Von Willebrand disease and aging: an evolving phenotype. (July 2014)

• Record Type: Journal Article
• Authors: Sanders, Y. V.; Giezenaar, M. A.; Laros‐van Gorkom, B. A. P.; Meijer, K.; van der Bom, J. G.; Cnossen, M. H.; Nijziel, M. R.; Ypma, P. F.; Fijnvandraat, K.; Eikenboom, J.; Mauser‐Bunschoten, E. P.; Leebeek, F. W. G.; the WiN study group
• Is Part Of: Journal of thrombosis and haemostasis. Volume 12:Number 7(2014:Jul.)
• Page Start: 1066
• Page End: 1075
• Publication Date: 2014-07
• Subjects: Thrombosis -- Periodicals; Hemostasis -- Periodicals; Blood coagulation disorders -- Periodicals; 616.1
• DOI: 10.1111/jth.12586
• Languages: English
• ISSNs: 1538-7933

Abstract

<abstract abstract-type="main" id="jth12586-abs-0001"> <title>Summary</title> <sec id="jth12586-sec-0001" sec-type="section"> <title>Background</title> <p>Because the number of elderly von Willebrand disease (VWD) patients is increasing, the pathophysiology of aging in VWD has become increasingly relevant.</p> </sec> <sec id="jth12586-sec-0002" sec-type="section"> <title>Objectives</title> <p>To assess age‐related changes in von Willebrand factor (VWF) and factor VIII (FVIII) levels and to compare age‐related differences in bleeding phenotype between elderly VWD patients and those < 65 years. We also studied co‐morbidity in elderly patients.</p> </sec> <sec id="jth12586-sec-0003" sec-type="section"> <title>Patients/Methods</title> <p>We included VWD patients with VWF levels ≤ 30 U dL<sup>−1</sup> in the nationwide cross‐sectional 'Willebrand in the Netherlands' (WiN‐) study. Patients reported bleeding episodes and treatment of VWD in the year preceding inclusion and during life. This was compared between VWD patients older (<italic>n </italic>=<italic> </italic>71) and younger (16–64 years, <italic>n </italic>=<italic> </italic>593) than 65 years. In elderly patients, age‐related changes in VWF and FVIII levels were studied longitudinally by including all historically measured levels. All medical records were examined for co‐morbidity.</p> </sec> <sec id="jth12586-sec-0004" sec-type="section"> <title>Results</title> <p>In elderly type 1 patients, a decade age increase was associated with a 3.5 U dL<sup>−1</sup> (95% CI, −0.6 to 7.6) VWF:Ag increase and 7.1 U dL<sup>−1</sup> (95% CI, 0.7 to 13.4) FVIII:C increase. This increase was not observed in elderly type 2 patients. Elderly type 2 patients reported significantly more bleeding symptoms in the year preceding inclusion than younger patients (16/27, 59% vs. 87/221, 39%; <italic>P </italic>=<italic> </italic>0.048), which was not observed in type 1 VWD.</p> </sec> <sec id="jth12586-sec-0005" sec-type="section"> <title>Conclusions</title> <p>von Willebrand factor parameters and bleeding phenotype evolve with increasing age in VWD. VWF and FVIII levels increase with age in type 1 patients with no mitigation in bleeding phenotype. In type 2 patients VWF parameters do not increase with age and in these patients aging is accompanied by increased bleeding.</p> </sec> </abstract>