BACKGROUND: Because the number of elderly von Willebrand disease (VWD) patients is increasing, the pathophysiology of aging in VWD has become increasingly relevant. OBJECTIVES: 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. PATIENTS/ METHODS: We included VWD patients with VWF levels ≤ 30 U dL(-1) 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 (n = 71) and younger (16-64 years, n = 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. RESULTS: In elderly type 1 patients, a decade age increase was associated with a 3.5 U dL(-1) (95% CI, -0.6 to 7.6) VWF:Ag increase and 7.1 U dL(-1) (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%; P = 0.048), which was not observed in type 1 VWD. CONCLUSIONS: 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.
BACKGROUND: Because the number of elderly von Willebrand disease (VWD) patients is increasing, the pathophysiology of aging in VWD has become increasingly relevant. OBJECTIVES: 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 VWDpatients and those < 65 years. We also studied co-morbidity in elderly patients. PATIENTS/ METHODS: We included VWDpatients with VWF levels ≤ 30 U dL(-1) 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 VWDpatients older (n = 71) and younger (16-64 years, n = 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. RESULTS: In elderly type 1 patients, a decade age increase was associated with a 3.5 U dL(-1) (95% CI, -0.6 to 7.6) VWF:Ag increase and 7.1 U dL(-1) (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%; P = 0.048), which was not observed in type 1 VWD. CONCLUSIONS: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 patientsVWF parameters do not increase with age and in these patients aging is accompanied by increased bleeding.
Authors: Veronica H Flood; Pamela A Christopherson; Joan Cox Gill; Kenneth D Friedman; Sandra L Haberichter; Daniel B Bellissimo; Rupa A Udani; Mahua Dasgupta; Raymond G Hoffmann; Margaret V Ragni; Amy D Shapiro; Jeanne M Lusher; Steven R Lentz; Thomas C Abshire; Cindy Leissinger; W Keith Hoots; Marilyn J Manco-Johnson; Ralph A Gruppo; Lisa N Boggio; Kate T Montgomery; Anne C Goodeve; Paula D James; David Lillicrap; Ian R Peake; Robert R Montgomery Journal: Blood Date: 2016-02-09 Impact factor: 22.113
Authors: Michelle Lavin; Sonia Aguila; Sonja Schneppenheim; Niall Dalton; Kenneth L Jones; Jamie M O'Sullivan; Niamh M O'Connell; Kevin Ryan; Barry White; Mary Byrne; Marie Rafferty; Mairead M Doyle; Margaret Nolan; Roger J S Preston; Ulrich Budde; Paula James; Jorge Di Paola; James S O'Donnell Journal: Blood Date: 2017-09-15 Impact factor: 22.113
Authors: Mariya H Apostolova; Craig D Seaman; Diane M Comer; Jonathan G Yabes; Margaret V Ragni Journal: Clin Appl Thromb Hemost Date: 2016-09-21 Impact factor: 2.389
Authors: Joan Cox Gill; Stephen F Conley; Victoria P Johnson; Pamela A Christopherson; Sandra L Haberichter; Christina D Diaz; Tatyana C Strong; Jian Zhang; Pippa Simpson; Thomas C Abshire; Robert R Montgomery; Veronica H Flood Journal: Blood Adv Date: 2020-01-14