Markers of hypercoagulability in patients with hemophilia B given repeated, large doses of factor IX concentrates during and after surgery.
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.
Santagostino
E
E
Angelo Bianchi Bonomi Hemophilia and Thrombosis Center, IRCCS Maggiore Hospital, Milan, Italy.
Mannucci
P M
PM
Gringeri
A
A
Tagariello
G
G
Baudo
F
F
Bauer
K A
KA
Rosenberg
R D
RD
D000293:Adolescent; D000328:Adult; D015415:Biomarkers / Q000097:blood; D001778:Blood Coagulation Disorders / Q000097:blood / Q000209:etiology*; D002648:Child; D004334:Drug Administration Schedule; D005164:Factor IX / Q000009:adverse effects*; D002836:Hemophilia B / Q000097:blood / Q000188:drug therapy*; D006801:Humans; D007262:Infusions, Intravenous; D007430:Intraoperative Care; D008875:Middle Aged; D011182:Postoperative Care; D011446:Prospective Studies; D013514:Surgical Procedures, Operative
D015415:Biomarkers; D005164:Factor IX