with the normalisation of the VWF CB/Ag ratio. In-vitro studies were performed by using whole blood of patients with VWD and adding various amounts of FVIII/VWF concentrate. Addition of Haemate-P induced an increase of the VWF CB/Ag ratio from 0.30 to 0.70 in blood of patients with VWD type 2 with correction of the PFA CTs. Immunate did not result in an increase of VWF CB/Ag ratio in blood of VWD type 2 patients, and the PFA CTs remained prolonged. We conclude that PFA-100 might be an adequate instrument not only for diagnosis but also for monitoring of DDAVP responses and FVIII/VWF substitution of patients with VWD type 1 and 2, but this is dependent upon the type of VWD and the concentrate used. SN - 0340-6245 UR - https://www.unboundmedicine.com/medline/citation/18766263/PFA_100_monitoring_of_von_Willebrand_factor__VWF__responses_to_desmopressin__DDAVP__and_factor_VIII/VWF_concentrate_substitution_in_von_Willebrand_disease_type_1_and_2_ L2 - http://www.diseaseinfosearch.org/result/9461 DB - PRIME DP - Unbound Medicine ER -