Periprocedural hemostatic prophylaxis and outcomes in bleeding disorder of unknown cause.

Background: Bleeding disorder of unknown cause (BDUC) is a diagnostic category encompassing patients with a clear bleeding phenotype but without identifiable abnormality on hemostatic testing. The optimal management of hemostasis in BDUC patients prior to invasive procedures and childbirth is uncertain. Objectives: Our objective was to characterize periprocedural hemostatic prophylaxis and bleeding outcomes in patients with BDUC. Methods: We conducted a retrospective cohort study of adult patients with BDUC at 2 academic medical centers. Following diagnosis of BDUC, subsequent surgical procedures and childbirths were analyzed using a combination of registry data and manual chart review. Results: We identified 127 patients with mean age of 39.9 years (SD = 16.6); the majority of patients were female (91.3%). Forty-eight major procedures, 70 minor procedures, and 19 childbirths were analyzed. Antifibrinolytic monotherapy was advised for 57% of major procedures, 59% of minor procedures, and 67% of childbirths. Perioperative platelet transfusion was recommended in 26% of major procedures and 9% of minor procedures in combination with other hemostatic agents. Major or clinically relevant nonmajor bleeding occurred in 4.1% (4/98) of procedures with prophylaxis and 10% (2/20) of procedures without prophylaxis. Postpartum hemorrhage occurred in 26% (5/19) of deliveries. Conclusion: In this multiinstitution experience, we found overall low rates of hemostatic complications in procedures completed with hemostatic prophylaxis, although preventing hemorrhage in childbirth and gynecologic procedures remain unmet needs.