Heart transplant in a factor VIII-deficient patient with a high-titre inhibitor: perioperative management using high-dose continuous infusion factor VIII and recombinant factor VIIa

Haemophilia ◽  
2001 ◽  
Vol 7 (2) ◽  
pp. 227-232 ◽  
Author(s):  
S. Sheth ◽  
D. Dimichele ◽  
M. Lee ◽  
J. Lamour ◽  
J. Quaegebeur ◽  
...  
2002 ◽  
Vol 36 (5) ◽  
pp. 882-891 ◽  
Author(s):  
Joan M Stachnik ◽  
Michael P Gabay

OBJECTIVE: To review clinical information related to the use of continuous infusion factor products in patients with hemophilia. Specifically, case reports and open-label trials are summarized involving the use of factor VIII and recombinant factor VIIa for a variety of indications including surgical prophylaxis, acute bleeding, primary prevention, and management of inhibitors. In addition, issues surrounding the use of continuous infusion of factor products such as pharmacokinetic rationale, stability/sterility, and cost are reviewed. DATA SOURCES: Primary and review articles were identified through a MEDLINE search (1990–June 2001) and through secondary sources. STUDY SELECTION AND DATA EXTRACTION: All articles identified from the data sources were evaluated, and all information deemed relevant was included in this review. DATA SYNTHESIS: Data concerning the administration of factor products are primarily detailed in open-label trials and case reports. Comparisons between intermittent bolus injections and continuous infusion of factor products are limited and primarily compare continuous infusion regimens with historical controls. The rationale behind the continuous-infusion approach is linked to the pharmacokinetics of factor products administered via this route. Pharmacokinetic data reveal that, with continuous infusion of factor products, a reduction in clearance and a maintenance of factor serum concentrations are noted. CONCLUSIONS: Administration of factor products (factor VIII and recombinant factor VIIa) via continuous infusion has produced favorable hemostatic effects compared with intermittent bolus injections. The advantages of continuous infusion include maintenance of a constant factor concentration, thereby reducing risk of bleeding from excessively low trough concentrations, and a decrease in factor consumption related to a reduction in factor clearance with constant infusion. Manufacturers recommend using reconstituted factor products either immediately or within 1–3 hours after reconstitution; however, several studies have found the products to be stable and sterile for longer periods.


2000 ◽  
Vol 99 (1) ◽  
pp. 21-24 ◽  
Author(s):  
Francesco Baudo ◽  
Rosaria Redaelli ◽  
Teresa Maria Caimi ◽  
Giovanni Mostarda ◽  
Gabriella Somaini ◽  
...  

2020 ◽  
Vol 4 (5) ◽  
pp. 855-857
Author(s):  
Matthew S. Evans ◽  
Charles Davis ◽  
M. Elaine Eyster

Key Points Prophylaxis with emicizumab was used in a patient with severe hemophilia with an inhibitor who underwent knee surgery. Use of emicizumab during surgery led to less recombinant factor VIIa and less bleeding, in addition to cost savings, for this patient.


2005 ◽  
Vol 94 (12) ◽  
pp. 1177-1180 ◽  
Author(s):  
Geir E. Tjønnfjord ◽  
Richard Wallensten ◽  
Uri Martinowitz ◽  
Gili Kenet ◽  
Sam Schulman

SummaryThe administration of recombinant activated factor VII (rFVIIa) by continuous infusion has provided a safe and convenient alternative to bolus injections in haemophiliacs with inhibitors, but it has only been reported in a single case with congenital factorVII (FVII) deficiency. The results of 12 consecutive surgical procedures in 7 patients with congenital FVII deficiency are reported here. rFVIIa was always given in continuous infusion, aiming at plasma FVII activity of 0.5 IU/mL. Treatment was given for 2 to 7 days with a mean total dose of 7.8 mg rFVIIa. Blood loss was as expected from the different types of procedures and the only thromboembolic complication was a superficial thrombophlebitis at the infusion site. This mode of substitution was therefore safe, effective and well tolerated.


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