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Keywords:

  • von Willebrand factor;
  • von Willebrand disease;
  • VWF propeptide ratio;
  • VWF survival;
  • type Vicenza VWD

Summary

Reduced von Willebrand factor (VWF) half-life has been suggested as a new pathogenic mechanism in von Willebrand disease (VWD). The usefulness of VWF propeptide (VWFpp) in exploring VWF half-life was assessed in 22 type 1 and 14 type Vicenza VWD patients, and in 30 normal subjects, by comparing the findings on post-Desmopressin (DDAVP) VWF t1/2 elimination (t1/2el). The VWFpp/VWF antigen ratio (VWFpp ratio) was dramatically increased in type Vicenza VWD (13·02 ± 0·49) when compared to normal subjects (1·45 ± 0·06), whereas it appeared to be normal in all type 1 VWD patients (1·56 ± 0·7), except for the four carrying the C1130F mutation (4·69 ± 0·67). A very short VWF t1/2el was found in type Vicenza VWD (1·3 ± 0·2 h), while all type 1 VWD patients had a t1/2el similar to that of the controls (11·6 ± 1·4 and 15·4 ± 2·5 h respectively), except for the four patients carrying the C1130F mutation, who had a significantly shorter VWF survival (4·1 ± 0·2 h). A significant inverse correlation emerged between VWFpp ratio and VWF t1/2el in both VWD patients and normal subjects. The VWFpp ratio thus seemed very useful for distinguishing between type 1 VWD cases with a normal and a reduced VWF survival, as well as for identifying type Vicenza VWD.