PB0576 - Perioperative Managements of Rare Factor Deficiencies, Single Center Experience

Background: Rare factor deficiencies(RFD) are defined as a decreased level or function of a coagulation factor expect FVIII and FIX. Clinical findings may vary from levels of factor, comorbidities, concomitant drugs, hormonal cycle alterations.

Aims: The objective of this study was to assess surgical interventions and management in cases of rare factor deficiencies.

Methods: A retrospective evaluation was conducted on patients who underwent surgical procedures at the Ege Adult Hemophilia and Thrombosis Center between Jan 2021 and Dec 2022. Descriptive analyses were used to evaluate the patient's age, gender, age of diagnosis, bleeding score(ISTH-BAT) and type of operation procedure (major/minor) as well as pre- postoperative treatments.

Results: Our study included 18 patients who underwent surgical procedures. Of the cases analysed, 27.8%(5) were male and 72.2%(13) were female. The mean ISTH-BAT score was 6.39(ranging from 0 to 16), and the median age was 34.5 (25-60). The majority of cases(61.1%) were associated with FVII deficiency, while 16.7% were linked to FXI deficiency, and 11.1% each were associated with afibrinogenemia and FX deficiency. Major operations were performed in 55.6% of cases, while minor operations were performed in 44.4% of cases. RFD-specific factor replacement was used in 50%(9) of cases, and fresh frozen plasma(FFP) was used for preoperative bleeding control in 38.9% of cases. Postoperative factor replacement was not administered to 55.6% of cases that received preoperative factor replacement. Minor surgery was performed in all of these cases. In 71.4% of cases where preoperative FFP was administered, postoperative FFP replacement was not applied.

Conclusion(s): During minor surgery, surgical hemostasis and factor replacements such as tranexamic acid may be sufficient. However, for major surgery, the lowest dose required for surgical hemostasis should be used, and postoperative coagulation parameters should be monitored to prevent postoperative bleeding. Individualized treatment approach keeps the touchstone as the primary focus.