PB1839 SUCCESSFUL PERIOPERATIVE HEMOSTATIC MANAGEMENT FOR SURGERY IN PATIENTS WITH CONGENITAL FACTOR IX DEFICIENCY: A SINGLE CENTER EXPERIENCE

Background: Hemophilia B is an X‐linked recessive bleeding disorder resulting from factor IX (FIX) deficiency. Prevalence of hemophilia B is one in 20,000 live male births, a quarter that of hemophilia A. While coagulation factor replacement is essential in surgical intervention in hemophilia B patients, evidence in support of the perioperative management is more lacking than hemophilia A. Aims: This study provides an updated evaluation of the safety and effectiveness of coagulation FIX concentrates in treating perioperative bleeding. Methods: We reviewed the records of hemophilia B patients without inhibitors who underwent surgery between June 2006 and May 2017 using coagulation FIX concentrates at a single center. The first bolus of [80 IU/kg X 1.2‐1.4] was given 30 min before surgery to achieve a preoperative FIX level of 80 % under the supervision of a multidisciplinary hemophilia team of our institute. Approximately 50% of the initial dose was administered every 12 h and the dosing intervals were increased to 24 h. Results: In total, 109 surgeries (93 orthopedic, 16 other) were conducted in 70 hemophilia B patients. The median age of the patients was 35.5 years (range, 6–78). The median duration of hospitalization was 14.3 days (range, 2–47), and median duration from operation to discharge was 11.5 days (1‐42). Recombinant FIX concentrates were administered in all cases except 2 patients. The major surgical procedures, that were defined as one that required hemostatic support for periods exceeding 5 consecutive days, were 94 cases (89.5%). Median difference between preoperative and postoperative hemoglobin was 2.35 g/dL (range, 0‐7.7). Total 18 cases of patients required packed RBC transfusion after surgery. In most cases, bleeding stopped or was well controlled; however, one patient expired after emergent surgery for intracranial hemorrhage. No thromboembolic event and development of antibody to FIX were observed in any case. Summary/Conclusion: Good control of hemostasis can be achieved using FIX concentrates in hemophilia B patients who are undergoing surgery. Thorough planning is needed before elective surgery and more active and aggressive management may be needed for emergency surgery.