Acquired haemophilia A initially diagnosed as angioedema

Acquired haemophilia (AH) is a bleeding diathesis caused by inhibitors to factor VIII. Patients developing AH may present with catastrophic bleeding episodes despite having no prior history of bleeding disorder. The authors report a patient with AH who presented with tongue swelling and was diagnosed with angioedema on presentation. However, the patient started bleeding spontaneously after presentation and was diagnosed with AH and the workup revealed haematoma of the tongue, which was the cause of tongue swelling. As previously reported in the literature, AH in this patient might have been precipitated secondary to exposure to an ear infection or exposure to the penicillin group of antibiotics. Laboratory examination was confirmatory for AH, which was appropriately treated with recombinant activated factor VIIa and immunosuppressive therapy with good outcomes. Understanding the pathophysiology of AH and appropriate management is important to prevent life-threatening bleeding.