Acquired hemophilia A (AHA) is an autoimmune disease that causes involuntary and excessive bleeding. In someone with AHA, the immune system prevents blood from clotting by inhibiting the factor FVIII. AHA is rare and is present in 1.3-1.5 million people per year.
In the study, patients ages 10-79 were treated with either corticosteroids or a combination of corticosteroids with cyclophosphamide or rituximab to lower their amount of inhibitors. The patients treated with just corticosteroids had a lower amount of inhibitors than the patients treated with the combination.
To cure AHA, patients can undergo therapy to eliminate the inhibitors. Inhibitors are most prevalent in patients aged 20-40 years, though also widespread in patients aged 60-80 years.
The median time to complete remission in patients treated with just corticosteroids was shorter than those who were treated with a combination of corticosteroids with cyclophosphamide.
The combination of corticosteroids and cyclophosphamide made remission easier in patients with higher amounts of inhibitors.
37/38 patients in the study achieved complete remission.