Periprocedural hemostatic prophylaxis and outcomes in bleeding disorder of unknown cause

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.

Our objective was to characterize periprocedural hemostatic prophylaxis and bleeding outcomes in patients with BDUC.

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.

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.

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.