An Update on Laboratory Diagnostics in Haemophilia A and B
Müller, J.; Miesbach, W.; Prüller, F.; Siegemund, T.; Scholz, U.; Sachs, U.-J. · Zeitschrift für Orthopädie und Unfallchirurgie · 2022 · Heft 04 · S. 248 bis 260
Bibliografische Angaben
Zusammenfassung
AbstractHaemophilia A (HA) and B (HB) are X-linked hereditary bleeding disorders caused by lack of activity of coagulation factors VIII (FVIII) or IX (FIX), respectively. Besides conventional products, modern replacement therapies include FVIII or FIX concentrates with an extended half-life (EHL-FVIII/FIX). Two main strategies for measuring plasma FVIII or FIX activity are applied: the one-stage clotting assay (OSCA) and the chromogenic substrate assay (CSA), both calibrated against plasma (FVIII/FIX) standards. Due to the structural modifications of EHL-FVIII/FIX, reagent-dependent assay discrepancies have been…