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An evaluation of the DDAVP infusion test with PFA-100 and vWF activity assays to distinguish vWD types in children.
Clin Appl Thromb Hemost 2011; 17(5):441-8CA

Abstract

von Willebrand disease (vWD) is classified into partial (type 1), qualitative (type 2), and total deficiency (type 3).The aims of the study were to evaluate prospectively the potency of the DDAVP infusion test together with von Willebrand factor (vWF) ristocetin cofactor (vWF:RCo), vWF antigen (vWF:Ag), factor VIII coagulant activity (FVIII:C), and platelet function analyzer (PFA)-100 to distinguish vWD types. Genetic analysis and multimeric analysis of vWF was not applied. We classified the 112 patients and 47 healthy children phenotypically according to the laboratory test results and bleeding severity score. PFA-100 closure times (CT), FVIII:C, vWF:RCo, vWF:Ag, ristocetin-induced platelet aggregation (RIPA), and the response of FVIII:C and vWF parameters to desmopressin (DDAVP) were used to define types 1, 2, and 3 vWD. Type 1 vWD is mild in 34 cases (vWF:RCo % 40-55), moderate in 29 (vWF:RCo %27-40), severe type 1 vWD or nonclassical type 2 vWD in 12 cases (vWF:RCo % 4-16), and type 2 vWD in 23 cases (vWF:RCo %4-38).The response to DDAVP of vWF parameters is normal in all patients with mild/moderate type 1 vWD, 6 patients with severe type 1 vWD or nonclassical type 2 vWD and 11 patients with type 2 vWD. In conclusion, this study showed that measurement of vWF:RCo, vWF:Ag, FVIII:C, and PFA-100 parameters can differentiate vWD types but not severe type 1 vWD or nonclassical type 2 vWD. In the differentiation of severe type 1 vWD and nonclassical type 2 vWD, DDAVP response may be used.

Authors+Show Affiliations

Department of Pediatric Hematology, Ege University Faculty of Medicine, Izmir, Turkey. drmehmetakin@yahoo.com.trNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Clinical Trial
Journal Article

Language

eng

PubMed ID

20460340

Citation

Akin, Mehmet, et al. "An Evaluation of the DDAVP Infusion Test With PFA-100 and vWF Activity Assays to Distinguish vWD Types in Children." Clinical and Applied Thrombosis/hemostasis : Official Journal of the International Academy of Clinical and Applied Thrombosis/Hemostasis, vol. 17, no. 5, 2011, pp. 441-8.
Akin M, Karapinar DY, Balkan C, et al. An evaluation of the DDAVP infusion test with PFA-100 and vWF activity assays to distinguish vWD types in children. Clin Appl Thromb Hemost. 2011;17(5):441-8.
Akin, M., Karapinar, D. Y., Balkan, C., Ay, Y., & Kavakli, K. (2011). An evaluation of the DDAVP infusion test with PFA-100 and vWF activity assays to distinguish vWD types in children. Clinical and Applied Thrombosis/hemostasis : Official Journal of the International Academy of Clinical and Applied Thrombosis/Hemostasis, 17(5), pp. 441-8. doi:10.1177/1076029610366440.
Akin M, et al. An Evaluation of the DDAVP Infusion Test With PFA-100 and vWF Activity Assays to Distinguish vWD Types in Children. Clin Appl Thromb Hemost. 2011;17(5):441-8. PubMed PMID: 20460340.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - An evaluation of the DDAVP infusion test with PFA-100 and vWF activity assays to distinguish vWD types in children. AU - Akin,Mehmet, AU - Karapinar,Deniz Yilmaz, AU - Balkan,Can, AU - Ay,Yilmaz, AU - Kavakli,Kaan, Y1 - 2010/05/11/ PY - 2010/5/13/entrez PY - 2010/5/13/pubmed PY - 2012/1/20/medline SP - 441 EP - 8 JF - Clinical and applied thrombosis/hemostasis : official journal of the International Academy of Clinical and Applied Thrombosis/Hemostasis JO - Clin. Appl. Thromb. Hemost. VL - 17 IS - 5 N2 - von Willebrand disease (vWD) is classified into partial (type 1), qualitative (type 2), and total deficiency (type 3).The aims of the study were to evaluate prospectively the potency of the DDAVP infusion test together with von Willebrand factor (vWF) ristocetin cofactor (vWF:RCo), vWF antigen (vWF:Ag), factor VIII coagulant activity (FVIII:C), and platelet function analyzer (PFA)-100 to distinguish vWD types. Genetic analysis and multimeric analysis of vWF was not applied. We classified the 112 patients and 47 healthy children phenotypically according to the laboratory test results and bleeding severity score. PFA-100 closure times (CT), FVIII:C, vWF:RCo, vWF:Ag, ristocetin-induced platelet aggregation (RIPA), and the response of FVIII:C and vWF parameters to desmopressin (DDAVP) were used to define types 1, 2, and 3 vWD. Type 1 vWD is mild in 34 cases (vWF:RCo % 40-55), moderate in 29 (vWF:RCo %27-40), severe type 1 vWD or nonclassical type 2 vWD in 12 cases (vWF:RCo % 4-16), and type 2 vWD in 23 cases (vWF:RCo %4-38).The response to DDAVP of vWF parameters is normal in all patients with mild/moderate type 1 vWD, 6 patients with severe type 1 vWD or nonclassical type 2 vWD and 11 patients with type 2 vWD. In conclusion, this study showed that measurement of vWF:RCo, vWF:Ag, FVIII:C, and PFA-100 parameters can differentiate vWD types but not severe type 1 vWD or nonclassical type 2 vWD. In the differentiation of severe type 1 vWD and nonclassical type 2 vWD, DDAVP response may be used. SN - 1938-2723 UR - https://www.unboundmedicine.com/medline/citation/20460340/An_evaluation_of_the_DDAVP_infusion_test_with_PFA_100_and_vWF_activity_assays_to_distinguish_vWD_types_in_children_ L2 - http://journals.sagepub.com/doi/full/10.1177/1076029610366440?url_ver=Z39.88-2003&rfr_id=ori:rid:crossref.org&rfr_dat=cr_pub=pubmed DB - PRIME DP - Unbound Medicine ER -