Mild hemophilia A with factor VIII assay discrepancy: using thrombin generation assay to assess the bleeding phenotype

Introduction: In some patients with mild hemophilia A, there are discrepancies between 1‐stage (1‐st) and 2‐stage (2‐st) factor VIII (FVIII) clotting assays, and also chromogenic assays for FVIII activity (FVIII:C). We examined whether thrombography could provide a better evaluation of the hemostati...

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Published in:Journal of thrombosis and haemostasis 2008-03, Vol.6 (3), p.486-493
Main Authors: TROSSAËRT, M., REGNAULT, V., SIGAUD, M., BOISSEAU, P., FRESSINAUD, E., LECOMPTE, T.
Format: Article
Language:English
Subjects:
Adolescent
Adult
Aged
Automation
Calibration
Case-Control Studies
chromogenic factor VIII
discrepant mild hemophilia
factor VIII
Factor VIII - biosynthesis
Hemophilia A - blood
Hemophilia A - diagnosis
Humans
Male
Mutation, Missense
Pedigree
Phenotype
Point Mutation
Thrombin - metabolism
thrombography
two‐stage factor VIII assay
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Summary:Introduction: In some patients with mild hemophilia A, there are discrepancies between 1‐stage (1‐st) and 2‐stage (2‐st) factor VIII (FVIII) clotting assays, and also chromogenic assays for FVIII activity (FVIII:C). We examined whether thrombography could provide a better evaluation of the hemostatic status of these patients. Methods: Two families with such discrepancies and markedly contrasting clinical histories were studied. Family X had no serious bleedings, in contrast to family Y. Sixty‐one moderate/mild hemophiliacs without discrepancy and 15 healthy subjects served as controls. Calibrated automated thrombography was performed with platelet‐rich plasma after one freeze‐thawing cycle and low tissue factor concentration. Results: The chromogenic FVIII:C levels were higher (0.90 ± 0.15 and 0.47 ± 0.13 IU mL−1) than the 1‐st clotting ones (0.14 ± 0.05 and 0.10 ± 0.05 IU mL−1) in family X and Y, respectively (P 
ISSN:1538-7933
1538-7836
1538-7836
DOI:10.1111/j.1538-7836.2007.02861.x