Perioperative Management of Hemophilia a Using Recombinant Factor VIII in Patients Undergoing Major or Minor Surgery

[Introduction]

It has been known that Hemophilia needs careful management from the bleeding tendency in the perioperative period. In this study, we performed endoscopic nasal pituitary adenomatectomy for growth hormone-producing pituitary adenoma in patients with hemophilia A. There were no reports using efraloctocog alpha (ELOCTATE®, Bioverativ, Cambridge, MA, USA, rFVIIIFc) for perioperative management in major surgery, intracranial surgery, for a hemophilia A patient.

[Case]

A 28-year-old man admitted to our Hospital because of endoscopic nasal pituitary adenomasectomy for growth hormone-producing pituitary adenoma. rFVIIIFc was used as a management of hemophilia A at the perioperative period. And we created a regimen for administration of rFVIIIFc regarding the guidelines for hemostasis treatment for hemophilia patients without inhibitors (revised 2013) published by the Japan Thrombohemorrhagic Society. In our hospital, the results of factor VIII activity can not be obtained as an emergent examination, so we used APTT as an indicator.

[Result]

The surgery was terminated with 150 ml of intraoperative bleeding volume that was almost the same amount as expected bleeding volume. Despite a risk of nasal bleeding after surgery was expected, only a small amount of nasal bleeding was occurred twice. The course of factor VIII activity was also good with APTT.

[Conclusion]

We concluded that rFVIIIFc may be available reagent in perioperative management of intracranial surgery with hemophilia A without inhibitors. The advantage of perioperative management by the bolus administration method (=BI method, This involves repeated administration of a bolus injections.) using half-life extended drugs is that these drugs need to be administered intravenous injection only once a day, and such a treatment protocol is easy to perform at a hospital. Furthermore, the BI method is also economical as it reduces the amount and thus the cost of the drug, as compared with the continuous administration method (=CI method, A syringe pump continuously administers coagulation factors after an initial bolus administration.) using the existing coagulation factor preparations. For perioperative management using extended half-life drugs, we consider that further case studies are necessary to prepare dosing regimens. However, such drugs have the potential to impact not only periodic replacement therapy, but also perioperative management in hemophilia patients. As mentioned above, we feel that the extended half-life drugs have the potential to significantly impact hemophilia treatment.