Hernandez-Navarro F, Quintana M, Jimenez-Yuste V, Alvarez M T, Fernandez-Morata R
Centro de Coagulopatias, Haematology Department, Hospital Universitario La Paz, Madrid, Spain.
Haemophilia. 2008 Sep;14(5):963-7. doi: 10.1111/j.1365-2516.2008.01784.x. Epub 2008 Jul 9.
Therapy with factor VIII/von Willebrand factor (FVIII/VWF) concentrate is the mainstay therapy in patients with von Willebrand disease (VWD) unresponsive to desmopressin. There are several commercially available FVIII/VWF concentrates that have been tested in VWD patients. We retrospectively analized the clinical efficacy in bleeding episodes and surgery of a highly purified FVIII/VWF complex with two inactivation steps (Fanhdi) in VWD patients. Sixty patients were included in the study. Treatment schedule consisted of one or more doses (standard dose 40 IU/kg body weight of FVIII) of Fanhdi. One hundred and fifty bleeding episodes were treated. These were: 28 serious bleedings; 92 moderate and 30 mild. An excellent clinical efficacy in almost 95% of cases was observed. Fanhdi was administered during 66 surgical procedures (38 major and 28 minor) with an overall efficacy of 98%. Fanhdi a highly purified, doubly virus-inactivated FVIII/VWF concentrate, with a high content of active VWF and an excellent record of clinical safety, is a valid choice in treating VWD.
使用因子VIII/血管性血友病因子（FVIII/VWF）浓缩物进行治疗是对去氨加压素无反应的血管性血友病（VWD）患者的主要治疗方法。有几种市售的FVIII/VWF浓缩物已在VWD患者中进行了测试。我们回顾性分析了一种经过两步灭活的高纯度FVIII/VWF复合物（Fanhdi）在VWD患者出血发作和手术中的临床疗效。该研究纳入了60名患者。治疗方案包括一剂或多剂（FVIII的标准剂量为40 IU/kg体重）的Fanhdi。共治疗了150次出血发作。其中包括：28次严重出血；92次中度出血和30次轻度出血。观察到几乎95%的病例临床疗效极佳。在66例手术过程中（38例大手术和28例小手术）使用了Fanhdi，总体有效率为98%。Fanhdi是一种高度纯化、双重病毒灭活的FVIII/VWF浓缩物，具有高含量的活性VWF和出色的临床安全记录，是治疗VWD的有效选择。
