NUR 03.5 - A lack of history makes it a mystery: Insights into Non-Severe Hemophilia

Background: Mild bleeding disorders remain undiagnosed or are diagnosed late following a clinically significant bleeding event provoked by trauma or surgery. Post-operative bleeding in an undiagnosed bleeding disorder is a complicated clinical muddle that is better avoided by appropriate pre-surgical screening. We present a series of non-severe hemophiliacs diagnosed following a catastrophic post-surgical bleed. We highlight the importance of subtle clinical clues and propose a pre-surgical checklist to avoid such accidents.

Aims: We highlight the importance of utilization of simple clinical tools that aid in suspecting this subset of hemophilia and thereby reduce the burden of hemorrhagic accidents, health care costs and improve quality of life.

Methods: All the cases were referred to the haematology department for further evaluation, and the data was collected retrospectively, Consent was taken for data gathering. A detailed history was collected using a demographic proforma and an ISTH bleeding assessment tool. Further clinical and laboratory assessments were done.

Results: A total of four cases were reported here, all are adults with histories of postsurgical bleeding. Post-surgical evaluation showed the hemostatic test (APTT) was at the borderline elevated in most of the cases, and post-surgical assessment of bleeding using the ISTH BAT revealed at least two bleeding symptoms reported by the cases (Table 1). Prior to the discovery, they had received a massive amount of blood transfusion for the low hemoglobin and blood loss post-operatively. All the cases have undergone surgical re-exploration or re-suturing of the surgical wound. longer hospital stays and higher healthcare costs doubled.

Conclusion(s): MBD are hence under diagnosed unless a patient develops clinically relevant bleeding manifestation following a surgical procedure or trauma. Delay is diagnosis can be fatal or result in serious health and economic morbidity.