Major cardiac surgery with recombinant FIX Fc fusion protein replacement in hemophilia B: a case report
| Autor: | Jan-Paul Bohn, Anna Fiala, Sebastian Bachmann, Christian Irsara, Dominik Wolf, Clemens Feistritzer |
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| Jazyk: | angličtina |
| Rok vydání: | 2022 |
| Předmět: | |
| Zdroj: | Therapeutic Advances in Hematology, Vol 13 (2022) |
| Druh dokumentu: | article |
| ISSN: | 2040-6215 20406207 |
| DOI: | 10.1177/20406207221104595 |
| Popis: | 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. |
| Databáze: | Directory of Open Access Journals |
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