The reduction of large von Willebrand factor multimers in plasma in essential thrombocythaemia is related to the platelet count

Authors

  • Perry J. J. Van Genderen,

    1. 1 Department of Haematology, University Hospital Dijkzigt, 3Institute of Epidemiology and Biostatistics, Erasmus University, Rotterdam, The Netherlands, 2Blood Transfusion Service, AK Harburg, Hamburg, Germany
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  • Ulrich Budde,

    1. 1 Department of Haematology, University Hospital Dijkzigt, 3Institute of Epidemiology and Biostatistics, Erasmus University, Rotterdam, The Netherlands, 2Blood Transfusion Service, AK Harburg, Hamburg, Germany
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  • Jan J. Michiels,

    1. 1 Department of Haematology, University Hospital Dijkzigt, 3Institute of Epidemiology and Biostatistics, Erasmus University, Rotterdam, The Netherlands, 2Blood Transfusion Service, AK Harburg, Hamburg, Germany
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  • Roel Van Strik,

    1. 1 Department of Haematology, University Hospital Dijkzigt, 3Institute of Epidemiology and Biostatistics, Erasmus University, Rotterdam, The Netherlands, 2Blood Transfusion Service, AK Harburg, Hamburg, Germany
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  • Huub H. D. M. Van Vliet

    1. 1 Department of Haematology, University Hospital Dijkzigt, 3Institute of Epidemiology and Biostatistics, Erasmus University, Rotterdam, The Netherlands, 2Blood Transfusion Service, AK Harburg, Hamburg, Germany
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Dr Jan J. Michiels Department of Haematology, University Hospital Dijkzigt, Dr Molewaterplein 40, 3015 GD Rotterdam, The Netherlands.

Abstract

We have investigated the relationship between platelet count and large VWF (von Willebrand factor) multimers in the plasma of 36 patients with essential thrombocythaemia (ET) and 26 patients with reactive thrombocytosis (RT). In both ET and RT patients an inverse relationship could be established between platelet count and large VWF multimers in plasma as well in relatively decreased ristocetin cofactor/von Willebrand factor antigen and collagen binding activity/von Willebrand factor antigen ratios. A normalization of the platelet count was accompanied by restoration of a normal plasma VWF multimeric distribution. Our data suggest that increasing numbers of platelets circulating in blood result in increased removal of large VWF multimers from plasma.

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