Replacement therapy for bleeding episodes in factor VII deficiency: A prospective evaluation

Mariasanta Napolitano, Jorgen Ingerslev, Benny Sørensen, Mehran Karimi, Anne-Marie Bertrand, Anne-Marie Bertrand, Guglielmo Mariani, Giovanni Di Minno, Jens Bjerre, Arlette Ruiz-Saez, Alberto Dolce, Angelika Batorova, Günter Auerswald, Rosario Perez Garrido, Giovanni Di Minno, Jean F. Schved, Guglielmo Mariani, Helen Platokouki, Alberto Dolce, Guglielmo Mariani

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45 Citazioni (Scopus)


Patients with inherited factor VII (FVII) deficiency display different clinical phenotypes requiring ad hoc management. This study evaluated treatments for spontaneous and traumatic bleeding using data from the Seven Treatment Evaluation Registry (STER). One-hundred one bleeds were analysed in 75 patients (41 females; FVII coagulant activity <1-20%). Bleeds were grouped as haemarthroses (n=30), muscle/subcutaneous haematomas (n=16), epistaxis (n=12), gum bleeding (n=13), menorrhagia (n=16), central nervous system (CNS; n=9), gastrointestinal (GI; n=2) and other (n=3). Of 93 evaluable episodes, 76 were treated with recombinant, activated FVII (rFVIIa), eight with fresh frozen plasma (FFP), seven with plasma-derived FVII (pdFVII) and two with prothrombin-complex concentrates. One-day replacement therapy resulted in very favourable outcomes in haemarthroses, and was successful in muscle/subcutaneous haematomas, epistaxis and gum bleeding. For menorrhagia, single- or multiple-dose schedules led to favourable outcomes. No thrombosis occurred; two inhibitors were detected in two repeatedly treated patients (one postrFVIIa, one post-pdFVII). In FVII deficiency, most bleeds were successfully treated with single 'intermediate' doses (median 60 μg/kg) of rFVIIa. For the most severe bleeds (CNS, GI) short- or long-term prophylaxis may be optimal. © Schattauer 2013.
Lingua originaleEnglish
pagine (da-a)238-247
Numero di pagine9
RivistaThrombosis and Haemostasis
Stato di pubblicazionePublished - 2013

All Science Journal Classification (ASJC) codes

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