A quantitative analysis of bleeding symptoms in type 1 von Willebrand disease: results from a multicenter European study (MCMDM-1 VWD)
(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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https://lup.lub.lu.se/record/416336
- author
- organization
- publishing date
- 2006
- type
- Contribution to journal
- publication status
- published
- subject
- 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
- Wiley-Blackwell
- external identifiers
-
- wos:000235938700012
- pmid:16634745
- scopus:33644977050
- ISSN
- 1538-7933
- DOI
- 10.1111/j.1538-7836.2006.01847.x
- language
- English
- LU publication?
- yes
- additional info
- The information about affiliations in this record was updated in December 2015. The record was previously connected to the following departments: Emergency medicine/Medicine/Surgery (013240200), Paediatrics (Lund) (013002000)
- id
- 917e9df2-c39e-4b86-88d5-27fdc1492924 (old id 416336)
- date added to LUP
- 2016-04-01 12:31:00
- date last changed
- 2022-04-21 08:27:05
@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 < 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.}},
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}},
keywords = {{von Willebrand disease diagnosis; von Willebrand disease; bleeding score; inherited bleeding disorders}},
language = {{eng}},
number = {{4}},
pages = {{766--773}},
publisher = {{Wiley-Blackwell}},
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}},
doi = {{10.1111/j.1538-7836.2006.01847.x}},
volume = {{4}},
year = {{2006}},
}