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Thromb Haemost 1993; 70(02): 270-272
DOI: 10.1055/s-0038-1649564
Original Articles
Clinical Studies
Schattauer GmbH Stuttgart

Pharmacokinetics of Monoclonally-Purified and Recombinant Factor VIII in Patients with Severe von Willebrand Disease

M Morfini
1   The Hematology Department and Hemophilia Center, University of Florence, Italy
,
P M Mannucci
2   The Angelo Bianchi Bonomi Hemophilia and Thrombosis Center, IRCCS Maggiore Hospital and University of Milan, Italy
,
P M Tenconi
2   The Angelo Bianchi Bonomi Hemophilia and Thrombosis Center, IRCCS Maggiore Hospital and University of Milan, Italy
,
G Longo
1   The Hematology Department and Hemophilia Center, University of Florence, Italy
,
M G Mazzucconi
3   The Department of Biopathology (Hematology Section), University of Rome, Italy
,
F Rodeghiero
4   The Hemophilia and Thrombosis Center, Vicenza, Italy
,
N Ciavarella
5   The Hemophilia and Thrombosis Center, Bari, Italy
,
V De Rosa
6   The Angiology Division and Hemophilia Center, University of Bologna, Italy
,
A Arter
7   Baxter Healthcare, Hyland Division, Glendale, California, USA
› Author Affiliations
Further Information

Publication History

Received 15 December 1992

Accepted after revision 19 February 1993

Publication Date:
04 July 2018 (online)

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Summary

A monoclonally-purified factor VIII (FVIII) concentrate, containing little von Willebrand factor (vWF), was infused to 11 patients with severe von Willebrand disease and unmeasurable levels of plasma vWF. In comparison with the historical data obtained infusing hemophiliacs in the same conditions, monoclonally-purified FVIII had a significantly shorter half-life and faster clearance from plasma but similar in vivo recovery and volume of distribution. Two additional patients with severe von Willebrand disease were also infused with recombinant FVIII totally devoid of vWF. Half-life was very short and in vivo recovery low, with a larger volume of distribution than for monoclonally-purified FVIII. We conclude that in patients with severe von Willebrand disease the small amounts of vWF contained in the monoclonally-purified FVIII concentrate are not sufficient to stabilize infused FVIII, nor to support the normal circulation of endogenous FVIII that these patients produce at a normal rate.

 

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