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Use of recombinant factor VIIa (NovoSeven®) in patients with Glanzmann thrombasthenia

2001; Elsevier BV; Volume: 38; Linguagem: Inglês

10.1016/s0037-1963(01)90143-x

1532-8686

Man-Chiu Poon, Roseline d’Oiron, Ian Hann, Claude Négrier, L. de Lumley, Angela Thomas, Anastasia Karafoulidou, Christine Demers, Alison Street, Angela Huth‐Kühne, Pia Petrini, E Fressinaud, Massimo Morfini, Lilian Tengborn, Alain Marquès-Verdier, R Musso, Ömer Devecioğlu, Donald S. Houston, Stefan Lethagen, Chris Van Geet, Mario von Depka, Claire Berger, Philippe Beurrier, Howard A Britton, Willem Gerrits, Christiane Güthner, Stefan Kuhle, Jose Juan Ponce Lorenzo, Pantelis E. Makris, Natascha Nohe, Patricia Paugy, Brigitte Pautard, Marie-Françoise Torchet, Nathalie Trillot, M Vicariot, Jonathan T. Wilde, Mark Winter, Hérvè Chambost, Jørgen Ingerslev, Majorlein Peters, Gabriele Strauß,

Blood properties and coagulation

Recombinant factor VIIa (rFVIIa; NovoSeven®, Novo Nordisk, Bagsvaerd, Denmark) appears effective and relatively safe for the treatment of bleeding and for surgical prophylaxis in patients with Glanzmann thrombasthenia as reported to the International Registry on rFVIIa and Congenital Platelet Disorders. One of the shortcomings of the Registry data is the heterogeneity of treatment protocol, including dosage, number of doses used, duration of treatment before declaration of failure, and mode of rFVIIa administration (bolus v continuous infusion). The data are not yet sufficient to define optimal regimens for various indications such as the type of bleeding or the type of procedures. The place of this drug compared to platelet transfusion in the overall management of patients with Glanzmann thrombasthenia will need to be determined in relationship to a number of challenges and unresolved issues in the clinical care of these patients. These issues include: how to improve local measures for patients with mucosal bleeds, optimal management of young women during menarche, optimal platelet transfusion regimens for various indications, the relationship between antiplatelet antibodies detected by monoclonal antibody-specific immobilization of platelet antigens (MAIPA) and effectiveness of platelet transfusion, whether there are other biological tests that may correlate with effectiveness of platelet transfusion, and management of pregnancy and delivery regarding antiplatelet immunization.