Lopes Maria Sueli da Silva Namen, Rezende Suely Meireles
Universidade Federal de Minas Gerais (UFMG), Belo Horizonte, MG, Brazil.
Universidade Federal de Minas Gerais (UFMG), Belo Horizonte, MG, Brazil.
Hematol Transfus Cell Ther. 2021 Jan-Mar;43(1):43-49. doi: 10.1016/j.htct.2019.12.004. Epub 2020 Jan 30.
von Willebrand's disease (VWD) is the most common inherited bleeding disorder. The 1-desamino-8-d-arginine vasopressin (DDAVP) is the treatment of choice for most responsive patients with VWD. The aim of this study was to evaluate DDAVP use in the management of VWD.
We implemented a survey targeting medical doctors involved in the management of VWD in Brazil. Data was collected during a national congress on Hematology in November 2017.
A total of 51/80 (63.8%) questionnaires were collected. Most participants (76.2%) were hematologists who assisted adult patients and approximately 60% worked at hemophilia treatment centers (HTCs). Approximately half of participants who reported treating patients with VWD, assisted on average, less than 5 patients per month, and approximately 60% declared not having used any DDAVP for treating VWD in the previous year. However, most participants (70%) prescribed FVIII-containing VWF concentrate (VWF/FVIII) for 1-10 patients in the previous year. More than 80% of the participants recognized the main indications for DDAVP. Physicians who recognized indication for DDAVP for type 1 VWD more often had prescribed DDAVP in previous year (p = 0.03). Barriers for prescribing DDAVP varied and included unavailability of laboratory facilities and consumables for DDAVP testing and lack of skills on its prescription.
The DDAVP is currently underused in Brazil, as opposed to the excessive use of VWF/FVIII in VWD patients. We suggest the adoption of measures targeting educational and auditing programs. Furthermore, availability of laboratory reagents is needed to evaluate response and increment the correct use of DDAVP.
血管性血友病(VWD)是最常见的遗传性出血性疾病。1-去氨基-8-D-精氨酸加压素(DDAVP)是大多数有反应的VWD患者的首选治疗方法。本研究的目的是评估DDAVP在VWD管理中的应用。
我们针对巴西参与VWD管理的医生开展了一项调查。数据于2017年11月在一次全国血液学大会期间收集。
共收集到51/80(63.8%)份问卷。大多数参与者(76.2%)是治疗成年患者的血液科医生,约60%在血友病治疗中心(HTC)工作。报告治疗VWD患者的参与者中,约一半平均每月治疗不到5名患者,约60%宣称上一年未使用任何DDAVP治疗VWD。然而,大多数参与者(70%)上一年为1 - 10名患者开具了含FVIII的血管性血友病因子浓缩物(VWF/FVIII)。超过80%的参与者认可DDAVP的主要适应证。认识到1型VWD使用DDAVP适应证的医生上一年更常开具DDAVP(p = 0.03)。开具DDAVP的障碍各不相同,包括缺乏用于DDAVP检测的实验室设施和耗材以及开具处方的技能不足。
与在VWD患者中过度使用VWF/FVIII相反,DDAVP目前在巴西未得到充分利用。我们建议采取针对教育和审核项目的措施。此外,需要有实验室试剂来评估反应并增加DDAVP的正确使用。