Congenital haemophilia A with inhibitor treated with recombinant factor VIIa in an infusion pump

Congenital haemophilia A is a rare disorder where low factor VIII activity is the result of gene defect.Human coagulation factor VIII concentrate is used to prevent bleeding or for therapy.The main therapy-related complication is development of an inhibitor against Factor VIII.Inhibitors are alloantibodies which neutralize procoagulant activity of exogenous factor VIII during replacement therapy.Bypassing agents are then the treatment of choice and can substitute factor VIII in maintaining haemostasis.Recombinant factor VIIa and activated prothrombin complex concentrates are of comparable effectiveness.Significant numbers of haemophilia A patients with inhibitor are treated with recombinant factor VIIa.So far, it has been administered in bolus injections every 2 to 12 hours.Infusion pump is a promising alternative to bolus injections.Delivery of recombinant factor VIIa in an infusion pump has been proved effective in terms of maintaining accurate drug levels as well as minimizing human errors by reducing burden on staff.

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Publication Details

Journal
Journal of Transfusion Medicine
Published
2024-09-30
DOI
https://doi.org/10.5603/jtm.103131
Primary Topic
Hemophilia Treatment and Research
Type
article
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article

Congenital haemophilia A with inhibitor treated with recombinant factor VIIa in an infusion pump

Michał Witkowski, Wiktoria Ryżewska, Tadeusz Robak
Journal of Transfusion Medicine
Hemophilia Treatment and Research
article

Congenital haemophilia A with inhibitor treated with recombinant factor VIIa in an infusion pump

Michał Witkowski, Wiktoria Ryżewska, Tadeusz Robak
article en

Abstract

Congenital haemophilia A is a rare disorder where low factor VIII activity is the result of gene defect.Human coagulation factor VIII concentrate is used to prevent bleeding or for therapy.The main therapy-related complication is development of an inhibitor against Factor VIII.Inhibitors are alloantibodies which neutralize procoagulant activity of exogenous factor VIII during replacement therapy.Bypassing agents are then the treatment of choice and can substitute factor VIII in maintaining haemostasis.Recombinant factor VIIa and activated prothrombin complex concentrates are of comparable effectiveness.Significant numbers of haemophilia A patients with inhibitor are treated with recombinant factor VIIa.So far, it has been administered in bolus injections every 2 to 12 hours.Infusion pump is a promising alternative to bolus injections.Delivery of recombinant factor VIIa in an infusion pump has been proved effective in terms of maintaining accurate drug levels as well as minimizing human errors by reducing burden on staff.

Journal of Transfusion MedicineVol. 17(3)
Medical University of Warsaw (PL), Copernicus Memorial Hospital (PL)
Zero hunger
Openalex Percentile: Top 66%
Hemophilia Treatment and Research
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