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CME Evaluation

Oral Communication Session

Inherited Bleeding Disorders and Inhibitors

Session: Surgery in hemophilia and rare bleeding disorders

OC 14.5 - "Surgery in patients with rare bleeding disorders"

Sunday, June 23, 2024
10:30 – 10:45 ICT
Room: Ballroom B4
Background: The hemostatic management of patients with rare bleeding disorder (RBD) during surgery procedures or child delivery can be challenging.

Aims: The aim of this study is to evaluate the efficacy and safety of hemostatic management in patients undergoing surgeries or deliveries in patients with RBDs.

Methods: This is an observational, descriptive, longitudinal, and retrospective cohort study of patients presenting to the Reference Center for Congenital Coagulopathies of Aragon (Spain) from 2010 to 2023. One hundred and two patients were included, 39 men (38%) with a median age of 38 years.

Results: Eighty-one patients (79.4%) underwent any surgical procedures; of them 36 (44.4%) were major surgeries and 45 (55.6%) were minor (Figure 1); and 21 women (20.6%) had full-term pregnancies. Among the 81 patients underwent surgeries, 47 (58.1%) had a deficiency of FVII, hemostatic treatment included antifibrinolytics in 28 patients (59.6%), rFVIIa along with antifibrinolytics in 11 (23.4%), and 8 (17%) received no hemostatic treatment. Regarding complications related with surgery we reported one hemorrhagic complication after adenoidectomy resolved with local hemostasis. Surgeries were performed on 17 patients (20.9%) with FXI deficiency, 8 (9.8%) with FXII deficiency, 6 (7.4%) with FX deficiency, 2 (2.4%) with FV deficiency, and 1 (1.2%) with FXIII deficiency. In the group of pregnant women, 12 women (57.1%) had an FVII deficiency, with 2 hemorrhagic complications—one patient without prior hemostatic treatment and another treated with rFVIIa along with antifibrinolytic. Table 1 shows hemostatic treatment peri-surgical and peri-partum of the patients.

Conclusion(s): In our experience, hemostatic personalized treatment in patients with RBD undergoing surgery or childbirth has been effective and safe. Most patients had mild forms of RBD successfully managed with tranexamic acid alone or in combination with other specific hemostatic treatments. Prospective studies providing higher evidence for the care of patients with RBD undergoing surgical procedures or deliveries are warranted.