The factor VIII/von Willebrand factor ratio discriminates between reduced synthesis and increased clearance of von Willebrand factor

It is often stated that a decrease in Von Willebrand factor (VWF), the carrier protein of factor VIII, results in a concordant change in factor VIII. Clinical data suggest that this is not always the case and we hypothesized that the ratio between factor VIII and VWF depends on the genetic defect th...

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Published inThrombosis and haemostasis Vol. 87; no. 2; p. 252
Main Authors Eikenboom, Jeroen C J, Castaman, Giancarlo, Kamphuisen, Pieter W, Rosendaal, Frits R, Bertina, Rogier M
Format Journal Article
LanguageEnglish
Published Germany 01.02.2002
Subjects
Alleles
Antigens - analysis
Factor VIII - analysis
Female
Gene Deletion
Gene Expression
Genotype
Humans
Male
Metabolic Clearance Rate
Mutation
Platelet Glycoprotein GPIb-IX Complex - metabolism
von Willebrand Diseases - blood
von Willebrand Diseases - classification
von Willebrand Diseases - genetics
von Willebrand Factor - genetics
von Willebrand Factor - metabolism
von Willebrand Factor - secretion
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Summary:It is often stated that a decrease in Von Willebrand factor (VWF), the carrier protein of factor VIII, results in a concordant change in factor VIII. Clinical data suggest that this is not always the case and we hypothesized that the ratio between factor VIII and VWF depends on the genetic defect that causes the VWF deficiency. We have analyzed the ratio between plasma factor VIII and VWF in several subtypes of Von Willebrand Disease and we show that the ratio is increased when VWF synthesis is reduced, but that the ratio remains one when VWF clearance is increased. These observations could be of clinical importance as an increased factor VIII/VWF ratio in combination with a borderline VWF level may indicate the presence of a true genetic defect, possibly a VWF null allele.
ISSN:0340-6245
DOI:10.1055/s-0037-1612981