Activated prothrombin complex concentrate to treat bleeding events in acquired hemophilia A: BAHAS study

Eur J Haematol. 2022 Dec;109(6):686-695. doi: 10.1111/ejh.13853. Epub 2022 Sep 12.

Abstract

Objective: Activated prothrombin complex concentrate (aPCC) is a bypassing agent indicated to treat bleeds in patients with acquired hemophilia A (AHA). Nevertheless, its efficacy and safety in the real-world setting have not often been addressed.

Methods: We report the experience of Spanish reference centers for coagulation disorders and from acquired hemophilia Spanish Registry (AHASR) from August 2012 to February 2021. Follow-up period of 30 days after aPCC withdrawal.

Results: Thirty patients with a median age of 70 years old, suffering from 51 bleeds treated with aPCC were finally evaluated. As first-line treatment, aPCC stopped bleeding in 13 of 14 (92.9%) cases. aPCC as the second line after recombinant factor VIIa failure, stopped bleeding in all cases. In 17 patients, aPCC was used far from initial bleed control as prophylaxis of rebleeding with 94% effectiveness. No thromboembolic episodes were communicated. One patient developed hypofibrinogenemia, which did not prevent aPCC from halting bleeding. No other serious adverse events possibly or probably associated with aPCC were reported.

Conclusions: This data support aPCC as hemostatic treatment in AHA with high effectiveness and excellent safety profile in acute bleeds and as extended use to prevent rebleedings, even in aging people with high cardiovascular risk.

Keywords: acquired hemophilia; bleeding; bypass agent; coagulation disorders; inhibitors; prophylaxis.

MeSH terms

  • Aged
  • Blood Coagulation Factors / therapeutic use
  • Cost-Benefit Analysis
  • Factor IX / therapeutic use
  • Factor VIII / therapeutic use
  • Hemophilia A* / complications
  • Hemophilia A* / drug therapy
  • Hemorrhage / drug therapy
  • Hemorrhage / etiology
  • Humans
  • Recombinant Proteins / therapeutic use

Substances

  • Blood Coagulation Factors
  • Factor IX
  • Factor VIII
  • prothrombin complex concentrates
  • Recombinant Proteins

Supplementary concepts

  • Factor 8 deficiency, acquired

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