An illustrative case and a review on the dosing of recombinant factor VIIa in congenital factor XI deficiency

Haemophilia : the Official Journal of the World Federation of Hemophilia
S Schulman, G Németh

Abstract

Recombinant activated factor VII (rFVIIa) has been used in a very limited number of patients with severe factor XI (FXI) deficiency. The dose and duration of treatment has varied greatly between these case reports. In a few of these cases there was also evidence of thrombotic complications. We present here a report on one additional patient with congenital FXI deficiency. For two major orthopaedic procedures in this patient we used rFVIIa as a single bolus dose followed by continuous infusion at a low rate. The data from these treatment episodes, together with those from a review of the published cases, lend support to the concept of using much lower doses than in haemophilia with inhibitors. A bolus dose of 20 microg kg(-1) and thereafter maintenance of the FVII activity at approximately 3 IU mL(-1) appears effective and safe.

References

Aug 1, 1992·Blood Coagulation & Fibrinolysis : an International Journal in Haemostasis and Thrombosis·S BerlinerU Seligsohn
Oct 29, 1991·Biochemistry·E W DavieW Kisiel
Sep 10, 1994·Lancet·P H Bolton-MaggsG S Lucas
Jan 5, 1999·Haemophilia : the Official Journal of the World Federation of Hemophilia·S Schulman
Oct 31, 2001·Blood Coagulation & Fibrinolysis : an International Journal in Haemostasis and Thrombosis·S BillonJ F Abgrall
Apr 16, 2002·Haemophilia : the Official Journal of the World Federation of Hemophilia·P LawlerC A Lee
Jul 23, 2003·Journal of Thrombosis and Haemostasis : JTH·O SalomonU Seligsohn
Oct 22, 2003·Blood Coagulation & Fibrinolysis : an International Journal in Haemostasis and Thrombosis·Niamh M O'Connell
Feb 12, 2004·Seminars in Hematology·Ulla Hedner
Jan 22, 2005·Haemophilia : the Official Journal of the World Federation of Hemophilia·M M BernW Mitchell
Jul 14, 2005·Haemophilia : the Official Journal of the World Federation of Hemophilia·S A Brown

❮ Previous
Next ❯

Citations

Jul 18, 2009·Annals of Hematology·Massimo FranchiniGiuseppe Lippi
May 26, 2012·Obstetrical & Gynecological Survey·Ryan G StewardThomas M Price
Dec 17, 2009·Hematology·Paula H B Bolton-Maggs
Apr 16, 2009·Blood Coagulation & Fibrinolysis : an International Journal in Haemostasis and Thrombosis·Ioannis VasileiadisAnastasia Kotanidou
May 18, 2011·Blood Coagulation & Fibrinolysis : an International Journal in Haemostasis and Thrombosis·Rita SantoroPiergiorgio Iannaccaro
Jun 3, 2009·Haemophilia : the Official Journal of the World Federation of Hemophilia·G KenetU Martinowitz
Jun 28, 2008·Haemophilia : the Official Journal of the World Federation of Hemophilia·C-E DempfleM Borggrefe
Apr 4, 2008·Haemophilia : the Official Journal of the World Federation of Hemophilia·N M O'ConnellC A Lee
Apr 26, 2014·Haemophilia : the Official Journal of the World Federation of Hemophilia·P JamesF Peyvandi
Dec 4, 2008·Clinical and Applied Thrombosis/hemostasis : Official Journal of the International Academy of Clinical and Applied Thrombosis/Hemostasis·Monica Martín-SalcesFernando Hernández-Navarro
Dec 28, 2007·Clinical and Applied Thrombosis/hemostasis : Official Journal of the International Academy of Clinical and Applied Thrombosis/Hemostasis·Christoph SuckerAndrea Gerhardt
Aug 5, 2018·Blood Reviews·Benjamin F TillmanDavid Gailani

❮ Previous
Next ❯

Related Concepts

Related Feeds

Blood Clotting Disorders

Thrombophilia includes conditions with increased tendency for excessive blood clotting. Blood clotting occurs when the body has insufficient amounts of specialized proteins that make blood clot and stop bleeding. Here is the latest research on blood clotting disorders.