Von Willebrand factor propeptide makes it easy to identify the shorter Von Willebrand factor survival in patients with type 1 and type Vicenza von Willebrand disease.
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 t(1/2) elimination (t(1/2el)). 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 t(1/2el) was found in type Vicenza VWD (1.3 +/- 0.2 h), while all type 1 VWD patients had a t(1/2el) 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 t(1/2el) 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.
Sztukowska
M
M
Department of Medical and Surgical Sciences, Second Chair of Internal Medicine, University of Padua Medical School, Padua, Italy.
Gallinaro
L
L
Cattini
M G
MG
Pontara
E
E
Sartorello
F
F
Daidone
V
V
Padrini
R
R
Pagnan
A
A
Casonato
A
A
D016022:Case-Control Studies; D004252:DNA Mutational Analysis; D003894:Deamino Arginine Vasopressin; D006207:Half-Life; D006490:Hemostatics; D006801:Humans; D009154:Mutation; D011498:Protein Precursors / Q000235:genetics / Q000378:metabolism*; D014842:von Willebrand Diseases / Q000097:blood / Q000145:classification* / Q000235:genetics; D014841:von Willebrand Factor / Q000032:analysis / Q000235:genetics / Q000378:metabolism*
D006490:Hemostatics; D011498:Protein Precursors; D014841:von Willebrand Factor; D003894:Deamino Arginine Vasopressin