Recent advances in surgery and its perioperative treatment in people with hemophilia

ABSTRACT

Introduction: People with hemophilia (PWH) may require some surgical procedures (orthopedic surgery, cardiac surgery, neurosurgery, et cetera) throughout their lives.

Areas covered: Articles on surgery and hemophilia published during the year 2020 were analyzed. The principal inclusion factor was a focal point on surgery in hemophilia. Articles that did not concentrate on this subject were not included.

Expert opinion: Turoctocog alfa pegol appears to be efficacious for perioperative hemostatic treatment of all kind of surgical operations (minor and major) in PWH A. Long-acting recombinant coagulation factor IX (FIX) albumin fusion protein (rIX-FP) seems to facilitate perioperative management in PWH B. In people suffering from von Willebrand disease (VWD), the utilization of concentrates of von Willebrand factor (VWF) is commonly used for the decrease of exaggerated bleeding during surgical operations. On-demand management with 30–60 IU/kg may keep hemostasis under control during all kind of surgical operations. Factor concentrates with extended half-life (EHL) are a good option to standard half-life (SHL) products in PWH undergoing surgery, permitting diminished number of infusions and inferior consuming, particularly for EHL factor IX (FIX). Supplementary factor VIII (FVIII) and/or bypassing management have demonstrated to be secure and efficacious in association with emicizumab for major surgical procedures.

KEYWORDS:

• Hemophilia A
• hemophilia B
• von Willebrand disease
• surgery
• perioperative hemostasis

Article highlights

• PWH may require many types of surgery (orthopedic surgery, cardiac surgery, neurosurgery, kidney, and liver transplants, etc.) throughout their lives.

• Turoctocog alpha pegol has shown to be efficacious in accomplishing perioperative hemostasis in all kinds of surgical procedures (minor and major) in subjects with hemophilia A.

• In the majority of subjects with hemophilia B, surgical operations can be carried out utilizing a single bolus of rIX-FP prior to surgery.

• In subjects with VWD, VWF/FVIII concentrates and desmopressin are effective in precluding hemorrhage during surgical operations.

• EHL concentrates can replace standard half-life products in PWH undergoing surgery, as they allow fewer injections and lower factor consuming, especially in the EHL FIX.

Reviewer disclosures

Peer reviewers on this manuscript have no relevant financial or other relationships to disclose.

Declaration of interest

E. C. Rodriguez-Merchan has received reimbursement for collaborating as a speaker and researcher from Pfizer and Roche and has received honoraria for consulting or collaborating as a speaker from Pfizer, Roche, Sobi, Takeda, Novo Nordisk, and Bayer.

The author has no other relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript apart from those disclosed.

Additional information

Funding

This paper was not funded.