Literature DB >> 28874064

The Association of Aging With Von Willebrand Factor Levels and Bleeding Risk in Type 1 Von Willebrand Disease.

Craig D Seaman1,2, Margaret V Ragni1,2.   

Abstract

Little is known about aging in von Willebrand disease (VWD). It is uncertain whether VWD patients experience an age-related increase in von Willebrand factor (VWF) levels, and if so, it is unknown whether normalization of VWF levels with aging ameliorates bleeding risk. We aimed to determine the association of age with VWF levels and bleeding risk in patients with type 1 VWD. This is a retrospective chart review of patients with type 1 VWD presenting to the Hemophilia Clinic of Western Pennsylvania for regularly scheduled clinic visits. Data collected included VWF antigen level and condensed molecular and clinical markers for the diagnosis and management of Type 1 (MCMDM-1) VWD bleeding assessment tool (BAT) bleeding score based on bleeding symptoms during the previous 3 years. Thirty-nine patients participated in the study, and 32 were female. The average age of participants was 41.8 ± 18.0 years. The mean VWF antigen level was 0.83 ± 0.37 IU/mL, and the mean bleeding score was 2.51 ± 2.90. The bleeding score was inversely associated with age, β = -0.080 (SE = 0.023), P < .01. There was a nonsignificant association between VWF antigen levels and age. To our knowledge, this is the first report showing an association between aging and decreased bleeding symptoms in patients with type 1 VWD. Determining whether or not bleeding risk is reduced in older patients with type 1 VWD is essential for optimal clinical management. Moreover, VWF concentrate is costly, and unwarranted use represents a significant waste of health-care dollars. These findings warrant further investigation.

Entities:  

Keywords:  aging; bleeding score; von Willebrand disease; von Willebrand factor

Mesh:

Substances:

Year:  2017        PMID: 28874064      PMCID: PMC6714664          DOI: 10.1177/1076029617724232

Source DB:  PubMed          Journal:  Clin Appl Thromb Hemost        ISSN: 1076-0296            Impact factor:   2.389


  11 in total

1.  A quantitative analysis of bleeding symptoms in type 1 von Willebrand disease: results from a multicenter European study (MCMDM-1 VWD).

Authors:  A Tosetto; F Rodeghiero; G Castaman; A Goodeve; A B Federici; J Batlle; D Meyer; E Fressinaud; C Mazurier; J Goudemand; J Eikenboom; R Schneppenheim; U Budde; J Ingerslev; Z Vorlova; D Habart; L Holmberg; S Lethagen; J Pasi; F Hill; I Peake
Journal:  J Thromb Haemost       Date:  2006-04       Impact factor: 5.824

2.  Clinical and laboratory variability in a cohort of patients diagnosed with type 1 VWD in the United States.

Authors:  Veronica H Flood; Pamela A Christopherson; Joan Cox Gill; Kenneth D Friedman; Sandra L Haberichter; Daniel B Bellissimo; Rupa A Udani; Mahua Dasgupta; Raymond G Hoffmann; Margaret V Ragni; Amy D Shapiro; Jeanne M Lusher; Steven R Lentz; Thomas C Abshire; Cindy Leissinger; W Keith Hoots; Marilyn J Manco-Johnson; Ralph A Gruppo; Lisa N Boggio; Kate T Montgomery; Anne C Goodeve; Paula D James; David Lillicrap; Ian R Peake; Robert R Montgomery
Journal:  Blood       Date:  2016-02-09       Impact factor: 22.113

3.  The mutational spectrum of type 1 von Willebrand disease: Results from a Canadian cohort study.

Authors:  Paula D James; Colleen Notley; Carol Hegadorn; Jayne Leggo; Angie Tuttle; Shawn Tinlin; Christine Brown; Chandler Andrews; Andrea Labelle; Yvette Chirinian; Lee O'Brien; Maha Othman; Georges Rivard; Dilys Rapson; Christine Hough; David Lillicrap
Journal:  Blood       Date:  2007-01-01       Impact factor: 22.113

4.  von Willebrand disease and aging: an evolving phenotype.

Authors:  Y V Sanders; M A Giezenaar; B A P Laros-van Gorkom; K Meijer; J G van der Bom; M H Cnossen; M R Nijziel; P F Ypma; K Fijnvandraat; J Eikenboom; E P Mauser-Bunschoten; F W G Leebeek
Journal:  J Thromb Haemost       Date:  2014-07       Impact factor: 5.824

5.  Phenotype and genotype of a cohort of families historically diagnosed with type 1 von Willebrand disease in the European study, Molecular and Clinical Markers for the Diagnosis and Management of Type 1 von Willebrand Disease (MCMDM-1VWD).

Authors:  Anne Goodeve; Jeroen Eikenboom; Giancarlo Castaman; Francesco Rodeghiero; Augusto B Federici; Javier Batlle; Dominique Meyer; Claudine Mazurier; Jenny Goudemand; Reinhard Schneppenheim; Ulrich Budde; Jorgen Ingerslev; David Habart; Zdena Vorlova; Lars Holmberg; Stefan Lethagen; John Pasi; Frank Hill; Mohammad Hashemi Soteh; Luciano Baronciani; Christer Hallden; Andrea Guilliatt; Will Lester; Ian Peake
Journal:  Blood       Date:  2006-09-19       Impact factor: 22.113

6.  Generation and validation of the Condensed MCMDM-1VWD Bleeding Questionnaire for von Willebrand disease.

Authors:  M Bowman; G Mundell; J Grabell; W M Hopman; D Rapson; D Lillicrap; P James
Journal:  J Thromb Haemost       Date:  2008-10-04       Impact factor: 5.824

7.  Determinants of bleeding phenotype in adult patients with moderate or severe von Willebrand disease.

Authors:  Eva M de Wee; Yvonne V Sanders; Eveline P Mauser-Bunschoten; Johanna G van der Bom; Manon E L Degenaar-Dujardin; Jeroen Eikenboom; Arja de Goede-Bolder; Britta A P Laros-van Gorkom; Karina Meijer; Karly Hamulyák; Marten R Nijziel; Karin Fijnvandraat; Frank W G Leebeek
Journal:  Thromb Haemost       Date:  2012-08-23       Impact factor: 5.249

8.  Von Willebrand factor in Italian centenarians.

Authors:  Raffaella Coppola; Daniela Mari; Antonella Lattuada; Claudio Franceschi
Journal:  Haematologica       Date:  2003-01       Impact factor: 9.941

Review 9.  von Willebrand disease: advances in pathogenetic understanding, diagnosis, and therapy.

Authors:  David Lillicrap
Journal:  Blood       Date:  2013-09-24       Impact factor: 22.113

10.  Changes in von Willebrand factor level and von Willebrand activity with age in type 1 von Willebrand disease.

Authors:  N Rydz; J Grabell; D Lillicrap; P D James
Journal:  Haemophilia       Date:  2015-03-10       Impact factor: 4.287

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