Critical role of laboratory and multidisciplinary strategies in the early diagnosis of acquired haemophilia a for life-saving management

Background

Acquired haemophilia A (AHA) is a rare but potentially life-threatening autoimmune disorder characterized by the development of autoantibodies against coagulation factor VIII (FVIII). It manifests with spontaneous, severe bleeding in patients without a personal or family history of bleeding disorders. Early recognition and rapid treatment are crucial to reduce morbidity and mortality.

Case presentation

Here we describe two cases of AHA in elderly female patients with no prior history of coagulopathy. The first case involved an 81-year-old woman presenting with extensive spontaneous haematomas and severe anaemia. Laboratory findings revealed an isolated prolonged activated partial thromboplastin time (aPTT), undetectable FVIII activity, and the presence of a low-titre FVIII inhibitor (2.24 BU/mL). Treatment with recombinant activated factor VII (rFVIIa) and corticosteroids led to clinical improvement and inhibitor reduction. The second case concerned an 85-year-old woman who developed severe haemorrhagic manifestations following ureteric stenting. Coagulation studies showed markedly prolonged aPTT and a high-titre FVIII inhibitor (165 BU/mL). Despite initiation of immunosuppressive therapy the patient experienced fatal complications due to uncontrolled bleeding and multi-organ failure.

Conclusions

These cases underscore the importance of considering a possible AHA in patients with isolated aPTT prolongation and unexplained bleeding. Punctual laboratory diagnosis, including mixing studies and FVIII inhibitor assays, is essential for early life-saving interventions. Multi-disciplinary management and rapid initiation of haemostatic and immunosuppressive therapies are key to improving outcomes in this challenging condition.