Safety of aPCC in Real Practice – Results of the BAHAS Project

Acquired Hemophilia and Its Treatment

Acquired hemophilia A (AHA) is a rare bleeding disorder characterized by an acquired deficiency of coagulation factor VIII (FVIII). AHA results from the formation of autoantibodies targeting epitopes on the FVIII molecule. These autoantibodies are referred to as inhibitors and their formation leads to the neutralization of FVIII and/or the acceleration of its clearance from the plasma.

AHA is typically diagnosed after the sudden onset of significant, sometimes life-threatening bleeding. Bleeding is often cited as the cause of death associated with AHA in approximately 10–15% of patients. In addition to immunosuppressive treatment aimed at eradicating the inhibitor, stopping the bleeding is crucial. The most commonly used treatments in this indication are aPCC or recombinant activated factor VII (rFVIIa), with recombinant porcine FVIII (rpFVIII) being used less frequently.

The BAHAS Project

Spanish authors evaluated their experience with aPCC in patients with AHA in real clinical practice, focusing on safety in the BAHAS project (Treatment of Bleeding in Acquired Hemophilia A with Activated Prothrombin Complex Concentrate in Spain). This involved monitoring patients treated between August 2012 and February 2021. The follow-up period was 30 days after the cessation of aPCC.

Findings

Thirty patients with a median age of 70 years experienced 51 bleeding events treated with aPCC. When aPCC was used as first-line therapy, bleeding was controlled in 13 out of 14 cases (92.9%). Administering aPCC as second-line therapy following the failure of rFVIIa controlled bleeding in all cases.

In 17 patients, aPCC was administered as prophylaxis for the recurrence of bleeding long after initial bleeding control. The efficacy of such prophylaxis was 94%.

No thrombotic adverse events were recorded. Hypofibrinogenemia was observed in one patient, which did not lead to the discontinuation of aPCC and did not adversely affect the efficacy of the treatment. No other serious adverse events potentially associated with aPCC administration were reported.

Conclusion

These data support the administration of aPCC as an effective and safe hemostatic agent in the treatment of acute bleeding associated with AHA, as well as in the prevention of bleeding recurrence, even in older populations with multiple comorbidities and high cardiovascular risk.

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Source: Mingot-Castellano M. E., García-Candel F., Benítez-Hidalgo O. et al. Activated prothrombin complex concentrate to treat bleeding events in acquired hemophilia A: BAHAS study. Eur J Haematol 2022; 109 (6): 686–695, doi: 10.1111/ejh.13853.