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Article Dans Une Revue Research and Practice in Thrombosis and Haemostasis Année : 2023

Surgery in rare bleeding disorders: the prospective MARACHI study

Thibault Mura
Pierre Chamouni
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Annie Borel-Derlon
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Sabine Castet
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Stéphane Girault
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Muriel Giansily-Blaizot
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Résumé

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. Previous article in issue
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Dates et versions

hal-04313517 , version 1 (28-03-2024)

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Florence Rousseau, Benoit Guillet, Thibault Mura, Alexandra Fournel, Fabienne Volot, et al.. Surgery in rare bleeding disorders: the prospective MARACHI study. Research and Practice in Thrombosis and Haemostasis, 2023, 7 (7), pp.102199. ⟨10.1016/j.rpth.2023.102199⟩. ⟨hal-04313517⟩
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