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. 2023 Sep 6;7(7):102199.
doi: 10.1016/j.rpth.2023.102199. eCollection 2023 Oct.

Surgery in rare bleeding disorders: the prospective MARACHI study

Florence Rousseau  1   2 , Benoit Guillet  3 , Thibault Mura  4 , Alexandra Fournel  5 , Fabienne Volot  6 , Hervé Chambost  7 , Pierre Suchon  8 , Brigit Frotscher  9 , Christine Biron-Andréani  2 , Raphaël Marlu  10 , Nathalie Hezard  11   12 , Ségolène Clayssens  13 , Elodie Boissier  14 , Florence Blanc-Jouvan  15 , Pierre Chamouni  16 , Nathalie Tieulie  17 , Lucia Rugeri  18 , Annie Borel-Derlon  19 , Emmanuelle de Raucourt  20 , Isabelle Martin-Toutain  21 , Sabine Castet  22 , Aurélien Lebreton  23 , Stéphane Girault  24 , Dominique Helley-Russick  25 , Roseline D'Oiron  26 , Jean-François Schved  1   2 , Muriel Giansily-Blaizot  1   27
Affiliations

Surgery in rare bleeding disorders: the prospective MARACHI study

Florence Rousseau et al. Res Pract Thromb Haemost. .

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.

Keywords: bleeding score; hemorrhagic disorders; rare bleeding disorder; replacement therapy; surgery; tranexamic acid.

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Figures

Figure
Figure
Influence of the basal coagulation factor level, surgery bleeding risk, and bleeding score on perioperative factor replacement decision-making in patients with FVII (A) and FXI deficiency (B). BR = bleeding risk. The total number of surgical procedures is indicated at the bottom of each bar. The number of patients with a bleeding ≥4 is indicated on the side of each hatched bar. ∗∗∗∗P < .00001, ∗∗∗.00001 ≤ P < .0001, ∗∗.0001 ≤ P < .001, ∗.001 ≤ P < .05.
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