title: Recombinant activated factor VII for hemostatic cover of orthopedic interventions in a girl with thrombocytopenia with absent radii syndrome.
abstract: Over the past 10 years recombinant activated factor VIIa (rFVIIa) has been successfully used for treatment and prophylaxis of bleeding in patients with platelet defects, including thrombocytopenia and congenital and acquired platelet function abnormalities. Most reported data concern patients with Glanzmann's thrombasthenia and the information available is still limited, especially for surgery. We report on a 15-year-old girl with thrombocytopenia ( approximately 60,000/microl) and platelet dysfunction (bleeding time 30 min, absent platelet aggregation and ATP secretion in response to collagen), related to thrombocytopenia with absent radii syndrome, undergoing two surgical interventions on the upper limbs due to forearm deformities, with prolonged postoperative revisions. In both surgeries rFVIIa was successfully employed as a bolus administration (80 microg/kg every 4 h during the first day, then every 6 h over the following 5 and 3 days, respectively; tranexamic acid was associated from the second day, administered for 2 weeks), avoiding the need for blood products. This report highlights rFVIIa as an attractive, alternative approach to secure hemostasis in patients with platelet defects; on the other hand, the heterogeneity of reported rFVIIa treatment regimens and, in particular, the lack of definite and easily available parameters (or assays) for monitoring rFVIIa efficacy and safety are the main open issues in this setting.
authors.affiliation: Regional Reference Centre for Coagulation Disorders, Department of Clinical and Experimental Medicine, 'Federico II' University of Naples, Naples, Italy. antocopp@unina.it
authors.forename: Antonio
authors.initials: A
authors.lastname: Coppola
authors.forename: Ciro De
authors.initials: CD
authors.lastname: Simone
authors.forename: Nicola Macarone
authors.initials: NM
authors.lastname: Palmieri
authors.forename: Daniela
authors.initials: D
authors.lastname: Coppola
authors.forename: Francesco
authors.initials: F
authors.lastname: Lanza
authors.forename: Carlo
authors.initials: C
authors.lastname: Ruosi
authors.forename: Aldo
authors.initials: A
authors.lastname: Amoriello
authors.forename: Giovanni
authors.initials: G
authors.lastname: Di Minno
mesh_terms: D000293:Adolescent; D016063:Blood Loss, Surgical / Q000517:prevention & control; D001791:Blood Platelet Disorders / Q000188:drug therapy; D004334:Drug Administration Schedule; D005167:Factor VII / Q000627:therapeutic use*; D015942:Factor VIIa; D005260:Female; D006487:Hemostasis / Q000187:drug effects*; D006801:Humans; D019637:Orthopedic Procedures / Q000379:methods*; D011994:Recombinant Proteins / Q000627:therapeutic use; D013921:Thrombocytopenia / Q000188:drug therapy*; D014148:Tranexamic Acid / Q000627:therapeutic use
chemical_list: D011994:Recombinant Proteins; D014148:Tranexamic Acid; D005167:Factor VII; C103587:recombinant FVIIa; D015942:Factor VIIa