Can the plasmaderived factor VIII still play a role in the treatment of acquired hemophilia A at the time of new drugs?

Abstract

: Bypassing agents are the first-line therapy in the treatment of acquired hemophilia A (AHA), but not the only one. Other options as recombinant porcine factor VIII or plasmaderived concentrates (pdFVIII) are available to clinicians. Aim of this study was to evaluate whether the pdFVIII can still play a role in the treatment of AHA, and which patients could benefit from this therapy. All patients with AHA, presenting severe cardiovascular comorbidities, and treated with pdFVIII with or without von Willebrand factor (vWF), referred to two different hospitals, were initially considered. Eight patients were studied and divided into two groups: first, patients treated with daily infusion of pdFVIII; second, patients treated with pdFVIII continuous infusion. After 6 months of follow-up, all patients reached complete response. Mean consumption of clotting factor (219 000 vs. 142 000 IU), mean duration of therapy (61.5 vs. 10.5 days), and mean time necessary to disappearance of the inhibitors (INHs) (64 vs. 9 days) were higher in group 1, and the differences between the two groups were statistically significant (P < 0.05). Patients in group 1 also had a mean INH titer of 20.4 BU, higher than that of group 2 patients (8.4 BU), with a lower detectable FVIII level. Our study showed that pdFVIII can be an effective option for patients at high thromboembolic risk, even for those with high-titer INHs, especially if combined with vWF. The immunomodulatory role of vWF should, however, be better investigated in wider trials. The days of treatment with pdFVIII continuous infusion was proven to be similar to those reported with other drugs.