Pharmacokinetic Studies with FVIII/von Willebrand Factor Concentrate Can Be a Diagnostic Tool to Distinguish between Subgroups of Patients with Acquired von Willebrand Syndrome

J. Luboshitz; A. Lubetsky; L. Schliamser; A. Kotler; I. Tamarin; A. Inbal

doi:10.1055/s-0037-1615752

Thrombosis and Haemostasis, Table of Contents

Thromb Haemost 2001; 85(05): 806-809
DOI: 10.1055/s-0037-1615752

Review Article

Schattauer GmbH

Pharmacokinetic Studies with FVIII/von Willebrand Factor Concentrate Can Be a Diagnostic Tool to Distinguish between Subgroups of Patients with Acquired von Willebrand Syndrome

J. Luboshitz^*

¹Institute of Thrombosis and Hemostasis, Sheba Medical Center, Tel Hashomer, Tel-Aviv University

,

A. Lubetsky

¹Institute of Thrombosis and Hemostasis, Sheba Medical Center, Tel Hashomer, Tel-Aviv University

,

L. Schliamser

²Bnei-Zion Medical Center

,

A. Kotler

³Carmel Medical Center, Haifa, Israel

,

I. Tamarin

¹Institute of Thrombosis and Hemostasis, Sheba Medical Center, Tel Hashomer, Tel-Aviv University

,

A. Inbal

¹Institute of Thrombosis and Hemostasis, Sheba Medical Center, Tel Hashomer, Tel-Aviv University

› Author Affiliations

Abstract

Summary

Acquired von Willebrand syndrome (AVWS) has been associated mainly with monoclonal gammopathy of uncertain significance (MGUS), clonal lymphoproliferative or myeloproliferative disorders and autoimmunity. In the present work we studied 6 patients with AVWS: four with MGUS IgG (λ or κ), one with small lymphocytic lymphoma and one with agnogenic myeloid metaplasia (AMM). All the patients underwent a pharmacokinetic analysis at presentation in order to study potential differences in recovery, clearance (CL) or terminal half-life (THL) following administration of von Willebrand factor (VWF) concentrate. In all the patients with AVWS an increase in clearance and a decrease in THL was observed as compared to these parameters in patients with hereditary type 3 von Willebrand disease (VWD). No difference in recovery was observed among the groups. The increase in clearance and the decrease in THL were significantly more pronounced in the group of MGUS patients (57.93 ± 25.6 ml/h/kg, and 1.39 ± 0.5 h, respectively) as compared to these parameters in the AMM (8.06 ml/h/kg, and 6.96 h, respectively) or the lymphoma (4.76 ml/h/kg, and 6.76 h, respectively) patients (p = 0.03 for clearance and 0.001 for THL). These data indicate that the pharmacokinetic analysis can be a useful tool to distinguish between MGUS-related and other causes of AVWS, and to plan an appropriate treatment accordingly.

Keywords

Acquired von Willebrand - pharmacokinetics

Full Text

References

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