Recombinant FVIIa in elective non‐orthopaedic surgery of adults with haemophilia and inhibitors: A systematic literature review

Dolan, Gerard; Astermark, Jan; Hermans, Cédric

doi:10.1111/hae.14291

Cited by 2 publications

(1 citation statement)

References 33 publications

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“…The CI of rFVIIa showed high efficacy and safety in the prevention of peri- and postoperative bleeding during total arthroplasty performed in people with hemophilia A and the FVIII inhibitor [ 33 , 34 ]. The rFVIIa is also very useful in nonorthopedic surgery [ 35 ].…”

Section: Management Of Bleeds Of People With Hemophilia a And Fviii I...mentioning

confidence: 99%

Managing Relevant Clinical Conditions of Hemophilia A/B Patients

Morfini,

Agnelli Giacchiello,

Baldacci

et al. 2023

Hematology Reports

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The Medical Directors of nine Italian Hemophilia Centers reviewed and discussed the key issues concerning the replacement therapy of hemophilia patients during a one-day consensus conference held in Rome one year ago. Particular attention was paid to the replacement therapy needed for surgery using continuous infusion (CI) versus bolus injection (BI) of standard and extended half-life Factor VIII (FVIII) concentrates in severe hemophilia A patients. Among the side effects, the risk of development of neutralizing antibodies (inhibitors) and thromboembolic complications was addressed. The specific needs of mild hemophilia A patients were described, as well as the usage of bypassing agents to treat patients with high-responding inhibitors. Young hemophilia A patients may take significant advantages from primary prophylaxis three times or twice weekly, even with standard half-life (SHL) rFVIII concentrates. Patients affected by severe hemophilia B probably have a less severe clinical phenotype than severe hemophilia A patients, and in about 30% of cases may undergo weekly prophylaxis with an rFIX SHL concentrate. The prevalence of missense mutations in 55% of severe hemophilia B patients allows the synthesis of a partially changed FIX molecule that can play some hemostatic role at the level of endothelial cells or the subendothelial matrix. The flow back of infused rFIX from the extravascular to the plasma compartment allows a very long half-life of about 30 h in some hemophilia B patients. Once weekly, prophylaxis can assure a superior quality of life in a large severe or moderate hemophilia B population. According to the Italian registry of surgery, hemophilia B patients undergo joint replacement by arthroplasty less frequently than hemophilia A patients. Finally, the relationships between FVIII/IX genotypes and the pharmacokinetics of clotting factor concentrates have been investigated.

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Section: Management Of Bleeds Of People With Hemophilia a And Fviii I...mentioning

confidence: 99%

Managing Relevant Clinical Conditions of Hemophilia A/B Patients

Morfini,

Agnelli Giacchiello,

Baldacci

et al. 2023

Hematology Reports

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Current status and future prospects of activated recombinant coagulation factor VIIa, NovoSeven®, in the treatment of haemophilia and rare bleeding disorders

Shima

2023

Ann Hematol

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rFVIIa, a human recombinant activated coagulation factor VII, has been used worldwide for more than two decades for the treatment of bleeding episodes and prevention of bleeding in patients undergoing surgery/invasive procedures with congenital haemophilia A or B with inhibitors (CHwI A or B), acquired haemophilia (AH), congenital factor VII deficiency and Glanzmann thrombasthenia (GT), refractory to platelet transfusion. The approved dosage, administration and indication of rFVIIa in the US, Europe and Japan differ, depending on the needs of the patient population and regulatory practices. This review presents an overview of the current status and future prospects, including that from a Japanese perspective, of using rFVIIa in the treatment of approved indications. The efficacy and safety of rFVIIa in the approved indications has been demonstrated in several randomised and observational studies and data from registries. The overall incidence of thrombosis across all approved indications in a retrospective safety assessment of clinical trials and registries, prelicensure studies and postmarketing surveillance studies of rFVIIa use was 0.17%. Specifically, the risk of thrombotic events was 0.11% for CHwI, 1.77% for AH, 0.82% for congenital factor VII deficiency and 0.19% for GT. Emerging non-factor therapies such as emicizumab have changed the treatment landscape of haemophilia A, including preventing bleeding in patients with CHwI. However, rFVIIa will continue to play a significant role in the treatment of such patients, particularly during breakthrough bleeding or surgical procedures.

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Recombinant FVIIa in elective non‐orthopaedic surgery of adults with haemophilia and inhibitors: A systematic literature review

Cited by 2 publications

References 33 publications

Managing Relevant Clinical Conditions of Hemophilia A/B Patients

Managing Relevant Clinical Conditions of Hemophilia A/B Patients

Current status and future prospects of activated recombinant coagulation factor VIIa, NovoSeven®, in the treatment of haemophilia and rare bleeding disorders

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