Surgery in rare bleeding disorders: the prospective MARACHI study

Area of Science:

• Hematology
• Surgical Management
• Rare Diseases

Background:

• Bleeding risk stratification before surgery is challenging for patients with rare inherited bleeding disorders.
• Existing methods like bleeding scores and clotting factor levels have limitations.

Purpose of the Study:

• To assess perioperative hemostatic management choices by experts in patients with rare coagulation factor deficiency.
• To evaluate bleeding outcomes after surgery in this patient population.

Main Methods:

• A multicenter observational prospective study included 178 patients with low coagulation activity (factor levels <50%).
• 207 surgical procedures were analyzed, collecting data on bleeding outcomes, Tosetto's bleeding score, and perioperative hemostatic protocols.

Main Results:

• 27/81 procedures in severe deficiency (≤10% factor level) were performed without factor replacement, including 6 high-risk cases, with no bleeding events.
• 100/126 procedures in mild deficiency were done without hemostatic treatment.
• Factor replacement was guided by procedure, bleeding risk, and history, with tranexamic acid used in nearly half of procedures.

Conclusions:

• Current perioperative hemostatic management for rare bleeding disorders is effective, with only 6/207 procedures experiencing excessive bleeding.
• Factor level and surgery type are more critical than bleeding scores for guiding perioperative factor replacement therapy.