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Thrombin generation assay using factor IXa as a trigger to quantify accurately factor VIII levels in haemophilia A.
J Thromb Haemost. 2011 Aug; 9(8):1549-55.JT

Abstract

BACKGROUND

The available methods for measuring factor VIII (FVIII) activity suffer reportedly from lack of sensitivity and precision in the < 1 IU dL(-1) range. This precludes correlation of clinical phenotype with FVIII levels.

OBJECTIVES

To study a possible association between clinical phenotype in patients with FVIII levels < 1 IU dL(-1).

METHODS/RESULTS

The FIXa-driven FVIII assay (FVIII-CAT) has a detection limit of 0.05 IU dL(-1). For the range of 0-2 IU dL(-1) FVIII, the intra-assay coefficient of variation (CV) is around 2% and the inter-assay CV is about 8%. We tested 30 hemophiliacs with FVIII:C between < 1 and 6 IU dL(-1) as measured in the one-stage clotting assay using the FVIII-CAT assay. For genetic defects related to moderate hemophilia, the FVIII-CAT test finds FVIII levels that are in good agreement with those determined with the one-stage assay. Of the 21 hemophilic patients with FVIII < 1 IU dL(-1), four patients exhibited a mild bleeding phenotype. When we applied TF-initiated thrombin generation, patients with a mild clinical phenotype showed significantly higher endogenous thrombin potentials.

CONCLUSION

The novel developed FVIII assay measures accurately FVIII levels below 1 IU dL(-1). Its application demonstrated that the clinical heterogeneity in individuals with < 1 IU dL(-1) FVIII is not associated with their FVIII level.

Authors+Show Affiliations

Synapse BV, Maastricht, The Netherlands.No affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article
Research Support, Non-U.S. Gov't

Language

eng

PubMed ID

21605333

Citation

Ninivaggi, M, et al. "Thrombin Generation Assay Using Factor IXa as a Trigger to Quantify Accurately Factor VIII Levels in Haemophilia A." Journal of Thrombosis and Haemostasis : JTH, vol. 9, no. 8, 2011, pp. 1549-55.
Ninivaggi M, Dargaud Y, van Oerle R, et al. Thrombin generation assay using factor IXa as a trigger to quantify accurately factor VIII levels in haemophilia A. J Thromb Haemost. 2011;9(8):1549-55.
Ninivaggi, M., Dargaud, Y., van Oerle, R., de Laat, B., Hemker, H. C., & Lindhout, T. (2011). Thrombin generation assay using factor IXa as a trigger to quantify accurately factor VIII levels in haemophilia A. Journal of Thrombosis and Haemostasis : JTH, 9(8), 1549-55. https://doi.org/10.1111/j.1538-7836.2011.04358.x
Ninivaggi M, et al. Thrombin Generation Assay Using Factor IXa as a Trigger to Quantify Accurately Factor VIII Levels in Haemophilia A. J Thromb Haemost. 2011;9(8):1549-55. PubMed PMID: 21605333.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Thrombin generation assay using factor IXa as a trigger to quantify accurately factor VIII levels in haemophilia A. AU - Ninivaggi,M, AU - Dargaud,Y, AU - van Oerle,R, AU - de Laat,B, AU - Hemker,H C, AU - Lindhout,T, PY - 2011/5/25/entrez PY - 2011/5/25/pubmed PY - 2012/2/4/medline SP - 1549 EP - 55 JF - Journal of thrombosis and haemostasis : JTH JO - J Thromb Haemost VL - 9 IS - 8 N2 - BACKGROUND: The available methods for measuring factor VIII (FVIII) activity suffer reportedly from lack of sensitivity and precision in the < 1 IU dL(-1) range. This precludes correlation of clinical phenotype with FVIII levels. OBJECTIVES: To study a possible association between clinical phenotype in patients with FVIII levels < 1 IU dL(-1). METHODS/RESULTS: The FIXa-driven FVIII assay (FVIII-CAT) has a detection limit of 0.05 IU dL(-1). For the range of 0-2 IU dL(-1) FVIII, the intra-assay coefficient of variation (CV) is around 2% and the inter-assay CV is about 8%. We tested 30 hemophiliacs with FVIII:C between < 1 and 6 IU dL(-1) as measured in the one-stage clotting assay using the FVIII-CAT assay. For genetic defects related to moderate hemophilia, the FVIII-CAT test finds FVIII levels that are in good agreement with those determined with the one-stage assay. Of the 21 hemophilic patients with FVIII < 1 IU dL(-1), four patients exhibited a mild bleeding phenotype. When we applied TF-initiated thrombin generation, patients with a mild clinical phenotype showed significantly higher endogenous thrombin potentials. CONCLUSION: The novel developed FVIII assay measures accurately FVIII levels below 1 IU dL(-1). Its application demonstrated that the clinical heterogeneity in individuals with < 1 IU dL(-1) FVIII is not associated with their FVIII level. SN - 1538-7836 UR - https://www.unboundmedicine.com/medline/citation/21605333/Thrombin_generation_assay_using_factor_IXa_as_a_trigger_to_quantify_accurately_factor_VIII_levels_in_haemophilia_A_ L2 - https://doi.org/10.1111/j.1538-7836.2011.04358.x DB - PRIME DP - Unbound Medicine ER -