Maria Carter-Febres , Matthew Fenchel , Jennifer Pomales , Cristina Tarango , Eric S. Mullins
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引用次数: 0
摘要
背景冯-威廉氏病(VWD)是最常见的遗传性出血性疾病。VWD 的特征是冯-威廉因子(VWF)的数量或质量异常。我们假设贫血与 VWF 和因子 VIII (FVIII) 的基线升高有关。我们还假设肥胖与 VWF 水平升高有关。方法我们对有近端 von Willebrand 图谱和血红蛋白数据的患者的电子健康记录进行了单中心审查。结果我们确定了 4552 名在 24 小时内进行过 VWF 研究和全血细胞计数的受试者。我们还发现,肥胖和黑人种族与 VWF 抗原、活性和 FVIII 活性的增加有独立关联。我们的研究表明,贫血、种族和体重指数与 VWF 抗原、VWF 活性和 FVIII 水平的升高有关。要点--贫血与 VWF 抗原、VWF 活性和 FVIII 水平的升高有关--在贫血时检测 von Willebrand 因子可能会掩盖 von Willebrand 病的诊断。
Hemoglobin concentration and body mass index are determinants of plasma von Willebrand factor and factor VIII levels
Background
Von Willebrand Disease (VWD) is the most common inherited bleeding disorder. VWD is characterized by an abnormal quantity or quality of von Willebrand Factor (VWF). Anemia is often found at presentation for a bleeding disorder evaluation due to chronic blood loss.
Objectives/Hypothesis
We hypothesized that anemia is associated with elevations in both VWF and factor VIII (FVIII) over baseline. We also hypothesized that obesity would be associated with increased levels of VWF.
Methods
We conducted a single-center review of the electronic health record for patients that had proximal von Willebrand profiles and Hb data.
Results
We identified 4552 unique subjects with VWF studies and a CBC within 24 h. We found that decreasing hemoglobin inversely correlated with VWF antigen, VWF ristocetin cofactor activity, and FVIII activity. We also found that obesity and Black race were independently associated with increased VWF antigen, activity, and FVIII activity. Hb, race, and body mass index (BMI) continued to be determinants of VWF and FVIII levels in multivariable analysis.
Conclusion
Our study demonstrates that anemia, race, and BMI were found to be associated with elevation of VWF antigen, VWF activity, and FVIII levels. As many individuals with anemia present for evaluation for a bleeding disorder, these variables need to be considered.
Key points
- Anemia was found to be associated with elevation of VWF antigen, VWF activity and FVIII levels.
- Testing von Willebrand factor at times of anemia may mask a diagnosis of von Willebrand Disease.
期刊介绍:
Thrombosis Research is an international journal dedicated to the swift dissemination of new information on thrombosis, hemostasis, and vascular biology, aimed at advancing both science and clinical care. The journal publishes peer-reviewed original research, reviews, editorials, opinions, and critiques, covering both basic and clinical studies. Priority is given to research that promises novel approaches in the diagnosis, therapy, prognosis, and prevention of thrombotic and hemorrhagic diseases.