Von Willebrand disease medical therapy: Difference between revisions
Prince Djan (talk | contribs) |
Nazia Fuad (talk | contribs) |
||
| Line 14: | Line 14: | ||
==Medical Therapy== | ==Medical Therapy== | ||
Disease Type Treatment Low VWF Desmopressin Type 1 Desmopressin Type 2 Desmopressin (if responsive) or FVIII-VWF concentrate or VWF concentrate Type 3 FVIII-VWF concen | |||
Medical treatment of [[von Willebrand's disease]] involves normalizing the [[von Willebrand factor]] and [[factor VIII]] levels. Endogenous factor levels can be increased by the use of [[desmopressin]] or by infusing exogenous coagulation factors example high-purity or low-purity [[von Willebrand factor]] concentrate.<ref name="pmid17403090">{{cite journal| author=Borel-Derlon A, Federici AB, Roussel-Robert V, Goudemand J, Lee CA, Scharrer I et al.| title=Treatment of severe von Willebrand disease with a high-purity von Willebrand factor concentrate (Wilfactin): a prospective study of 50 patients. | journal=J Thromb Haemost | year= 2007 | volume= 5 | issue= 6 | pages= 1115-24 | pmid=17403090 | doi=10.1111/j.1538-7836.2007.02562.x | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=17403090 }} </ref><ref name="pmid15086321">{{cite journal| author=Lethagen S, Carlson M, Hillarp A| title=A comparative in vitro evaluation of six von Willebrand factor concentrates. | journal=Haemophilia | year= 2004 | volume= 10 | issue= 3 | pages= 243-9 | pmid=15086321 | doi=10.1111/j.1365-2516.2004.00893.x | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15086321 }} </ref> | Medical treatment of [[von Willebrand's disease]] involves normalizing the [[von Willebrand factor]] and [[factor VIII]] levels. Endogenous factor levels can be increased by the use of [[desmopressin]] or by infusing exogenous coagulation factors example high-purity or low-purity [[von Willebrand factor]] concentrate.<ref name="pmid17403090">{{cite journal| author=Borel-Derlon A, Federici AB, Roussel-Robert V, Goudemand J, Lee CA, Scharrer I et al.| title=Treatment of severe von Willebrand disease with a high-purity von Willebrand factor concentrate (Wilfactin): a prospective study of 50 patients. | journal=J Thromb Haemost | year= 2007 | volume= 5 | issue= 6 | pages= 1115-24 | pmid=17403090 | doi=10.1111/j.1538-7836.2007.02562.x | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=17403090 }} </ref><ref name="pmid15086321">{{cite journal| author=Lethagen S, Carlson M, Hillarp A| title=A comparative in vitro evaluation of six von Willebrand factor concentrates. | journal=Haemophilia | year= 2004 | volume= 10 | issue= 3 | pages= 243-9 | pmid=15086321 | doi=10.1111/j.1365-2516.2004.00893.x | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15086321 }} </ref> | ||
Revision as of 15:03, 30 August 2018
|
Von Willebrand disease Microchapters |
|
Diagnosis |
|---|
|
Treatment |
|
Case Studies |
|
Von Willebrand disease medical therapy On the Web |
|
American Roentgen Ray Society Images of Von Willebrand disease medical therapy |
|
Risk calculators and risk factors for Von Willebrand disease medical therapy |
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Prince Tano Djan, BSc, MBChB [2]
Overview
The mainstay of management is medical therapy.[1][2][3]
Medical therapy of von Willebrand's disease ( vWD) involves normalizing the von Willebrand factor and factor VIII levels. Endogenous factor levels can be increased by the use of desmopressin or by infusing exogenous coagulation factors example high-purity or low-purity von Willebrand factor concentrate.[4][5]
Pharmacologic medical therapy is recommended in von Willebrand's disease. Medical therapy depends on the type of von Willebrand's disease. Desmopressin is used for type 1 and 2 von Willebrand's disease. von Willebrand factor-factor VIII or von Willebrand factor concentrate is used in some of type 2 von Willebrand's disease and all of type 3 von Willebrand's disease. Alternate or additional therapy involves the use of tranexamic acid, IL-11 [6][7] or aminocaproic acid.
Medical Therapy
Disease Type Treatment Low VWF Desmopressin Type 1 Desmopressin Type 2 Desmopressin (if responsive) or FVIII-VWF concentrate or VWF concentrate Type 3 FVIII-VWF concen
Medical treatment of von Willebrand's disease involves normalizing the von Willebrand factor and factor VIII levels. Endogenous factor levels can be increased by the use of desmopressin or by infusing exogenous coagulation factors example high-purity or low-purity von Willebrand factor concentrate.[4][5]
Pharmacologic medical therapy is recommended in von Willebrand's disease patients as shown below:[1][2][3]
| Type | Treatment | Additional/Alternative treatement |
|---|---|---|
| Low vWF | Desmopressin administered intravenously 0.3μg per kilogram body weight,
intranasally 300μg (150μg per nostril); in patients with body weight <50Kg, only one dose of 150μg or subcutaneously 0.3μg/kilogram |
Tranexamic acid 1g 3 to 4 times daily |
| 1 | Demospressin at same dose as above | Tranexamic acid 1g 3 to 4 times daily |
| 2 | Demospressin at same dose as above or von Willebrand factor-Factor VIII or von Willebrand factor concentrate | Tranexamic acid 1g 3 to 4 times daily |
| 3 | Von Willebrand factor-Factor VIII or von Willebrand factor concentrate | Tranexamic acid 1g 3 to 4 times daily |
Desmopressin is contraindicated in patients with type 2B disease.
For women with heavy menstrual bleeding, the combined oral contraceptive pill may be effective in reducing bleeding or in reducing the length or frequency of periods. Prophylactic treatment is sometimes given for patients with von Willebrand's disease who are scheduled for surgery. They can be treated with human derived medium purity factor VIII concentrates complexed to von Willebrand factor. Desmopressin works by raising the patient's own plasma levels of von Willebrand factor by inducing release of von Willebrand factor stored in the Weibel-Palade bodies in the endothelial cells.
Daily subcutaneous administration of interleukin-11 have been used with success as an alternative modality which increases von Willebrand factor and factor VIII levels by a factor of 1.3 to 2. It is presumabed to increase von Willebrand factor messenger RNA levels in patients with type 1 von Willebrand’s disease that is unresponsive to treatment with desmopressin.[6] Interleukin-11 reduced the severity of bleeding in women with menorrhagia.[7]
References
- ↑ 1.0 1.1 Leissinger C, Carcao M, Gill JC, Journeycake J, Singleton T, Valentino L (2014). "Desmopressin (DDAVP) in the management of patients with congenital bleeding disorders". Haemophilia. 20 (2): 158–67. doi:10.1111/hae.12254. PMID 23937614.
- ↑ 2.0 2.1 Lavin M, O'Donnell JS (2016). "New treatment approaches to von Willebrand disease". Hematology Am Soc Hematol Educ Program. 2016 (1): 683–689. doi:10.1182/asheducation-2016.1.683. PMID 27913547.
- ↑ 3.0 3.1 Castaman G, Goodeve A, Eikenboom J, European Group on von Willebrand Disease (2013). "Principles of care for the diagnosis and treatment of von Willebrand disease". Haematologica. 98 (5): 667–74. doi:10.3324/haematol.2012.077263. PMC 3640108. PMID 23633542.
- ↑ 4.0 4.1 Borel-Derlon A, Federici AB, Roussel-Robert V, Goudemand J, Lee CA, Scharrer I; et al. (2007). "Treatment of severe von Willebrand disease with a high-purity von Willebrand factor concentrate (Wilfactin): a prospective study of 50 patients". J Thromb Haemost. 5 (6): 1115–24. doi:10.1111/j.1538-7836.2007.02562.x. PMID 17403090.
- ↑ 5.0 5.1 Lethagen S, Carlson M, Hillarp A (2004). "A comparative in vitro evaluation of six von Willebrand factor concentrates". Haemophilia. 10 (3): 243–9. doi:10.1111/j.1365-2516.2004.00893.x. PMID 15086321.
- ↑ 6.0 6.1 Ragni MV, Novelli EM, Murshed A, Merricks EP, Kloos MT, Nichols TC (2013). "Phase II prospective open-label trial of recombinant interleukin-11 in desmopressin-unresponsive von Willebrand disease and mild or moderate haemophilia A." Thromb Haemost. 109 (2): 248–54. doi:10.1160/TH12-06-0447. PMC 3689588. PMID 23238591.
- ↑ 7.0 7.1 Ragni MV, Jankowitz RC, Jaworski K, Merricks EP, Kloos MT, Nichols TC (2011). "Phase II prospective open-label trial of recombinant interleukin-11 in women with mild von Willebrand disease and refractory menorrhagia". Thromb Haemost. 106 (4): 641–5. doi:10.1160/TH11-04-0274. PMC 3947632. PMID 21833452.