Replacement therapy for bleeding episodes in factor VII deficiency. A prospective evaluation
- Mariani, G
- Napolitano, M
- Dolce, A
- Pérez Garrido, R
- Batorova, A
- Karimi, M
- Platokouki, H
- Auerswald, G
- Bertrand, AM
- Schved, JF
- Bjerre, J
- Ingerslev, J
- Sørensen, B
- Ruiz Saez, A.
- DI MINNO, GIOVANNI
DOIAbstract
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...
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