Cattaneo M, Federici A B, Lecchi A, Agati B, Lombardi R, Stabile F, Bucciarelli P
Angelo Bianchi Bonomi Hemophilia and Thrombosis Center, Department of Internal Medicine, University of Milano, Italy.
Thromb Haemost. 1999 Jul;82(1):35-9.
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.
我们使用PFA-100系统评估了不同亚型血管性血友病（vWD）患者在静脉输注去氨加压素或因子VIII/血管性血友病因子（vWF）浓缩物前后，在高剪切力下的血小板功能。对52例vWD患者（9例1型“血小板正常”、5例1型“血小板不一致”、8例1型“血小板减少”、6例2A型、9例2B型、6例2M维琴察型、6例3型和3例获得性vWD）和40例对照者，测定了胶原/ADP和胶原/肾上腺素检测卡的PFA-100系统封闭时间。对26例1型、2M维琴察型或2A型vWD患者静脉输注去氨加压素（0.3μg/kg）后1小时和4小时，以及对4例3型vWD患者静脉输注因子VIII/vWF浓缩物（Alphanate HT，60U/kg）后，重复进行测量。在所有时间点，还测定了vWF血浆水平和出血时间（Symplate II）。在胶原/ADP和胶原/肾上腺素检测卡检测中，vWD患者的基线封闭时间均长于对照者。PFA-100系统的敏感性（两种检测卡分别为88%和87%）高于出血时间的敏感性（65%）。去氨加压素治疗使1型“血小板正常”或2M维琴察型vWD患者的封闭时间恢复正常，对1型“血小板减少”、1型“血小板不一致”或2A型vWD患者无显著影响。对3型vWD患者输注因子VIII/vWF浓缩物可略微缩短其延长的封闭时间。总体而言，PFA-100的变化与出血时间缩短和血浆vWF水平升高平行。PFA-100检测反映了高剪切应力下vWF依赖的血小板功能，可用于vWD患者的诊断和治疗监测。
