Von Willebrand disease: Difference between revisions
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==Treatment== | ==Treatment== | ||
*Avoid ASA, NSAIDs, heparin | *Avoid ASA, NSAIDs, heparin | ||
*Intermediate purity factor VIII | |||
**Goal to increase VWF activity by 50-100% | |||
**Initial infusion of 20-40 IU/Kg | |||
**High replacement doses may be indicated in more severe disease | |||
*Platelet transfusion | |||
**consider if replacement therapy instituted and persistent bleeding | |||
*Desmopressin | *Desmopressin | ||
**Induces release of vWF from endothelial storage sites | **Induces release of vWF from endothelial storage sites | ||
**0.3mcg/kg IV (max 20mcg) over 30min | **0.3mcg/kg IV (max 20mcg) over 30min | ||
*Aminocaproic acid | *Aminocaproic acid | ||
*Recombinant Factor VIIa | |||
**Consider in type 3 VWD patients who have developed antibodies to VWF replacement | |||
**Increased risk of thrombosis, especially in patients with coronary artery disease | |||
==Source== | ==Source== | ||
Revision as of 04:28, 26 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
- Intermediate purity factor VIII
- Goal to increase VWF activity by 50-100%
- Initial infusion of 20-40 IU/Kg
- High replacement doses may be indicated in more severe disease
- Platelet transfusion
- consider if replacement therapy instituted and persistent bleeding
- Desmopressin
- Induces release of vWF from endothelial storage sites
- 0.3mcg/kg IV (max 20mcg) over 30min
- Aminocaproic acid
- Recombinant Factor VIIa
- Consider in type 3 VWD patients who have developed antibodies to VWF replacement
- Increased risk of thrombosis, especially in patients with coronary artery disease
Source
Tintinalli