Abstract
Background
Acquired haemophilia A (AHA) is a rare autoimmune bleeding disorder characterized by the formation of inhibitory antibodies against endogenous Factor VIII (FVIII), interfering with its function. Though a rare disorder, with around 1.5 cases per million, it is more prevalent among the elderly. There is a wide phenotype of bleed presentation, and nearly half of patients will have an underlying malignancy or autoimmune disorder. Mortality is estimated to be more than 20% in patients aged over 65 years.
Case
We report the case of an 82-year-old woman who presented with a sudden onset of spontaneous ecchymosis, and haematoma over the right scapular region, extending to the right breast, flank, and abdomen. She was planned for exploration in view of impending compartment syndrome when a diagnosis of AHA was confirmed. Investigations revealed isolated prolonged activated partial thromboplastin time (aPTT) and FVIII activity estimated at 2.8%. During the first week of treatment, she received haemostatic management with bypassing agent NovoSeven (recombinant Factor VIIa (rFVIIa)), starting at a dose of 90 mcg/kg, every 4 hours, along with tranexamic acid. Combination immunosuppressive therapy (IST) was initiated with methylprednisolone @1mg/kg and cyclophosphamide (50–100mg/day). Bypassing agents were continued for 2 days; the patient had a transient response, and no expansion of haematoma was observed. As the serial inhibitor titre was persistently > 20 BU, the patient received 1 dose of rituximab @ 375mg/m2 in combination with corticosteroids. During the second week of treatment, the patient developed respiratory distress and was managed in intensive care for possible complications of IST. She later developed pneumonia and died due to its complications. The patient’s age at presentation, the presence of a high inhibitor titre, and dual IST were possible risks and prognostic factors in this case.
Conclusion
Early diagnosis and careful, effective management of AHA is essential for achieving remission. Its prevalence among the elderly population increases the likelihood of comorbidities and raises the need for a multi-departmental approach. Appropriate use of bypassing agents for haemostatic control is key, alongside careful stratification and consideration of the risks of IST. IST-related mortality and complications mandate an individualized approach to treatment.
