Abstract |
Measurement of the von Willebrand factor (vWF) propeptide, also known as von Willebrand antigen II, has been suggested to be helpful in the discrimination of congenital von Willebrand disease type I from type 2 and in assessing the extent of activation of the endothelium. We performed a quantitative analysis of mature vWF and its propeptide in plasma in 8 patients with acquired von Willebrand syndrome (AvWS) and in 20 normal individuals. Mature vWF levels were significantly lower in AvWS as compared with normal individuals (13.4+/-3.5 vs 35.6+/-3.3 nM, p <0.001). In contrast, propeptide levels were significantly higher in AvWS (11.4+/-1.1 vs 4.7+/-0.2 nM, p <0.001), probably reflecting a compensatory increase in vWF synthesis or increased perturbation of the endothelium in AvWS. After treatment with DDAVP, propeptide and mature vWF levels rose 5-fold in AvWS, whereas propeptide levels were not altered by the infusion of a vWF concentrate or treatment with high dose intravenous immunoglobulins, indicating that plasma propeptide levels are a reliable reflection of vWF synthesis. Measurement of propeptide levels may provide additional information in AvWS as to whether decreased levels of mature vWF in the circulation are due to a decrease in synthesis or due to an accelerated removal of vWF from the circulation.
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Authors | P J van Genderen, R C Boertjes, J A van Mourik |
Journal | Thrombosis and haemostasis
(Thromb Haemost)
Vol. 80
Issue 3
Pg. 495-8
(Sep 1998)
ISSN: 0340-6245 [Print] Germany |
PMID | 9759633
(Publication Type: Journal Article, Research Support, Non-U.S. Gov't)
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Chemical References |
- Protein Precursors
- von Willebrand Factor
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Topics |
- Adult
- Aged
- Female
- Humans
- Male
- Middle Aged
- Protein Precursors
(blood)
- von Willebrand Diseases
(blood)
- von Willebrand Factor
(metabolism)
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