PB0707 - An Integrated Clinical and Pathological Approach to Rare Bleeding Disorders

Background: Prompt recognition of rare acquired bleeding disorders is imperative, especially amongst older patients who often present with life-threatening bleeds.

Aims: To highlight the diagnostic challenges and working relationship between clinicians and biomedical scientists in making a timely diagnosis.

Methods: We present three cases of rare acquired coagulation disorders.

Results: Patient 1 is a 77 year old man presenting with extensive bruising over the chest and arm and a haemoglobin of 60g/L. Normal Clotting screen (PT, APTT and Fibrinogen) prompted the lab staff to discuss immediately with the clinician and test factor XIII levels, diagnosing acquired factor XIII deficiency.
Patient 2 is a 99 year old man on long term warfarin for arrhythmia, presenting to another hospital several times with bruises over the arms, especially at venepuncture sites. INR checked and given vitamin K. Eventually, when tested in our laboratory both the PT and APTT were performed for this warfarinised patient. The APTT was disproportionately prolonged relative to the PT enabling the diagnosis of acquired haemophilia A.
Patient 3 is an 84 year old lady who visited casualty of another hospital several times with GI bleeds necessitating blood transfusions and upper/lower GI endoscopies. Referred to our hospital for double balloon pushed enteroscopy where a clotting screen showed a prolonged APTT with no 50/50 correction. Von Willebrand disease (VWD) screening was conducted in addition to factor VIII levels, confirming acquired VWD which is strongly associated with angiodysplasia.

Conclusion(s): The unique working relationship between clinical and laboratory staff at our UK tertiary haemostasis centre is exemplified by the following: Weekly laboratory results and MDT meetings; co-location of the haemostasis laboratory and clinical area including a shared common room; an inclusive working environment where all staff feel empowered to contribute positively to patient care. We have highlighted how this effective partnership can streamline diagnostics and enhance patient care.