TY - JOUR
T1 - Determinants of bleeding phenotype in adult patients with moderate or severe von Willebrand disease
AU - Wee, EM
AU - Sanders, Yvonne
AU - Mauser-Bunschoten, EP
AU - van der Bom, JG (Anske)
AU - Degenaar-Dujardin, MEL
AU - Eikenboom, J
AU - Bolder, A
AU - Laros-Van Gorkom, BAP
AU - Meijer, K (Karina)
AU - Hamulyak, K
AU - Nijziel, MR
AU - Fijnvandraat, K
AU - Leebeek, Frank
PY - 2012
Y1 - 2012
N2 - We performed a nation-wide cross-sectional study to evaluate determinants of bleeding symptoms in a large unselected cohort of adults with von Willebrand disease (VWD). VWD patients were included (n=664), based on lowest historically measured VWF:Ag and VWF:Act levels <= 30 U/dl. Menorrhagia (85%), cutaneous bleeding (77%), bleeding from minor wounds (77%) and oral-cavity bleeding (62%) occurred most frequently. Higher age was associated with a higher bleeding score (BS), determined according to Tosetto, in females. A 10 year increase in age was associated with 0.8 point (95% confidence interval [CI] 0.4-1.1) higher BS. Females had higher BS than males (median 12 vs. 10, p=0.012). BS differed significantly between VWD type 1, 2 and 3: median 9 (-2-31), 13 (-1-33) and 19.5 (1-35), respectively (p < 0.001). BS was strongly associated with VWF and FVIII levels: individuals with VWF:Ag levels <= 10 IU/dl, VWF:Act <= 10 IU/dl and FVIII:C <= 10 IU/dl had, respectively, 5.3 point (95%CI 3.2-7.3), 4.3 point (95%CI 2.9-5.8) and 9.6 point (95%CI 6.5-12.7) higher BS, than those with levels > 30 IU/dl. In type 3 patients 1 IU/dl FVIII:C decrease was associated with 0.6 point (95%CI 0.1-1.1) BS increase (p=0.021). In conclusion, in VWD patients the bleeding phenotype is strongly associated with type of VWD and VWF and FVIII levels.
AB - We performed a nation-wide cross-sectional study to evaluate determinants of bleeding symptoms in a large unselected cohort of adults with von Willebrand disease (VWD). VWD patients were included (n=664), based on lowest historically measured VWF:Ag and VWF:Act levels <= 30 U/dl. Menorrhagia (85%), cutaneous bleeding (77%), bleeding from minor wounds (77%) and oral-cavity bleeding (62%) occurred most frequently. Higher age was associated with a higher bleeding score (BS), determined according to Tosetto, in females. A 10 year increase in age was associated with 0.8 point (95% confidence interval [CI] 0.4-1.1) higher BS. Females had higher BS than males (median 12 vs. 10, p=0.012). BS differed significantly between VWD type 1, 2 and 3: median 9 (-2-31), 13 (-1-33) and 19.5 (1-35), respectively (p < 0.001). BS was strongly associated with VWF and FVIII levels: individuals with VWF:Ag levels <= 10 IU/dl, VWF:Act <= 10 IU/dl and FVIII:C <= 10 IU/dl had, respectively, 5.3 point (95%CI 3.2-7.3), 4.3 point (95%CI 2.9-5.8) and 9.6 point (95%CI 6.5-12.7) higher BS, than those with levels > 30 IU/dl. In type 3 patients 1 IU/dl FVIII:C decrease was associated with 0.6 point (95%CI 0.1-1.1) BS increase (p=0.021). In conclusion, in VWD patients the bleeding phenotype is strongly associated with type of VWD and VWF and FVIII levels.
U2 - 10.1160/TH12-04-0244
DO - 10.1160/TH12-04-0244
M3 - Article
VL - 108
SP - 683
EP - 692
JO - Thrombosis and Haemostasis
JF - Thrombosis and Haemostasis
SN - 0340-6245
IS - 4
ER -