Von Willebrand disease natural history, complications and prognosis: Difference between revisions
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==Overview== | ==Overview== | ||
Patients with | Patients with VWD can become symptomatic at any age. A typical history in a patient with mild to moderate disease includes epistaxis lasting longer than 10 minutes in childhood lifelong easy bruising, bleeding following dental extractions, other invasive dental procedures, or other forms of surgery. Women with VWD usually have a history of heavy menstrual bleeding and may have bleeding during the peripartum period, often at or within hours of delivery and at 5 to 10 days after delivery. Menorrhagia is a major complication. Angiodysplasia is serious, and possibly life-threatening complication. Intraarticular bleeding may be a presenting symptom in those with type 2N or type 3 disease. For some patients, vWD is a mild bleeding disorder and can be managed easily. Patients with mild disease may experience clinically severe hemorrhage following trauma or invasive procedures. Variability of symptomatology exists among family members. People with vWD types II and III face severe and potentially life threatening bleeding episodes. Type III disease patients have low FVIII levels and present with arthropathies. Levels of vWF normally increase with age in patients with type I vWD, In patients with type II vWD, vWF levels does not increase with aging. | ||
==Natural history, Complications and Prognosis== | ==Natural history, Complications and Prognosis== | ||
=== Natural History === | === Natural History === | ||
* Patients with VWD can become symptomatic at any age. | |||
* A typical history in a patient with mild to moderate disease includes | |||
** Epistaxis lasting longer than 10 minutes in childhood | |||
** Lifelong easy bruising | |||
** Bleeding with or following dental extractions, other invasive dental procedures, or other forms of surgery. | |||
* Women with VWD usually have a history of heavy menstrual bleeding and may have bleeding during the peripartum period, often at or within hours of delivery and at 5 to 10 days after delivery. The bleeding may be delayed until 14 to 21 days postpartum, or persists. | |||
=== Complications === | === Complications === | ||
Complications of vWD include the followings:<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><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><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><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> | |||
* Menorrhagia is a major complication, which also impairs the quality of life. | |||
*Angiodysplasia is serious, and possibly life-threatening complication. | |||
*Intraarticular bleeding may be a presenting symptom in those with type 2N or type 3 disease. | |||
*Primary and secondary postpartum bleeding is a commmon complication. | |||
=== Prognosis === | |||
* For some patients, vWD is a mild bleeding disorder and can be managed easily. | |||
* Patients with mild disease may experience clinically severe hemorrhage following trauma or invasive procedures. | |||
* Variability of symptomatology exists among family members. | |||
* People with vWD types II and III face severe and potentially life threatening bleeding episodes. | |||
* Type III disease patients have low FVIII levels and present with arthropathies. | |||
* Levels of vWF normally increase with age in patients with type I vWD, | |||
* | * Elderly patients with type I exhibit no change in their pattern of bleeding though.<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> | ||
* | * In patients with type II vWD, vWF levels does not increase with aging. | ||
* | |||
==References== | ==References== | ||
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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 VWD can become symptomatic at any age. A typical history in a patient with mild to moderate disease includes epistaxis lasting longer than 10 minutes in childhood lifelong easy bruising, bleeding following dental extractions, other invasive dental procedures, or other forms of surgery. Women with VWD usually have a history of heavy menstrual bleeding and may have bleeding during the peripartum period, often at or within hours of delivery and at 5 to 10 days after delivery. Menorrhagia is a major complication. Angiodysplasia is serious, and possibly life-threatening complication. Intraarticular bleeding may be a presenting symptom in those with type 2N or type 3 disease. For some patients, vWD is a mild bleeding disorder and can be managed easily. Patients with mild disease may experience clinically severe hemorrhage following trauma or invasive procedures. Variability of symptomatology exists among family members. People with vWD types II and III face severe and potentially life threatening bleeding episodes. Type III disease patients have low FVIII levels and present with arthropathies. Levels of vWF normally increase with age in patients with type I vWD, In patients with type II vWD, vWF levels does not increase with aging.
Natural history, Complications and Prognosis
Natural History
- Patients with VWD can become symptomatic at any age.
- A typical history in a patient with mild to moderate disease includes
- Epistaxis lasting longer than 10 minutes in childhood
- Lifelong easy bruising
- Bleeding with or following dental extractions, other invasive dental procedures, or other forms of surgery.
- Women with VWD usually have a history of heavy menstrual bleeding and may have bleeding during the peripartum period, often at or within hours of delivery and at 5 to 10 days after delivery. The bleeding may be delayed until 14 to 21 days postpartum, or persists.
Complications
Complications of vWD include the followings:[1][2][3][4][5]
- Menorrhagia is a major complication, which also impairs the quality of life.
- Angiodysplasia is serious, and possibly life-threatening complication.
- Intraarticular bleeding may be a presenting symptom in those with type 2N or type 3 disease.
- Primary and secondary postpartum bleeding is a commmon complication.
Prognosis
- For some patients, vWD is a mild bleeding disorder and can be managed easily.
- Patients with mild disease may experience clinically severe hemorrhage following trauma or invasive procedures.
- Variability of symptomatology exists among family members.
- People with vWD types II and III face severe and potentially life threatening bleeding episodes.
- Type III disease patients have low FVIII levels and present with arthropathies.
- Levels of vWF normally increase with age in patients with type I vWD,
- Elderly patients with type I exhibit no change in their pattern of bleeding though.[6]
- In patients with type II vWD, vWF levels does not increase with aging.
References
- ↑ 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.
- ↑ 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.