51. Surgery in rare bleeding disorders: the prospective MARACHI study.
- Author
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Rousseau F, Guillet B, Mura T, Fournel A, Volot F, Chambost H, Suchon P, Frotscher B, Biron-Andréani C, Marlu R, Hezard N, Clayssens S, Boissier E, Blanc-Jouvan F, Chamouni P, Tieulie N, Rugeri L, Borel-Derlon A, de Raucourt E, Martin-Toutain I, Castet S, Lebreton A, Girault S, Helley-Russick D, D'Oiron R, Schved JF, and Giansily-Blaizot M
- Abstract
Background: Despite the wide use of bleeding scores and the reliability of clotting factor level measurement, bleeding risk stratification before surgery remains challenging in patients with rare inherited bleeding disorders., Objectives: This multicenter observational prospective study assessed in patients with rare coagulation factor deficiency, the perioperative hemostatic management choices by hemostasis experts and the bleeding outcomes after surgery., Methods: One hundred seventy-eight patients with low coagulation activity level (factor [F] II, FV, combined FV-FVIII, FVII, FX, or FXI <50%) underwent 207 surgical procedures. The bleeding outcome, Tosetto's bleeding score, and perioperative hemostatic protocols were collected., Results: Among the 81 procedures performed in patients with severe factor deficiency (level ≤10%), 27 were done without factor replacement (including 6 in patients at high bleeding risk), without any bleeding event. Factor replacement therapy was used mainly for orthopedic procedures. In patients with mild deficiency, 100/126 surgical procedures were carried out without perioperative hemostatic treatment. In patients with FVII or FXI deficiency, factor replacement therapy was in function of the procedure, bleeding risk, and to a lesser extent previous bleeding history. Tranexamic acid was used in almost half of the procedures, particularly in case of surgery in tissues with high fibrinolytic activity (76.8%)., Conclusions: The current perioperative hemostatic management of patients with rare bleeding disorders appears to be adapted. Among the 207 procedures, only 6 were associated with excessive bleeding. Our findings suggest that rather than the bleeding score, factor level and surgery type are the most relevant criteria for perioperative factor replacement therapy., (© 2023 The Authors.)
- Published
- 2023
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