Major cardiac surgery with recombinant FIX Fc fusion protein replacement in hemophilia B: a case report.
| Autor: | Bohn JP; Department of Internal Medicine V, Hematology and Oncology, Medical University of Innsbruck, Anichstrasse 35, A-6020 Innsbruck, Austria., Fiala A; Department of Anesthesiology and Critical Care Medicine, Medical University of Innsbruck, Innsbruck, Austria., Bachmann S; Central Institute of Clinical and Chemical Laboratory Diagnostics, University Hospital of Innsbruck, Innsbruck, Austria., Irsara C; Central Institute of Clinical and Chemical Laboratory Diagnostics, University Hospital of Innsbruck, Innsbruck, Austria., Wolf D; Department of Internal Medicine V, Hematology and Oncology, Medical University of Innsbruck, Innsbruck, Austria., Feistritzer C; Department of Internal Medicine V, Hematology and Oncology, Medical University of Innsbruck, Innsbruck, Austria. |
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| Jazyk: | angličtina |
| Zdroj: | Therapeutic advances in hematology [Ther Adv Hematol] 2022 Jul 26; Vol. 13, pp. 20406207221104595. Date of Electronic Publication: 2022 Jul 26 (Print Publication: 2022). |
| DOI: | 10.1177/20406207221104595 |
| Abstrakt: | The introduction of extended factor IX (FIX) products has significantly facilitated the treatment of hemophilia B patients. However, optimal perioperative management remains a topic of hot debate, particularly in surgeries with high bleeding risk. For the first time, we report here a patient with mild hemophilia B and degenerative aneurysms of aortic root and ascending aorta undergoing elective Bentall's operation with full cardiopulmonary bypass, who was successfully managed with eftrenonacog alfa (Alprolix ® ), a recombinant FIX Fc fusion protein (rFIXFc). rFIXFc could safely be monitored using the Pathromtin SL aPTT-reagent. No significant bleeding was noted intraoperatively despite systemic heparinization as well as postoperatively. Higher doses of rFIXFc were inevitable to reach target FIX levels intraoperatively, whereas in the post-surgery setting stable FIX concentrations were maintained with only few rFIXFc injections facilitating fast wound healing and remobilization of the patient. Competing Interests: Competing interests: The authors disclosed receipt of the following financial support for the research, authorship, and/or publication of this article: CF reports research grant, reimbursement for attending symposium, and consulting fees from Sobi. The remaining authors declare that they have no conflict of interest. (© The Author(s), 2022.) |
| Databáze: | MEDLINE |
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