Lubetsky A, Schulman S, Varon D, Martinowitz U, Kenet G, Gitel S, Inbal A
Institute of Thrombosis and Hemostasis and National Haemophilia Center, Sheba Medical Center, Tel-Hashomer, Israel.
Thromb Haemost. 1999 Feb;81(2):229-33.
We studied the safety and efficacy of treatment with continuous infusion of a von Willebrand factor (vWF) concentrate Haemate-P in patients with von Willebrand disease (vWD). Three patients with mild and 5 patients with severe forms of vWD, were treated with continuous infusion of Haemate-P by minipump. The indications for treatment were: to prevent bleeding during 9 surgical procedures or 1 vaginal delivery in 6 patients and to treat 2 bleeding episodes in 2 patients. The patients were monitored daily for factor VIII (FVIII:C) and ristocetin cofactor (vWF: RCo) levels and the infusion rate was adjusted to maintain the desired therapeutic level of vWF:RCo. The treatment was effective in preventing surgical bleeding and controlling bleeding episodes. All factor VIII:C and most of the vWF:RCo levels measured during the study period were above the target therapeutic levels. A significant decrease in clearance of FVIII:C and vWF:RCo was observed over the treatment period. Haemate-P consumption averaged 24.3+/-7.9 vWF:RCo U/kg/day which is approximately half the expected dose had intermittent bolus injections been used. We suggest that continuous Haemate-P infusion is superior to intermittent bolus injections for the treatment of vWD patients by virtue of its efficiency, simplicity and considerable savings.
我们研究了用von Willebrand因子（vWF）浓缩物Haemate-P持续输注治疗血管性血友病（vWD）患者的安全性和有效性。3例轻度和5例重度vWD患者通过微型泵持续输注Haemate-P进行治疗。治疗指征为：6例患者在9次外科手术或1次阴道分娩期间预防出血，以及2例患者治疗2次出血发作。每天监测患者的因子VIII（FVIII:C）和瑞斯托霉素辅因子（vWF:RCo）水平，并调整输注速率以维持所需的vWF:RCo治疗水平。该治疗在预防手术出血和控制出血发作方面有效。在研究期间测量的所有FVIII:C和大多数vWF:RCo水平均高于目标治疗水平。在治疗期间观察到FVIII:C和vWF:RCo清除率显著降低。Haemate-P的消耗量平均为24.3±7.9 vWF:RCo U/kg/天，若采用间歇性推注注射，该剂量约为预期剂量的一半。我们认为，持续输注Haemate-P治疗vWD患者优于间歇性推注注射，因为其具有高效、简便和显著节省的优点。
