Acquired haemophilia is a rare autoimmune disease that, through the production of autoantibodies against Factor VIII, triggers a predisposition for severe bleeding. Early recognition is critical, as early therapy directed toward achieving haemostasis and eradicating inhibitors can be lifesaving.
Several studies on acquired haemophilia have not addressed the issue of diagnostic delay, and the only data in this regard have been published in the European Acquired Haemophilia Registry (EACH2). The authors found that most patients reached a diagnosis in the first week after the onset of bleeding, with a median of 3 days, and that diagnostic delay had a significant impact on the interval between onset of onset and the initiation of haemostatic therapy.
One cause of diagnostic delay in patients is the use of anticoagulant treatment, which is easily blamed for the initial bleeding.
Other aspects of diagnostic delay are related to coagulation testing. In fact, 35% of patients do not have a baseline coagulation test when they come to medical centres on their first visits for bleeding.
Delayed initiation of haemostatic treatment in the acute bleeding phase of acquired haemophilia is associated with longer haemorrhagic time periods and greater requirements over the course of the disease. In fact, patients with more than one month of diagnostic delay require significantly more days of haemostatic therapy.
Reference:
- J. Pardos-Gea et al. Diagnostic delay in acquired haemophilia: Analysis of causes and consequences in a 20-year Spanish cohort, Haemophilia. 2018;1–3. DOI: 10.1111/hae.13499
