Markers of Hypercoagulability in Patients with Hemophilia B Given Repeated, Large Doses of Factor IX Concentrates during and after Surgery

E Santagostino; P M Mannucci; A Gringeri; G Tagariello; F Baudo; K A Bauer; R D Rosenberg

doi:10.1055/s-0038-1642515

Thrombosis and Haemostasis, Inhaltsverzeichnis

Thromb Haemost 1994; 71(06): 737-740
DOI: 10.1055/s-0038-1642515

Review Article

Schattauer GmbH Stuttgart

Markers of Hypercoagulability in Patients with Hemophilia B Given Repeated, Large Doses of Factor IX Concentrates during and after Surgery

Authors

E Santagostino

¹Angelo Bianchi Bonomi Hemophilia and Thrombosis Center and Institute of Internal Medicine, IRCCS Maggiore Hospital and University of Milan, Italy

Department of Medicine, Beth Israel Hospital and Harvard Medical School Boston, MA, USA
P M Mannucci

¹Angelo Bianchi Bonomi Hemophilia and Thrombosis Center and Institute of Internal Medicine, IRCCS Maggiore Hospital and University of Milan, Italy

Department of Medicine, Beth Israel Hospital and Harvard Medical School Boston, MA, USA
A Gringeri

¹Angelo Bianchi Bonomi Hemophilia and Thrombosis Center and Institute of Internal Medicine, IRCCS Maggiore Hospital and University of Milan, Italy

Department of Medicine, Beth Israel Hospital and Harvard Medical School Boston, MA, USA
G Tagariello

²Transfusion Center, Castelfranco, Veneto, Italy

Department of Medicine, Beth Israel Hospital and Harvard Medical School Boston, MA, USA
F Baudo

³Hematology Division, Ca Granda Hospital, Milan, Italy

Department of Medicine, Beth Israel Hospital and Harvard Medical School Boston, MA, USA
K A Bauer

⁴Charles A. Dana Research Institute and the Harward-Thorndike Laboratory, Boston, MA, USA

Department of Medicine, Beth Israel Hospital and Harvard Medical School Boston, MA, USA
R D Rosenberg

⁴Charles A. Dana Research Institute and the Harward-Thorndike Laboratory, Boston, MA, USA

Department of Medicine, Beth Israel Hospital and Harvard Medical School Boston, MA, USA

Abstract

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Summary

Purer factor IX (FIX) concentrates have been produced for the treatment of hemophilia B in the attempt to reduce the risk of thrombotic complications associated with the use of prothrombin complex concentrates. To evaluate ex vivo whether or not FIX concentrates activate the coagulation system in conditions associated with a high risk for thrombosis, we measured markers of hypercoagulability in 10 patients with hemophilia B who underwent surgery, mainly orthopedic procedures, covered by multiple concentrate infusions (40-80 U/kg/day). Postinfusion plasma levels of prothrombin fragment 1+2 and factor X activation peptide did not differ significantly from the presurgical levels, neither before nor after each concentrate dose. Therefore, it appears that prolonged treatment of patients with hemophilia B undergoing high risk surgical procedures with high doses of FIX concentrate does not cause systemic activation of coagulation. This suggests that purified FIX concentrates are preferable to prothrombin complex concentrates for conditions associated with an increased risk of thrombosis.

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Referenzen

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