Seaman Craig D, Ragni Margaret V
1 Division of Hematology/Oncology, Department of Medicine, University of Pittsburgh Medical Center, Pittsburgh, PA, USA.
2 Hemophilia Center of Western Pennsylvania, Pittsburgh, PA, USA.
Clin Appl Thromb Hemost. 2018 Apr;24(3):434-438. doi: 10.1177/1076029617724232. Epub 2017 Sep 6.
Little is known about aging in von Willebrand disease (VWD). It is uncertain whether VWD patients experience an age-related increase in von Willebrand factor (VWF) levels, and if so, it is unknown whether normalization of VWF levels with aging ameliorates bleeding risk. We aimed to determine the association of age with VWF levels and bleeding risk in patients with type 1 VWD. This is a retrospective chart review of patients with type 1 VWD presenting to the Hemophilia Clinic of Western Pennsylvania for regularly scheduled clinic visits. Data collected included VWF antigen level and condensed molecular and clinical markers for the diagnosis and management of Type 1 (MCMDM-1) VWD bleeding assessment tool (BAT) bleeding score based on bleeding symptoms during the previous 3 years. Thirty-nine patients participated in the study, and 32 were female. The average age of participants was 41.8 ± 18.0 years. The mean VWF antigen level was 0.83 ± 0.37 IU/mL, and the mean bleeding score was 2.51 ± 2.90. The bleeding score was inversely associated with age, β = -0.080 (SE = 0.023), P < .01. There was a nonsignificant association between VWF antigen levels and age. To our knowledge, this is the first report showing an association between aging and decreased bleeding symptoms in patients with type 1 VWD. Determining whether or not bleeding risk is reduced in older patients with type 1 VWD is essential for optimal clinical management. Moreover, VWF concentrate is costly, and unwarranted use represents a significant waste of health-care dollars. These findings warrant further investigation.
关于血管性血友病(VWD)的衰老情况,人们了解甚少。目前尚不确定VWD患者的血管性血友病因子(VWF)水平是否会随年龄增长而升高,即便如此,也不清楚VWF水平随年龄增长而恢复正常是否能改善出血风险。我们旨在确定1型VWD患者的年龄与VWF水平及出血风险之间的关联。这是一项对前往宾夕法尼亚西部血友病诊所进行定期门诊的1型VWD患者的回顾性病历审查。收集的数据包括VWF抗原水平以及用于1型(MCMDM - 1)VWD诊断和管理的简明分子和临床标志物、基于过去3年出血症状的VWD出血评估工具(BAT)出血评分。39名患者参与了该研究,其中32名女性。参与者的平均年龄为41.8±18.0岁。VWF抗原水平的平均值为0.83±0.37 IU/mL,出血评分的平均值为2.51±2.90。出血评分与年龄呈负相关,β = -0.080(标准误 = 0.023),P < 0.01。VWF抗原水平与年龄之间存在无统计学意义的关联。据我们所知,这是首份显示1型VWD患者衰老与出血症状减轻之间存在关联的报告。确定老年1型VWD患者的出血风险是否降低对于优化临床管理至关重要。此外,VWF浓缩物成本高昂,不必要的使用会造成医疗保健资金的重大浪费。这些发现值得进一步研究。