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. 2021 Jan;19(1):96-106.
doi: 10.1111/jth.15116. Epub 2020 Oct 23.

Increasing levels of von Willebrand factor and factor VIII with age in patients affected by von Willebrand disease

Eugenia Biguzzi  1 , Simona Maria Siboni  1 , Saskia le Cessie  2   3 , Luciano Baronciani  1 , Frits R Rosendaal  2 , Astrid van Hylckama Vlieg  2 , Flora Peyvandi  1   4
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Free article

Increasing levels of von Willebrand factor and factor VIII with age in patients affected by von Willebrand disease

Eugenia Biguzzi et al. J Thromb Haemost. 2021 Jan.
Free article

Abstract

Essentials VWF and FVIII increase with age in patients affected by VWD. VWF and FVIII increase in type 1 and in low levels of VWF patients. VWF and FVIII do not increase in type 1 Vicenza. FVIII increases in type 2 VWD patients. ABSTRACT: Background Increasing levels of von Willebrand factor (VWF) and factor VIII (FVIII:C) was associated with age in type 1 von Willebrand disease (VWD). Objectives To evaluate VWF and FVIII:C increase with age in a large group of patients with VWD and low levels of VWF, in whom levels were repeatedly measured. Methods Clinical charts from all patients evaluated at the A. Bianchi Bonomi Center between 1970 and 2018 were reviewed and data on VWF and FVIII:C collected. Patients affected by type 3, severe type 1 and 2N VWD were excluded. The repeated measurements were evaluated by linear mixed-effects models. A linear association between age and VWF/FVIII:C was shown after the age of 40 years in the linear mixed models and analyzed by calculating the regression slope coefficient (β). Results A total of 617 patients were included in the study (314 type 2, 112 type 1, 181 low VWF levels), with a median age at first measurement of 28 years (interquartile range 14/42) and a mean follow-up of 16 years (standard deviation 11). VWF and FVIII:C increased with age in the whole group. The increase became linear after the age of 40 years (3.68 and 7.44 IU/dL per decade for VWF:activity and FVIII:C). In type 2, FVIII:C increased with age, whereas an increase of both VWF:activity and FVIII:C were shown in patients with type 1 VWD and low levels of VWF. Conclusions A differential increase of VWF and FVIII:C with age was shown among in different ages and types of VWD.

Keywords: age groups; coagulation factor VIII; coagulation factors; von Willebrand disease; von Willebrand protein.

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References

REFERENCES

    1. Lenting PJ, Christophe OD, Denis CV. von Willebrand factor biosynthesis, secretion, and clearance: connecting the far ends. Blood. 2015;125(13):2019-2028.
    1. O'Sullivan JM, Ward S, Lavin M, O'Donnell JS. von Willebrand factor clearance - biological mechanisms and clinical significance. Br J Haematol. 2018;183(2):185-195.
    1. Sadler JE. Von Willebrand disease type 1: a diagnosis in search of a disease. Blood. 2003;101(6):2089-2093.
    1. Flood VH, Christopherson PA, Gill JC, et al. Clinical and laboratory variability in a cohort of patients diagnosed with type 1 VWD in the United States. Blood. 2016;127(20):2481-2488.
    1. Orstavik KH, Magnus P, Reisner H, Berg K, Graham JB, Nance W. Factor VIII and factor IX in a twin population. Evidence for a major effect of ABO locus on factor VIII level. Am J Hum Genet. 1985;37(1):89-101.

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