Kris Foley, MD
Resident
Mount Sinai Hospital
New York, New York, United States
Sal Girardi, MD
Attending
Mount Sinai West
New York, New York, United States
Carley Trentman, MD
Attending Physician
Mount Sinai Hospital
NYC, New York, United States
Claire E. Becker, DO
PGY-2 Resident Physician
Mount Sinai Hospital
New York, New York, United States
A 66-year-old woman with Factor VII deficiency (F7D) and knee osteoarthritis was evaluated for a total knee replacement. Her F7D was diagnosed in the 1970s during workup for menorrhagia. During prior surgeries, she received F7 replacement without subsequent bleeding. Prior to her TKR, F7 levels were 2%. Hematology recommended a dose of preoperative activated F7 30mcg/kg followed by postoperative replacement for two weeks, tapering from twice to once daily infusions. She tolerated surgery well without increased intra or postoperative bleeding. Her repeat F7 levels 280% during the treatment period. She was admitted to acute rehabilitation for intensive PT/OT and continued F7 replacement and monitoring for bleeding.
Discussions:
F7D is considered the most common of rare congenital bleeding disorders and the level of deficiency corresponds to the degree of bleeding symptoms patients will experience. Most patients will not experience bleeding if F7 levels are >10% and even those less can have mild symptoms, leading to delays in diagnosis1. Those with minor symptoms may not need regular replacement therapy, however during major surgeries with high risk of bleeding, replacement is necessary. Prior reports recommend replacement of 15-30mcg/kg during episodes of acute bleeding2. However, there are few clear guidelines for prophylactic management of bleeding during surgery. General practice by hematologists is dosing based on prior bleeding history, level of F7D, and bleeding risk of current surgery. Furthermore, even though these patients are hypocoagulable, they are still at risk of postoperative thrombosis with or without replacement therapy.
Conclusions:
Patients with a unilateral joint replacement may require acute rehabilitation if they have medical complexities such as bleeding disorders. In the perioperative and postoperative period, it is essential to manage the increased risk of hemorrhage through factor replacement under guidance of hematology recommendations.
