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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.<ref name="pmid25756206">{{cite journal |vauthors=Rydz N, Grabell J, Lillicrap D, James PD |title=Changes in von Willebrand factor level and von Willebrand activity with age in type 1 von Willebrand disease |journal=Haemophilia |volume=21 |issue=5 |pages=636–41 |year=2015 |pmid=25756206 |pmc=4678413 |doi=10.1111/hae.12664 |url=}}</ref> 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<ref name="pmid24750783">{{cite journal |vauthors=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 |title=von Willebrand disease and aging: an evolving phenotype |journal=J. Thromb. Haemost. |volume=12 |issue=7 |pages=1066–75 |year=2014 |pmid=24750783 |doi=10.1111/jth.12586 |url=}}</ref> Menorrhagia is a major complication, which also impairs the quality of life.<ref name="pmid20584085">{{cite journal |vauthors=Kadir RA, Edlund M, Von Mackensen S |title=The impact of menstrual disorders on quality of life in women with inherited bleeding disorders |journal=Haemophilia |volume=16 |issue=5 |pages=832–9 |year=2010 |pmid=20584085 |doi=10.1111/j.1365-2516.2010.02269.x |url=}}</ref> Angiodysplasia is serious, and possibly life-threatening complication.<ref name="pmid25381842">{{cite journal |vauthors=Makris M, Federici AB, Mannucci PM, Bolton-Maggs PH, Yee TT, Abshire T, Berntorp E |title=The natural history of occult or angiodysplastic gastrointestinal bleeding in von Willebrand disease |journal=Haemophilia |volume=21 |issue=3 |pages=338–42 |year=2015 |pmid=25381842 |doi=10.1111/hae.12571 |url=}}</ref> Intraarticular bleeding (although rare) may be a presenting symptom in those with type 2N or type 3 disease.<ref name="pmid23010260">{{cite journal |vauthors=van Galen KP, Mauser-Bunschoten EP, Leebeek FW |title=Hemophilic arthropathy in patients with von Willebrand disease |journal=Blood Rev. |volume=26 |issue=6 |pages=261–6 |year=2012 |pmid=23010260 |doi=10.1016/j.blre.2012.09.002 |url=}}</ref> Primary and secondary postpartum bleeding is a commmon complication.<ref name="pmid26164463">{{cite journal |vauthors=Kouides PA |title=An update on the management of bleeding disorders during pregnancy |journal=Curr. Opin. Hematol. |volume=22 |issue=5 |pages=397–405 |year=2015 |pmid=26164463 |doi=10.1097/MOH.0000000000000167 |url=}}</ref><ref name="pmid21947221">{{cite journal |vauthors=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 |title=Gynaecological and obstetric bleeding in moderate and severe von Willebrand disease |journal=Thromb. Haemost. |volume=106 |issue=5 |pages=885–92 |year=2011 |pmid=21947221 |doi=10.1160/TH11-03-0180 |url=}}</ref><ref name="pmid25688733">{{cite journal |vauthors=Stoof SC, van Steenbergen HW, Zwagemaker A, Sanders YV, Cannegieter SC, Duvekot JJ, Leebeek FW, Peters M, Kruip MJ, Eikenboom J |title=Primary postpartum haemorrhage in women with von Willebrand disease or carriership of haemophilia despite specialised care: a retrospective survey |journal=Haemophilia |volume=21 |issue=4 |pages=505–12 |year=2015 |pmid=25688733 |doi=10.1111/hae.12635 |url=}}</ref> | 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.<ref name="pmid25756206">{{cite journal |vauthors=Rydz N, Grabell J, Lillicrap D, James PD |title=Changes in von Willebrand factor level and von Willebrand activity with age in type 1 von Willebrand disease |journal=Haemophilia |volume=21 |issue=5 |pages=636–41 |year=2015 |pmid=25756206 |pmc=4678413 |doi=10.1111/hae.12664 |url=}}</ref> 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<ref name="pmid24750783">{{cite journal |vauthors=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 |title=von Willebrand disease and aging: an evolving phenotype |journal=J. Thromb. Haemost. |volume=12 |issue=7 |pages=1066–75 |year=2014 |pmid=24750783 |doi=10.1111/jth.12586 |url=}}</ref> Menorrhagia is a major complication, which also impairs the quality of life.<ref name="pmid20584085">{{cite journal |vauthors=Kadir RA, Edlund M, Von Mackensen S |title=The impact of menstrual disorders on quality of life in women with inherited bleeding disorders |journal=Haemophilia |volume=16 |issue=5 |pages=832–9 |year=2010 |pmid=20584085 |doi=10.1111/j.1365-2516.2010.02269.x |url=}}</ref> Angiodysplasia is serious, and possibly life-threatening complication.<ref name="pmid25381842">{{cite journal |vauthors=Makris M, Federici AB, Mannucci PM, Bolton-Maggs PH, Yee TT, Abshire T, Berntorp E |title=The natural history of occult or angiodysplastic gastrointestinal bleeding in von Willebrand disease |journal=Haemophilia |volume=21 |issue=3 |pages=338–42 |year=2015 |pmid=25381842 |doi=10.1111/hae.12571 |url=}}</ref> Intraarticular bleeding (although rare) may be a presenting symptom in those with type 2N or type 3 disease.<ref name="pmid23010260">{{cite journal |vauthors=van Galen KP, Mauser-Bunschoten EP, Leebeek FW |title=Hemophilic arthropathy in patients with von Willebrand disease |journal=Blood Rev. |volume=26 |issue=6 |pages=261–6 |year=2012 |pmid=23010260 |doi=10.1016/j.blre.2012.09.002 |url=}}</ref> Primary and secondary postpartum bleeding is a commmon complication.<ref name="pmid26164463">{{cite journal |vauthors=Kouides PA |title=An update on the management of bleeding disorders during pregnancy |journal=Curr. Opin. Hematol. |volume=22 |issue=5 |pages=397–405 |year=2015 |pmid=26164463 |doi=10.1097/MOH.0000000000000167 |url=}}</ref><ref name="pmid21947221">{{cite journal |vauthors=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 |title=Gynaecological and obstetric bleeding in moderate and severe von Willebrand disease |journal=Thromb. Haemost. |volume=106 |issue=5 |pages=885–92 |year=2011 |pmid=21947221 |doi=10.1160/TH11-03-0180 |url=}}</ref><ref name="pmid25688733">{{cite journal |vauthors=Stoof SC, van Steenbergen HW, Zwagemaker A, Sanders YV, Cannegieter SC, Duvekot JJ, Leebeek FW, Peters M, Kruip MJ, Eikenboom J |title=Primary postpartum haemorrhage in women with von Willebrand disease or carriership of haemophilia despite specialised care: a retrospective survey |journal=Haemophilia |volume=21 |issue=4 |pages=505–12 |year=2015 |pmid=25688733 |doi=10.1111/hae.12635 |url=}}</ref> | ||
==Natural history, | ==Natural history, Complications and Prognosis== | ||
=== Natural History === | |||
=== Complications === | |||
=== prognosis === | |||
*Patients with von willebrand disease have a lower health-related quality of life compared to the general population especially with the bleeding phenotype.<ref name="pmid20345712">{{cite journal |vauthors=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 |title=Health-related quality of life among adult patients with moderate and severe von Willebrand disease |journal=J. Thromb. Haemost. |volume=8 |issue=7 |pages=1492–9 |year=2010 |pmid=20345712 |doi=10.1111/j.1538-7836.2010.03864.x |url=}}</ref> | *Patients with von willebrand disease have a lower health-related quality of life compared to the general population especially with the bleeding phenotype.<ref name="pmid20345712">{{cite journal |vauthors=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 |title=Health-related quality of life among adult patients with moderate and severe von Willebrand disease |journal=J. Thromb. Haemost. |volume=8 |issue=7 |pages=1492–9 |year=2010 |pmid=20345712 |doi=10.1111/j.1538-7836.2010.03864.x |url=}}</ref> | ||
*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.<ref name="pmid25756206">{{cite journal |vauthors=Rydz N, Grabell J, Lillicrap D, James PD |title=Changes in von Willebrand factor level and von Willebrand activity with age in type 1 von Willebrand disease |journal=Haemophilia |volume=21 |issue=5 |pages=636–41 |year=2015 |pmid=25756206 |pmc=4678413 |doi=10.1111/hae.12664 |url=}}</ref> 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<ref name="pmid24750783">{{cite journal |vauthors=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 |title=von Willebrand disease and aging: an evolving phenotype |journal=J. Thromb. Haemost. |volume=12 |issue=7 |pages=1066–75 |year=2014 |pmid=24750783 |doi=10.1111/jth.12586 |url=}}</ref> | *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.<ref name="pmid25756206">{{cite journal |vauthors=Rydz N, Grabell J, Lillicrap D, James PD |title=Changes in von Willebrand factor level and von Willebrand activity with age in type 1 von Willebrand disease |journal=Haemophilia |volume=21 |issue=5 |pages=636–41 |year=2015 |pmid=25756206 |pmc=4678413 |doi=10.1111/hae.12664 |url=}}</ref> 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<ref name="pmid24750783">{{cite journal |vauthors=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 |title=von Willebrand disease and aging: an evolving phenotype |journal=J. Thromb. Haemost. |volume=12 |issue=7 |pages=1066–75 |year=2014 |pmid=24750783 |doi=10.1111/jth.12586 |url=}}</ref> | ||
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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
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]
Natural history, Complications and Prognosis
Natural History
Complications
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
- ↑ 1.0 1.1 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.
- ↑ 2.0 2.1 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.
- ↑ 3.0 3.1 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.
- ↑ 4.0 4.1 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.
- ↑ 5.0 5.1 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.
- ↑ 6.0 6.1 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.
- ↑ 7.0 7.1 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.
- ↑ 8.0 8.1 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.
- ↑ 9.0 9.1 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.