Von Willebrand disease: Difference between revisions
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==Background== | ==Background== | ||
*Most common inherited bleeding disorder | *Most common inherited bleeding disorder | ||
*vWF has two roles: | |||
**1. Acts as cofactor for platelet adhesion | |||
**2. Acts as carrier protein for factor VIII extending its half life | |||
*vWD results from quantitative or qualitative dysfunction of Von Willebrand factor | |||
==Clinical Features== | |||
*Skin and mucosal bleeding | |||
**Epistaxis, gingival bleeding, menorrhagia | |||
*Hemarthrosis is unusual | |||
==Diagnosis== | |||
*Bleeding time: prolonged | |||
*PT: normal | |||
*PTT: normal-mildly prolonged | |||
*vWF activity level: low | |||
==Treatment== | ==Treatment== | ||
* | *Avoid ASA, NSAIDs, heparin | ||
* | *Desmopressin | ||
**Induces release of vWF from endothelial storage sites | |||
**0.3mcg/kg IV (max 20mcg) over 30min | |||
*Aminocaproic acid | *Aminocaproic acid | ||
Revision as of 03:01, 13 October 2011
Background
- Most common inherited bleeding disorder
- vWF has two roles:
- 1. Acts as cofactor for platelet adhesion
- 2. Acts as carrier protein for factor VIII extending its half life
- vWD results from quantitative or qualitative dysfunction of Von Willebrand factor
Clinical Features
- Skin and mucosal bleeding
- Epistaxis, gingival bleeding, menorrhagia
- Hemarthrosis is unusual
Diagnosis
- Bleeding time: prolonged
- PT: normal
- PTT: normal-mildly prolonged
- vWF activity level: low
Treatment
- Avoid ASA, NSAIDs, heparin
- Desmopressin
- Induces release of vWF from endothelial storage sites
- 0.3mcg/kg IV (max 20mcg) over 30min
- Aminocaproic acid
Source
Tintinalli