Little discrepancy between one-stage and chromogenic factor VIII (FVIII)/IX assays in a large international cohort of persons with nonsevere hemophilia A and B
| Autor: | Zwagemaker, Anne Fleur, Kloosterman, Fabienne R., Gouw, Samantha C., Boyce, Sara, Brons, Paul, Cnossen, Marjon H., Collins, Peter W., Eikenboom, Jeroen, Hay, Charles, Hengeveld, Rutger C.C., Jackson, Shannon, Klopper-Tol, Caroline A.M., Kruip, Marieke J.H.A., Gorkom, Britta Laros van, Male, Christoph, Nieuwenhuizen, Laurens, Shapiro, Susan, Fijnvandraat, Karin, Coppens, Michiel |
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| Přispěvatelé: | Pediatrics, Hematology |
| Rok vydání: | 2023 |
| Předmět: | |
| Zdroj: | Journal of Thrombosis and Haemostasis, 21, 850-861 Journal of Thrombosis and Haemostasis, 21, 4, pp. 850-861 Journal of Thrombosis and Haemostasis, 21(4), 850-861. Wiley-Blackwell Publishing Ltd |
| ISSN: | 1538-7933 |
| Popis: | Item does not contain fulltext BACKGROUND: Accurate measurements of coagulation factor activity form an essential part of hemophilia management and are performed by the one-stage or chromogenic assay. Current literature suggests that approximately one-third of persons with nonsevere hemophilia A exhibit assay discrepancy, albeit with a high variability between studies. Such data are scarce in nonsevere hemophilia B. OBJECTIVES: To investigate the extent of factor VIII/IX one-stage and chromogenic assay discrepancy in moderate and mild hemophilia A and B. METHODS: Persons with previously diagnosed nonsevere hemophilia A and B with a factor level of 2 to 35 IU/dL were included from the international DYNAMO cohort study. Central measurements of the factor VIII and IX activity levels were performed by the one-stage and chromogenic assay. Relative and absolute discrepancy definitions were used, with the International Society on Thrombosis and Haemostasis-Scientific and Standardization Committee proposed ratio of >2.0 or 10 IU/dL between the assay results. In addition, with more lenient definitions, over 90% of participants (n = 197) had no discrepant results. Only 1 out of 13 persons with a mutation previously associated with discrepancy had significant assay discrepancy. CONCLUSION: Little assay discrepancy was observed despite the presence of mutations previously associated with discrepancy, suggesting that the presence and magnitude of assay discrepancy are largely determined by laboratory variables. 01 april 2023 |
| Databáze: | OpenAIRE |
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