The Factor VIII/Von Willebrand Factor Ratio Discriminates between Reduced Synthesis and Increased Clearance of Von Willebrand Factor

Jeroen C. J. Eikenboom; Giancarlo Castaman; Pieter W. Kamphuisen; Frits R. Rosendaal; Rogier M. Bertina

doi:10.1055/s-0037-1612981

Thrombosis and Haemostasis, Table of Contents

Thromb Haemost 2002; 87(02): 252-257
DOI: 10.1055/s-0037-1612981

Letters to the Editor

Schattauer GmbH

The Factor VIII/Von Willebrand Factor Ratio Discriminates between Reduced Synthesis and Increased Clearance of Von Willebrand Factor

Jeroen C. J. Eikenboom

¹Department of Hematology, Hemostasis and Thrombosis Research Center, Leiden, The Netherlands

,

Giancarlo Castaman

³Hemophilia and Thrombosis Center, Department of Hematology, San Bortolo Hospital, Vicenza, Italy

,

Pieter W. Kamphuisen

¹Department of Hematology, Hemostasis and Thrombosis Research Center, Leiden, The Netherlands

,

Frits R. Rosendaal

¹Department of Hematology, Hemostasis and Thrombosis Research Center, Leiden, The Netherlands

²Department of Clinical Epidemiology, Leiden University Medical Center, Leiden, The Netherlands

,

Rogier M. Bertina

¹Department of Hematology, Hemostasis and Thrombosis Research Center, Leiden, The Netherlands

› Author Affiliations

Abstract

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.

Keywords

Factor VIII - Willebrand - mutations - blood group - null alleles

Full Text

References

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