Abstract |
Von willebrand disease (VWD) is a common inherited bleeding disorder. The diagnosis may need a large panel of tests that differ in term of sensibility and specificity, and because of the effect of multifactorial modifiers (genetic or environmental); there is difficulty in defining diagnostic limits. We performed a panel of tests on 19 patients suffering from recurrent bleeding, to diagnose and classify VWD subtypes, by introducing the von Willebrand factor (VWF) collagen binding test (VWF:CB), then comparing the results with the activity of VWF risticetin cofactor (VWF:RCo) and multimer pattern. We considered 30% limit rate of VWF, as described by many authors, to make the diagnosis of VWD. The diagnosis of type 1 of VWD has been confirmed in 7patients, subtype 2A in 2 patients, subtype 2M in 2 patients and type 3 in 2 patients. We also defined a new group of 6 patients named "uncertain type 1" that didn't fill into the type 1 diagnostic criterion. The comparison between VWF:CB and VWF:RCo showed good correlation for all types of VWD except for type 2 while comparison between VWF:CB and multimer pattern showed good concordance for all types of VWD diagnosed. In conclusion, VWF:CB can be a good alternative to VWF:RCo for the diagnosis of quantitative deficiencies of VWF. It can also replace the multimer pattern study. However, the introduction of VWF:CB didn't help in the diagnosis of the "uncertain type 1" group of patients and cannot be a replacement for qualitative defect.
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Authors | Meriem Yasmine Ferhat-Hamida, Houda Boukerb, Ghania Hariti |
Journal | Annales de biologie clinique
(Ann Biol Clin (Paris))
2015 Jul-Aug
Vol. 73
Issue 4
Pg. 461-8
ISSN: 1950-6112 [Electronic] France |
PMID | 26411913
(Publication Type: Journal Article)
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Chemical References |
- von Willebrand Factor
- Collagen
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Topics |
- Adolescent
- Adult
- Collagen
(metabolism)
- Female
- Hematologic Tests
- Humans
- Male
- von Willebrand Diseases
(blood, classification, diagnosis)
- von Willebrand Factor
(analysis)
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