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Evaluation of the PFA-100 system in the diagnosis and therapeutic monitoring of patients with von Willebrand disease.
Thromb Haemost. 1999 Jul; 82(1):35-9.TH

Abstract

We have evaluated platelet function at high shear with the PFA-100 system in different subtypes of von Willebrand disease (vWD), before and after the intravenous infusions of desmopressin or a factor-VIII/von Willebrand factor (vWF) concentrate. Closure times with the PFA-100 system were determined for both the collagen/ADP and the collagen/epinephrine cartridges in 52 patients with vWD (9 type 1 "platelet normal", 5 type 1 "platelet-discordant", 8 type 1 "platelet-low", 6 type 2A, 9 type 2B, 6 type 2M Vicenza. 6 type 3 and 3 acquired vWD) and 40 controls. Measurements were repeated 1 and 4 h after the i.v. infusion of desmopressin (0.3 microg/Kg) in 26 patients with types 1, type 2M Vicenza or type 2A vWD, or of a factorVIII/vWF concentrate (Alphanate HT, 60 U/Kg) in 4 patients with type 3 vWD. At all time points, vWF plasma levels and the bleeding time (Symplate II) were also determined. Baseline closure times were longer in vWD patients than in controls with both the collagen/ADP and the collagen/ epinephrine cartridges. The sensitivity of the PFA-100 system (88% and 87% with the two cartridges) was higher than that of the bleeding time (65%). Treatment with desmopressin normalized the closure times in patients with type 1 "platelet-normal" or type 2M Vicenza vWD, had no significant effects in patients with type 1 "platelet-low", type 1 "platelet-discordant" or type 2A vWD. Infusion of a factorVIII/vWF concentrate in patients with type 3 vWD slightly shortened their prolonged closure times. In general, changes in PFA-100 were paralleled by shortenings of the bleeding times and increases in plasma vWF levels. The PFA-100 test reflects vWF-dependent platelet function under high shear stress and could be useful in the diagnosis and therapeutic monitoring of patients with vWD.

Authors+Show Affiliations

Angelo Bianchi Bonomi Hemophilia and Thrombosis Center, Department of Internal Medicine, University of Milano, Italy. marco.cattaneo@unimi.itNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article

Language

eng

PubMed ID

10456451

Citation

Cattaneo, M, et al. "Evaluation of the PFA-100 System in the Diagnosis and Therapeutic Monitoring of Patients With Von Willebrand Disease." Thrombosis and Haemostasis, vol. 82, no. 1, 1999, pp. 35-9.
Cattaneo M, Federici AB, Lecchi A, et al. Evaluation of the PFA-100 system in the diagnosis and therapeutic monitoring of patients with von Willebrand disease. Thromb Haemost. 1999;82(1):35-9.
Cattaneo, M., Federici, A. B., Lecchi, A., Agati, B., Lombardi, R., Stabile, F., & Bucciarelli, P. (1999). Evaluation of the PFA-100 system in the diagnosis and therapeutic monitoring of patients with von Willebrand disease. Thrombosis and Haemostasis, 82(1), 35-9.
Cattaneo M, et al. Evaluation of the PFA-100 System in the Diagnosis and Therapeutic Monitoring of Patients With Von Willebrand Disease. Thromb Haemost. 1999;82(1):35-9. PubMed PMID: 10456451.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Evaluation of the PFA-100 system in the diagnosis and therapeutic monitoring of patients with von Willebrand disease. AU - Cattaneo,M, AU - Federici,A B, AU - Lecchi,A, AU - Agati,B, AU - Lombardi,R, AU - Stabile,F, AU - Bucciarelli,P, PY - 1999/8/24/pubmed PY - 1999/8/24/medline PY - 1999/8/24/entrez SP - 35 EP - 9 JF - Thrombosis and haemostasis JO - Thromb. Haemost. VL - 82 IS - 1 N2 - We have evaluated platelet function at high shear with the PFA-100 system in different subtypes of von Willebrand disease (vWD), before and after the intravenous infusions of desmopressin or a factor-VIII/von Willebrand factor (vWF) concentrate. Closure times with the PFA-100 system were determined for both the collagen/ADP and the collagen/epinephrine cartridges in 52 patients with vWD (9 type 1 "platelet normal", 5 type 1 "platelet-discordant", 8 type 1 "platelet-low", 6 type 2A, 9 type 2B, 6 type 2M Vicenza. 6 type 3 and 3 acquired vWD) and 40 controls. Measurements were repeated 1 and 4 h after the i.v. infusion of desmopressin (0.3 microg/Kg) in 26 patients with types 1, type 2M Vicenza or type 2A vWD, or of a factorVIII/vWF concentrate (Alphanate HT, 60 U/Kg) in 4 patients with type 3 vWD. At all time points, vWF plasma levels and the bleeding time (Symplate II) were also determined. Baseline closure times were longer in vWD patients than in controls with both the collagen/ADP and the collagen/ epinephrine cartridges. The sensitivity of the PFA-100 system (88% and 87% with the two cartridges) was higher than that of the bleeding time (65%). Treatment with desmopressin normalized the closure times in patients with type 1 "platelet-normal" or type 2M Vicenza vWD, had no significant effects in patients with type 1 "platelet-low", type 1 "platelet-discordant" or type 2A vWD. Infusion of a factorVIII/vWF concentrate in patients with type 3 vWD slightly shortened their prolonged closure times. In general, changes in PFA-100 were paralleled by shortenings of the bleeding times and increases in plasma vWF levels. The PFA-100 test reflects vWF-dependent platelet function under high shear stress and could be useful in the diagnosis and therapeutic monitoring of patients with vWD. SN - 0340-6245 UR - https://www.unboundmedicine.com/medline/citation/10456451/Evaluation_of_the_PFA_100_system_in_the_diagnosis_and_therapeutic_monitoring_of_patients_with_von_Willebrand_disease_ L2 - https://ClinicalTrials.gov/search/term=10456451 [PUBMED-IDS] DB - PRIME DP - Unbound Medicine ER -