BACKGROUND: A quantitative description of bleeding symptoms in type 1 von Willebrand disease (VWD) has never been reported. OBJECTIVES: The aim was to quantitatively evaluate the severity of bleeding symptoms in type 1 VWD and its correlation with clinical and laboratory features. PATIENTS AND METHODS: Bleeding symptoms were retrospectively recorded in a European cohort of VWD type 1 families, and for each subject a quantitative bleeding score (BS) was obtained together with phenotypic tests. RESULTS: A total of 712 subjects belonging to 144 families and 195 controls were available for analysis. The BS was higher in index cases than in affected family members (BS 9 vs. 5, P < 0.0001) and in unaffected family members than in controls (BS 0 vs. -1, P < 0.0001). There was no effect of ABO blood group. BS showed a strong significant inverse relation with either von Willebrand ristocetin cofactor (VWF:RCo), von Willebrand antigen (VWF:Ag) or factor VIII procoagulant activity (FVIII:C) measured at time of enrollment, even after adjustment for age, sex and blood group (P < 0.001 for all the four upper quintiles of BS vs. the first quintile, for either VWF:RCo, VWF:Ag or FVIII:C). Higher BS was related with increasing likelihood of VWD, and a mucocutaneous BS (computed from spontaneous, mucocutaneous symptoms) was strongly associated with bleeding after surgery or tooth extraction. CONCLUSIONS: Quantitative analysis of bleeding symptoms is potentially useful for a more accurate diagnosis of type 1 VWD and to develop guidelines for its optimal treatment.
BACKGROUND: A quantitative description of bleeding symptoms in type 1 von Willebrand disease (VWD) has never been reported. OBJECTIVES: The aim was to quantitatively evaluate the severity of bleeding symptoms in type 1 VWD and its correlation with clinical and laboratory features. PATIENTS AND METHODS: Bleeding symptoms were retrospectively recorded in a European cohort of VWD type 1 families, and for each subject a quantitative bleeding score (BS) was obtained together with phenotypic tests. RESULTS: A total of 712 subjects belonging to 144 families and 195 controls were available for analysis. The BS was higher in index cases than in affected family members (BS 9 vs. 5, P < 0.0001) and in unaffected family members than in controls (BS 0 vs. -1, P < 0.0001). There was no effect of ABO blood group. BS showed a strong significant inverse relation with either von Willebrand ristocetin cofactor (VWF:RCo), von Willebrand antigen (VWF:Ag) or factor VIII procoagulant activity (FVIII:C) measured at time of enrollment, even after adjustment for age, sex and blood group (P < 0.001 for all the four upper quintiles of BS vs. the first quintile, for either VWF:RCo, VWF:Ag or FVIII:C). Higher BS was related with increasing likelihood of VWD, and a mucocutaneous BS (computed from spontaneous, mucocutaneous symptoms) was strongly associated with bleeding after surgery or tooth extraction. CONCLUSIONS: Quantitative analysis of bleeding symptoms is potentially useful for a more accurate diagnosis of type 1 VWD and to develop guidelines for its optimal treatment.
Authors: Daniel B Bellissimo; Pamela A Christopherson; Veronica H Flood; Joan Cox Gill; Kenneth D Friedman; Sandra L Haberichter; Amy D Shapiro; Thomas C Abshire; Cindy Leissinger; W Keith Hoots; Jeanne M Lusher; Margaret V Ragni; Robert R Montgomery Journal: Blood Date: 2011-12-23 Impact factor: 22.113
Authors: Veronica H Flood; Joan Cox Gill; Kenneth D Friedman; Daniel B Bellissimo; Sandra L Haberichter; Robert R Montgomery Journal: Semin Thromb Hemost Date: 2011-11-18 Impact factor: 4.180
Authors: V H Flood; J C Gill; P A Christopherson; J S Wren; K D Friedman; S L Haberichter; R G Hoffmann; R R Montgomery Journal: J Thromb Haemost Date: 2012-07 Impact factor: 5.824
Authors: V H Flood; J C Gill; P A Christopherson; D B Bellissimo; K D Friedman; S L Haberichter; S R Lentz; R R Montgomery Journal: J Thromb Haemost Date: 2012-07 Impact factor: 5.824
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: E Berntorp; B Fuchs; M Makris; R Montgomery; V Flood; J S O'Donnell; A B Federici; D Lillicrap; P James; U Budde; M Morfini; P Petrini; S Austin; C Kannicht; V Jiménez-Yuste; C Lee Journal: Haemophilia Date: 2013-03 Impact factor: 4.287