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Eur J Haematol ; 105(2): 103-115, 2020 Aug.
Artículo en Inglés | MEDLINE | ID: mdl-32277501

RESUMEN

Replacement therapy with plasma-derived or recombinant FVIII and FIX (pdFVIII/pdFIX or rFVIII/rFIX) concentrates is the standard of treatment in patients with haemophilia A and B, respectively. Measurement of factor VIII (FVIII:C) or factor IX (FIX:C) levels can be done by one-stage clotting assay (OSA) or chromogenic substrate assay (CSA). The French study group on the Biology of Hemorrhagic Diseases (a collaborative group of the GFHT and MHEMO network) presents a literature review and proposals for the monitoring of FVIII:C and FIX:C levels in treated haemophilia A and B patients, respectively. The use of CSA is recommended for the monitoring of patients treated with pdFVIII or rFVIII including extended half-life (EHL) rFVIII. Except for rFVIII-Fc, great caution is required when measuring FVIII:C levels by OSA in patients substituted by EHL-rFVIII. The OSA is recommended for the monitoring of patients treated with pdFIX or rFIX. Large discordances in the FIX:C levels measured for extended half-life rFIX (EHL-rFIX), depending on the method and reagents used, must lead to great attention when OSA is used for measuring FIX:C levels in patients substituted by EHL-rFIX. Data of most of recent studies, obtained with spiked plasmas, deserve to be confirmed in plasma samples of treated patients.


Asunto(s)
Factor IX/farmacocinética , Factor VIII/farmacocinética , Hemofilia A/sangre , Hemofilia A/tratamiento farmacológico , Hemofilia B/sangre , Hemofilia B/tratamiento farmacológico , Coagulación Sanguínea , Pruebas de Coagulación Sanguínea/métodos , Toma de Decisiones Clínicas , Manejo de la Enfermedad , Monitoreo de Drogas , Factor IX/administración & dosificación , Factor VIII/administración & dosificación , Hemofilia A/diagnóstico , Hemofilia B/diagnóstico , Humanos , Proteínas Recombinantes/administración & dosificación , Proteínas Recombinantes/farmacocinética , Resultado del Tratamiento
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