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

Abstract

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

Disclosure

MR is a consultant for and DC is an employee of Novo Nordisk Inc, the company that sponsored this study and manufactures a product approved for treatment of bleeding episodes in acquired hemophilia. The quantitative and qualitative research surveys described in this paper were completed by Harris Interactive, New York, NY, under funding provided by Novo Nordisk Inc, Princeton, NJ. A descriptive analysis of the results was prepared by Harris Interactive. The case study that forms the basis for the market research is based on an actual case treated by MR. Writing and editorial assistance was provided by Lara Primak, MD, of Ethos Health Communications in Newtown, PA, with financial support from Novo Nordisk Inc, in compliance with international guidelines for good publication practice.