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Influence of blood group, von Willebrand factor levels, and age on factor VIII levels in non‐severe haemophilia A

BACKGROUND: Data on the effect of ABO blood group (ABO), von Willebrand factor (VWF) levels, and age on factor VIII (FVIII) in non‐severe haemophilia A (HA) is scarce. OBJECTIVE: To investigate if ABO, VWF levels, and age have an influence on the variability of FVIII levels and consequently on the a...

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Autores principales: Rejtő, Judit, Königsbrügge, Oliver, Grilz, Ella, Hofer, Stefanie, Mauracher, Lisa‐Marie, Gabler, Cornelia, Schuster, Gerhard, Feistritzer, Clemens, Sunder‐Plaßmann, Raute, Quehenberger, Peter, Gebhart, Johanna, Ay, Cihan, Pabinger, Ingrid
Formato: Online Artículo Texto
Lenguaje:English
Publicado: John Wiley and Sons Inc. 2020
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7318586/
https://www.ncbi.nlm.nih.gov/pubmed/32073230
http://dx.doi.org/10.1111/jth.14770
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author Rejtő, Judit
Königsbrügge, Oliver
Grilz, Ella
Hofer, Stefanie
Mauracher, Lisa‐Marie
Gabler, Cornelia
Schuster, Gerhard
Feistritzer, Clemens
Sunder‐Plaßmann, Raute
Quehenberger, Peter
Gebhart, Johanna
Ay, Cihan
Pabinger, Ingrid
author_facet Rejtő, Judit
Königsbrügge, Oliver
Grilz, Ella
Hofer, Stefanie
Mauracher, Lisa‐Marie
Gabler, Cornelia
Schuster, Gerhard
Feistritzer, Clemens
Sunder‐Plaßmann, Raute
Quehenberger, Peter
Gebhart, Johanna
Ay, Cihan
Pabinger, Ingrid
author_sort Rejtő, Judit
collection PubMed
description BACKGROUND: Data on the effect of ABO blood group (ABO), von Willebrand factor (VWF) levels, and age on factor VIII (FVIII) in non‐severe haemophilia A (HA) is scarce. OBJECTIVE: To investigate if ABO, VWF levels, and age have an influence on the variability of FVIII levels and consequently on the assessment of severity in non‐severe HA. PATIENTS/METHODS: Eighty‐nine patients with non‐severe HA and 82 healthy controls were included. Data on ABO was collected and FVIII clotting activity (FVIII:C) with one‐stage clotting assay (FVIII:C OSA) and chromogenic substrate assay (FVIII:C CSA), FVIII antigen (FVIII:Ag) and VWF antigen (VWF:Ag) and activity (VWF:Act) were determined. RESULTS: In HA, FVIII:C OSA and CSA and FVIII:Ag were not different between non‐O (n = 42, median 15.5, interquartile range 10.4‐24.0; 10.0, 6.8‐26.0 and 15.2, 10.7‐24.9) and O (n = 47, 14.1, 9.0‐23.0; 10.0, 5.0‐23.0 and 15.2, 9.3‐35.5), whereas in healthy controls, non‐O individuals had significantly higher FVIII levels. FVIII:C showed no relevant correlation with VWF levels in HA, but we observed strong correlations in healthy controls. Age had only a minor influence in HA, but had a considerable impact on FVIII:C in healthy controls. In multivariable regression analysis ABO, VWF:Ag and age were not associated with FVIII:C in HA, whereas this model explained 61.3% of the FVIII:C variance in healthy controls. CONCLUSIONS: We conclude that in non‐severe HA ABO and VWF levels do not substantially influence the variability of FVIII levels and age has only minor effects on it, which is important information for diagnostic procedures.
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spelling pubmed-73185862020-06-29 Influence of blood group, von Willebrand factor levels, and age on factor VIII levels in non‐severe haemophilia A Rejtő, Judit Königsbrügge, Oliver Grilz, Ella Hofer, Stefanie Mauracher, Lisa‐Marie Gabler, Cornelia Schuster, Gerhard Feistritzer, Clemens Sunder‐Plaßmann, Raute Quehenberger, Peter Gebhart, Johanna Ay, Cihan Pabinger, Ingrid J Thromb Haemost HAEMOSTASIS BACKGROUND: Data on the effect of ABO blood group (ABO), von Willebrand factor (VWF) levels, and age on factor VIII (FVIII) in non‐severe haemophilia A (HA) is scarce. OBJECTIVE: To investigate if ABO, VWF levels, and age have an influence on the variability of FVIII levels and consequently on the assessment of severity in non‐severe HA. PATIENTS/METHODS: Eighty‐nine patients with non‐severe HA and 82 healthy controls were included. Data on ABO was collected and FVIII clotting activity (FVIII:C) with one‐stage clotting assay (FVIII:C OSA) and chromogenic substrate assay (FVIII:C CSA), FVIII antigen (FVIII:Ag) and VWF antigen (VWF:Ag) and activity (VWF:Act) were determined. RESULTS: In HA, FVIII:C OSA and CSA and FVIII:Ag were not different between non‐O (n = 42, median 15.5, interquartile range 10.4‐24.0; 10.0, 6.8‐26.0 and 15.2, 10.7‐24.9) and O (n = 47, 14.1, 9.0‐23.0; 10.0, 5.0‐23.0 and 15.2, 9.3‐35.5), whereas in healthy controls, non‐O individuals had significantly higher FVIII levels. FVIII:C showed no relevant correlation with VWF levels in HA, but we observed strong correlations in healthy controls. Age had only a minor influence in HA, but had a considerable impact on FVIII:C in healthy controls. In multivariable regression analysis ABO, VWF:Ag and age were not associated with FVIII:C in HA, whereas this model explained 61.3% of the FVIII:C variance in healthy controls. CONCLUSIONS: We conclude that in non‐severe HA ABO and VWF levels do not substantially influence the variability of FVIII levels and age has only minor effects on it, which is important information for diagnostic procedures. John Wiley and Sons Inc. 2020-03-20 2020-05 /pmc/articles/PMC7318586/ /pubmed/32073230 http://dx.doi.org/10.1111/jth.14770 Text en © 2020 The Authors. Journal of Thrombosis and Haemostasis published by Wiley Periodicals, Inc. on behalf of International Society on Thrombosis and Haemostasis This is an open access article under the terms of the http://creativecommons.org/licenses/by-nc-nd/4.0/ License, which permits use and distribution in any medium, provided the original work is properly cited, the use is non‐commercial and no modifications or adaptations are made.
spellingShingle HAEMOSTASIS
Rejtő, Judit
Königsbrügge, Oliver
Grilz, Ella
Hofer, Stefanie
Mauracher, Lisa‐Marie
Gabler, Cornelia
Schuster, Gerhard
Feistritzer, Clemens
Sunder‐Plaßmann, Raute
Quehenberger, Peter
Gebhart, Johanna
Ay, Cihan
Pabinger, Ingrid
Influence of blood group, von Willebrand factor levels, and age on factor VIII levels in non‐severe haemophilia A
title Influence of blood group, von Willebrand factor levels, and age on factor VIII levels in non‐severe haemophilia A
title_full Influence of blood group, von Willebrand factor levels, and age on factor VIII levels in non‐severe haemophilia A
title_fullStr Influence of blood group, von Willebrand factor levels, and age on factor VIII levels in non‐severe haemophilia A
title_full_unstemmed Influence of blood group, von Willebrand factor levels, and age on factor VIII levels in non‐severe haemophilia A
title_short Influence of blood group, von Willebrand factor levels, and age on factor VIII levels in non‐severe haemophilia A
title_sort influence of blood group, von willebrand factor levels, and age on factor viii levels in non‐severe haemophilia a
topic HAEMOSTASIS
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7318586/
https://www.ncbi.nlm.nih.gov/pubmed/32073230
http://dx.doi.org/10.1111/jth.14770
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