(MED-TH-05.4) Management of patients with BDUC undergoing surgery and invasive procedures

In patients with Bleeding Disorder of Unknown Cause, determining optimal hemostatic management around procedures remains a challenge, noting that many patients are referred to hematology due to a history of surgical bleeding. Prophylactic treatment strategies, including the periprocedural administration of tranexamic acid, desmopressin, and platelet transfusion, have been evaluated in retrospective cohort studies and case series, although estimates of procedural bleeding risk vary between studies. In accordance with guidance from the ISTH SSC, a stepwise approach towards procedural prophylaxis is recommended, incorporating both patient and procedure-specific factors. While observation alone may be appropriate for select low-risk procedures, tranexamic acid should be considered a cornerstone of treatment given its favorable safety profile, wide availability, and efficacy in a variety of different bleeding disorders. Usage of desmopressin and platelet transfusion varies significantly between centers, and risks (hyponatremia from desmopressin, alloimmunization from platelet transfusion) should be considered. Patients should be screened and treated for iron deficiency anemia prior to major procedures. Bleeding events may be more common after certain gynecologic procedures, and optimal management of bleeding with childbirth remains an area of unmet need. At present, there are no functional or global assays of hemostasis that can be used to guide perioperative therapy, although this is an active area of investigation. Procedural day is a potential harm of BDUC diagnosis, and close collaboration with surgical and anesthesia colleagues is necessary to ensure patients with BDUC receive necessary care.