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J Thromb Thrombolysis. 2019 Jan;47(1):129-133. doi: 10.1007/s11239-018-1750-y.

Combined use of antifibrinolytics and activated prothrombin complex concentrate (aPCC) is not related to thromboembolic events in patients with acquired haemophilia A: data from FAIR Registry.

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Haemophilia Centre, University Hospital of Padua, Via Giustiniani, 35128, Padua, Italy.
Haemophilia Centre, S.Matteo Hospital of Pavia, Pavia, Italy.
Haemophilia and Thrombosis Centre, S.Giovanni Bosco Hospital of Neaples, Naples, Italy.
Cellular Biotecnology and Haematology Department, Umberto I University Hospital of Rome, Rome, Italy.
Centre of Coagulation Diseases, Hospital of Macerata, Macerata, Italy.
Haemophilia Centre, University Hospital of Padua, Via Giustiniani, 35128, Padua, Italy.


Antifibrinolytics combined with aPCC are not routinely administered to patients with acquired hemophilia A due to increased thrombotic risk. This association normalizes clot stability, and improves the efficacy of therapy, but can increase the risk of severe side effects. Due to these premises it has always raised doubts and perplexities in the clinics. We now report the data of the "FEIBA® on acquired haemophilia A Italian Registry (FAIR Registry)", a retrospective-prospective study that included 56 patients. This is the first study that assessed the clinical response of the combination of aPCC and antifibrinolytic agents in patients with acquired haemophilia A. A total of 101 acute bleeds were treated with aPCC. Antifibrinolytic agents were used in the treatment of 39.6% of total bleeds, based on both, a clinical assessment and an evaluation of bleeding. Twenty-five of the 30 patients (57.1%) treated with antifibrinolytic drugs showed serious co-morbidity. Among them, 40% presented severe cardiovascular diseases. All bleeds treated with combined therapy had a shorter duration of treatment (mean reduction 16.3%). All the treated patients presented a good tolerability and no arterial or venous thromboembolic events were reported. In our retrospective registry the combination of antifibrinolytics and aPCC appears safe and effective in the treatment of patients with AHA, especially in the case of severe and life-threatening bleeding, but this hypothesis needs to be confirmed in adequate, larger clinical trials.


Acquired haemophilia A; Activated prothrombin complex concentrate; Antifibrinolytics; Thromboembolic risk

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