RSS-Feed abonnieren
PDF herunterladen
DOI: 10.1055/s-0037-1614626
Evaluation of the PFA-100® System in the Diagnosis and Therapeutic Monitoring of Patients with von Willebrand Disease
Authors
Publikationsverlauf
Received
31. Dezember 1998
Accepted after resubmission
07. April 1999
Publikationsdatum:
11. Dezember 2017 (online)
Summary
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 μg/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.
-
References
- 1 Rodeghiero F, Castaman G, Dini E. Epidemiological investigation of the prevalence of von Willebrand's disease. Blood 1987; 69: 454-9.
- 2 Miller CH, Lenzi R, Breen C. Prevalence of von Willebrand's disease among US adults. Blood 1987; 70: 377-83.
- 3 Cattaneo M, Federici AB, Mannucci PM. Diagnosis and treatment of von Willebrand's disease. Int J Ped Hematol/Oncol 1994; 1: 499-508.
- 4 Wagner DD. The Weibel-Palade Body: the storage granule for von Wille-brand factor and P-selectin. Thromb Haemost 1993; 70: 105-10.
- 5 Meyer D, Girma JP. von Willebrand factor: Structure and function. Thromb Haemost 1993; 70: 99-104.
- 6 Ruggeri ZM. von Willebrand factor. J Clin Invest 1997; 99: 559-64.
- 7 Savage B, Shattil SJ, Ruggeri Z. Modulation of platelet function through adhesion receptors: A dual role for glycoprotein IIb-IIIa (integrin αIIbβ3) mediated by fibrinogen and glycoprotein Ib-von Willebrand factor. J Biol Chem 1992; 267: 11300-6.
- 8 Goto S, Salomon DR, Ikeda Y, Ruggeri ZM. Characterization of the unique mechanism mediating the shear-dependent binding of soluble von Wille-brand factor to platelets. J Biol Chem 1995; 270: 23352-61.
- 9 Sadler JE. A revised classification of von Willebrand disease. For the Subcommittee on von Willebrand Factor of the Scientific and Standardization Committee of the International Society on Thrombosis and Haemostasis. Thromb Haemost 1994; 71: 520-5.
- 10 Mannucci PM, Lombardi R, Castaman G, Dent JA, Lattuada A, Rode-ghiero F, Zimmerman TS. von Willebrand disease “"Vicenza” with larger-than-normal (supranormal) von Willebrand factor multimers. Blood 1988; 71: 65-70.
- 11 O'Brien JR, Salmon GP. Shear stress activation of platelet glycoprotein IIb/IIIa plus von Willebrand factor causes aggregation: filter blockage and the long bleeding time in von Willebrand's Disease. Blood 1987; 70: 1354-61.
- 12 Pareti I F, Cattaneo M, Carpinelli L, Zighetti ML, Bressi C, Mannucci PM, Ruggeri ZM. Evaluation of the abnormal platelet function in von Wille-brand disease by the blood filtration test. Thromb Haemost 1996; 75: 460-8.
- 13 Kundu SK, Heilmann E, Sio R, Garcia C, Ostgaart R. Characterization of an in vitro platelet function analyzer, PFA-100™. Clin Appl Thromb Hemost 1996; 2: 2419
- 14 Fressinaud E, Veyradiers A, Truchaud F, Martin I, Boyer-Neumann C, Trossaert M, Meyer D. Screening for von Willebrand disease with a new analyzer using high shear stress: a study of 60 cases. Blood 1998; 91: 1325-31.
- 15 Federici AB, Stabile F, Castaman G, Canciani MT, Mannucci PM. Treatment of acquired von Willebrand syndrome in patients with monoclonal gammopathy of uncertain significance: comparison of three different therapeutic approaches. Blood 1998; 92: 2707-11.
- 16 Kundu SK, Heilmann EJ, Sio R, Garcia C, Davidson RM, Ostgaard RA. Description of an “in vitro” platelet function analyzer- PFA-100™. Sem Thromb Hemost 1995; 21: 106-12.
- 17 Mammen EF, Alshameeri RS, Comp PC. Preliminary data from a field trial of the PFA-100™ system. Sem Thromb Hemost 1995; 21: 113-21.
- 18 Italian Working Group. Spectrum of von Willebrand's disease: A study of 100 cases. Br J Haematol 1977; 35: 101-12.
- 19 Gralnick HR, Rick ME, McKeown LP, Williams SB, Parker RI, Maisonneuve P, Jenneau C, Sultan Y. Platelet von Willebrand factor: an important determinant of the bleeding time in type I von Willebrand disease. Blood 1986; 68: 58-61.