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A quantitative analysis of bleeding symptoms in type 1 von Willebrand disease: results from a multicenter European study (MCMDM-1 VWD)

Tosetto, A; Rodeghiero, F; Castaman, G; Goodeve, A; Federici, AB; Batlle, J; Meyer, D; Fressinaud, E; Mazurier, C and Goudemand, J, et al. (2006) In Journal of Thrombosis and Haemostasis 4(4). p.766-773
Abstract
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... (More)
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. (Less)
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Contribution to journal
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published
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keywords
von Willebrand disease diagnosis, von Willebrand disease, bleeding score, inherited bleeding disorders
in
Journal of Thrombosis and Haemostasis
volume
4
issue
4
pages
766 - 773
publisher
Federation of European Neuroscience Societies and Blackwell Publishing Ltd
external identifiers
  • wos:000235938700012
  • pmid:16634745
  • scopus:33644977050
ISSN
1538-7933
DOI
10.1111/j.1538-7836.2006.01847.x
language
English
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yes
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917e9df2-c39e-4b86-88d5-27fdc1492924 (old id 416336)
date added to LUP
2007-10-20 12:02:15
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2019-07-16 01:44:12
@article{917e9df2-c39e-4b86-88d5-27fdc1492924,
  abstract     = {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 &lt; 0.0001) and in unaffected family members than in controls (BS 0 vs. -1, P &lt; 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 &lt; 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.},
  author       = {Tosetto, A and Rodeghiero, F and Castaman, G and Goodeve, A and Federici, AB and Batlle, J and Meyer, D and Fressinaud, E and Mazurier, C and Goudemand, J and Eikenboom, J and Schneppenheim, R and Budde, U and Ingerslev, J and Vorlova, Z and Habart, D and Holmberg, Lars and Lethagen, Stefan and Pasi, J and Hill, F and Peake, I},
  issn         = {1538-7933},
  keyword      = {von Willebrand disease diagnosis,von Willebrand disease,bleeding score,inherited bleeding disorders},
  language     = {eng},
  number       = {4},
  pages        = {766--773},
  publisher    = {Federation of European Neuroscience Societies and Blackwell Publishing Ltd},
  series       = {Journal of Thrombosis and Haemostasis},
  title        = {A quantitative analysis of bleeding symptoms in type 1 von Willebrand disease: results from a multicenter European study (MCMDM-1 VWD)},
  url          = {http://dx.doi.org/10.1111/j.1538-7836.2006.01847.x},
  volume       = {4},
  year         = {2006},
}