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Influence of blood group, von Willebrand factor levels, and age on factor VIII levels in non‐severe haemophilia A
Author(s) -
Rejtő Judit,
Königsbrügge Oliver,
Grilz Ella,
Hofer Stefanie,
Mauracher LisaMarie,
Gabler Cornelia,
Schuster Gerhard,
Feistritzer Clemens,
SunderPlaßmann Raute,
Quehenberger Peter,
Gebhart Johanna,
Ay Cihan,
Pabinger Ingrid
Publication year - 2020
Publication title -
journal of thrombosis and haemostasis
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.947
H-Index - 178
eISSN - 1538-7836
pISSN - 1538-7933
DOI - 10.1111/jth.14770
Subject(s) - abo blood group system , von willebrand factor , haemophilia , haemophilia a , medicine , von willebrand disease , coagulopathy , interquartile range , clotting factor , immunology , gastroenterology , platelet , surgery
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.