Von Willebrand disease natural history, complications and prognosis: Difference between revisions
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==Overview== | ==Overview== | ||
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Prince Tano Djan, BSc, MBChB [2]
Overview
Natural history, complications and prognosis
- Patients with von willebrand disease have a lower health-related quality of life compared to the general population especially with the bleeding phenotype.[1]
- There is physiologic rise in von Willebrand factor levels throughout life. As a result of this, patients with type 1 von Willebrand’s disease may have normal levels of vWF at older age.[2] However, bleeding symptoms occur at similar frequency in patients older than 65 years as well as in those who are 18 to 65 years of age[3]
- Menorrhagia is a major complication, which also impairs the quality of life.[4]
- Angiodysplasia is serious, and possibly life-threatening complication.[5]
- Intraarticular bleeding (although rare) may be a presenting symptom in those with type 2N or type 3 disease.[6]
- Primary and secondary postpartum bleeding is a commmon complication.[7][8][9]
References
- ↑ de Wee EM, Mauser-Bunschoten EP, Van Der Bom JG, Degenaar-Dujardin ME, Eikenboom HC, Fijnvandraat K, de Goede-Bolder A, Laros-van Gorkom BA, Meijer K, Raat H, Leebeek FW (2010). "Health-related quality of life among adult patients with moderate and severe von Willebrand disease". J. Thromb. Haemost. 8 (7): 1492–9. doi:10.1111/j.1538-7836.2010.03864.x. PMID 20345712.
- ↑ Rydz N, Grabell J, Lillicrap D, James PD (2015). "Changes in von Willebrand factor level and von Willebrand activity with age in type 1 von Willebrand disease". Haemophilia. 21 (5): 636–41. doi:10.1111/hae.12664. PMC 4678413. PMID 25756206.
- ↑ Sanders YV, Giezenaar MA, Laros-van Gorkom BA, Meijer K, van der Bom JG, Cnossen MH, Nijziel MR, Ypma PF, Fijnvandraat K, Eikenboom J, Mauser-Bunschoten EP, Leebeek FW (2014). "von Willebrand disease and aging: an evolving phenotype". J. Thromb. Haemost. 12 (7): 1066–75. doi:10.1111/jth.12586. PMID 24750783.
- ↑ Kadir RA, Edlund M, Von Mackensen S (2010). "The impact of menstrual disorders on quality of life in women with inherited bleeding disorders". Haemophilia. 16 (5): 832–9. doi:10.1111/j.1365-2516.2010.02269.x. PMID 20584085.
- ↑ Makris M, Federici AB, Mannucci PM, Bolton-Maggs PH, Yee TT, Abshire T, Berntorp E (2015). "The natural history of occult or angiodysplastic gastrointestinal bleeding in von Willebrand disease". Haemophilia. 21 (3): 338–42. doi:10.1111/hae.12571. PMID 25381842.
- ↑ van Galen KP, Mauser-Bunschoten EP, Leebeek FW (2012). "Hemophilic arthropathy in patients with von Willebrand disease". Blood Rev. 26 (6): 261–6. doi:10.1016/j.blre.2012.09.002. PMID 23010260.
- ↑ Kouides PA (2015). "An update on the management of bleeding disorders during pregnancy". Curr. Opin. Hematol. 22 (5): 397–405. doi:10.1097/MOH.0000000000000167. PMID 26164463.
- ↑ De Wee EM, Knol HM, Mauser-Bunschoten EP, van der Bom JG, Eikenboom JC, Fijnvandraat K, De Goede-Bolder A, Laros-van Gorkom B, Ypma PF, Zweegman S, Meijer K, Leebeek FW (2011). "Gynaecological and obstetric bleeding in moderate and severe von Willebrand disease". Thromb. Haemost. 106 (5): 885–92. doi:10.1160/TH11-03-0180. PMID 21947221.
- ↑ Stoof SC, van Steenbergen HW, Zwagemaker A, Sanders YV, Cannegieter SC, Duvekot JJ, Leebeek FW, Peters M, Kruip MJ, Eikenboom J (2015). "Primary postpartum haemorrhage in women with von Willebrand disease or carriership of haemophilia despite specialised care: a retrospective survey". Haemophilia. 21 (4): 505–12. doi:10.1111/hae.12635. PMID 25688733.