Extended Half-Life Factor VIII/Factor IX Products: Assay Discrepancies and Implications for Hemophilia Management

Abstract

Due to structural differences between extended half-life (EHL) factor VIII (FVIII) or FIX products and equivalent plasma wild-type molecules used for assay calibration, reagent-dependent discrepancies during monitoring of FVIII- and FIX-replacement therapies with EHL products have been described. To assess the performance of available one-stage clotting and chromogenic substrate assays on the Siemens Atellica COAG 360 analyzer, an in vitro study using spiked plasma samples was performed. The described results confirm previously described findings and allowed allocation of each EHL product to an appropriate assay. In addition, corresponding EHL product–specific analytes were defined within the order entry system of the University Hospital Bonn. The requirement of product-specific FVIII and FIX assays complicates patient monitoring and demonstrates the need for both continuous education and communication between treating physicians and the coagulation laboratory.

Zusammenfassung

Aufgrund struktureller Unterschiede zwischen FVIII- und FIX-Produkten mit verlängerter Halbwertszeit (EHL) und den zur Kalibration der Testsysteme verwendeten plasmatischen Faktoren kommt es zu reagenzabhängigen Diskrepanzen von im Rahmen der Therapie mit EHL-Konzentraten erhobenen Aktivitätswerten. Zur entsprechenden Überprüfung der uns auf dem Atellica Coag 360 Analysensystem zur Verfügung stehenden Einstufen- und chromogenen Testverfahren wurde eine in-vitro-Studie mit entsprechend präparierten Plasmaproben durchgeführt. Die gefundenen und hier beschriebenen Ergebnisse bestätigen zuvor publizierte Daten und erlaubten die Zuordnung der einzelnen EHL-Produkte zu einem jeweils geeigneten Testverfahren. Die produktspezifischen Testverfahren wurde durch die Anlage entsprechender Analyte im Order-Entry-System des Universitätsklinikums Bonn (UKB) hinterlegt. Die Notwendigkeit produktspezifischer FVIII- und FIX-Tests kompliziert das Patientenmonitoring und verdeutlicht die Notwendigkeit entsprechender Fortbildungen als auch einer guten Kommunikation zwischen behandelndem Arzt und Labor.

Keywords

hemophilia A/B - factor concentrates - extended half-life products - prophylaxis

Schlüsselwörter

Hämophilie A/B - Faktorenkonzentrate - Verlängerte Halbwertszeit - Prophylaxe

Publication History

Received: 15 September 2020

Accepted: 05 October 2020

Article published online:
13 November 2020

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