Barriers to effective diagnosis and management of a bleeding patient with undiagnosed bleeding disorder across multiple specialties: results of a quantitative case-based survey

Mark T Reding,¹ David L Cooper<sup>2

1</sup>Center for Bleeding and Clotting Disorders, University of Minnesota Medical Center, Minneapolis, MN, ²Medical Affairs, Novo Nordisk Inc, Princeton, NJ, USA

Background: Bleeding symptoms commonly seen by multiple physician specialties may belie undiagnosed congenital or acquired bleeding disorders. Acquired hemophilia is a potentially life-threatening cause of unexplained acute bleeding manifested by an abnormal activated partial thromboplastin time (aPTT) that does not correct with 1:1 mixing with normal plasma.
Methods: Practicing physicians (hematology/oncology, emergency medicine, geriatrics, internal medicine, rheumatology, obstetrics and gynecology, critical care medicine, and general surgery) completed an online survey based on a hypothetical case scenario.
Results: Excluding surgeons and obstetrician/gynecologist respondents, 302 physicians (about 50 per specialty) were presented with an older adult woman complaining of recurrent epistaxis. Nearly 90% ordered a complete blood count and coagulation studies (aPTT, prothrombin time [PT]/international normalized ratio [INR]). Despite a prolonged aPTT of 42 seconds, <50% of nonhematologists would repeat the aPTT, and <45% would consult a hematologist; emergency medicine physicians were least likely (10%) and rheumatologists were most likely (43%) to consult. After presentation weeks later with bruising and abdominal/back pain, ≥90% of physicians within each specialty ordered a complete blood count or PT/INR/aPTT. Despite an aPTT of 63 seconds, the majority did not repeat the aPTT. At this point, approximately 75% of internal medicine and geriatric physicians indicated they would consult a hematologist, versus 47% in emergency medicine and 50% in critical care. All participants preferred abdominal computed tomography (80%–84%). After 12 hours of additional observation, 73% to 94% of respondents consulted a hematologist. Complete blood count revealed anemia and an aPTT twice the upper limit of normal; emergency medicine physicians remained least likely to request a consult.
Conclusion: Determining the cause of an abnormal coagulation study result should carry equal weight as looking for the site of bleeding and could be facilitated by consultation with a hematologist. Insight from this survey highlights knowledge and practice gaps that could be the target of focused educational initiatives.

Keywords: acquired hemophilia, hemorrhage, aPTT, partial thromboplastin time, coagulation disorders