Congenital factor X deficiency is an extremely rare coagulation disorder that can place patients at risk for spontaneous hemorrhage or excessive bleeding in the setting of trauma or invasive procedures. Given the rarity of this disorder, there is little published guidance on how best to prevent or treat bleeding. Herein, we report a case of a 56-year-old white man with congenital factor X deficiency who was scheduled for major neurosurgery and who was treated perioperatively with 4-factor prothrombin complex concentrate (4F-PCC). Doses of 4F-PCC at 15 U per kg, administered immediately preoperatively and once at 24 hours postoperatively, allowed for successful completion of an anterior cervical discectomy and fusion without excessive bleeding. Moreover, no thromboembolic complications were observed. As such, given the wide availability of 4F-PCC, it may be considered as a first-line therapy and an alternative to fresh frozen plasma for factor X deficiencies, particularly in high-risk operative cases.
Keywords: Congenital factor X deficiency; blood banking; coagulation; neurosurgery; prothrombin complex concentrate; transfusion medicine.
Published by Oxford University Press on behalf of the American Academy of Laboratory Medicine. 2016. This work is written by US Government employees and is in the public domain in the US.