Von Willebrand Disease with a History of Severe and Frequent Bleeds
Clinical Scenario
This protocol addresses patients with Von Willebrand disease (VWD) who have a history of severe and frequent bleeding episodes. In this sub-population, the pattern of recurrent significant bleeds drives a specific long-term management strategy distinct from episodic on-demand treatment.
Guideline Recommendation
Current evidence-based guidelines suggest active, sustained intervention over a period of at least six months rather than an approach of no prophylaxis, based on conditional recommendations from the available evidence.
Treatment Approach (partial overview)
The structured regimen centres on long-term prophylaxis using intravenous VWF concentrate. Several concentrate formulations are available and considered within this protocol.
Specific agent selection, dosing, frequency, and full algorithm are in the complete protocol below.
References
- In patients with VWD with a history of severe and frequent bleeds, the guideline panel suggests using long-term prophylaxis rather than no prophylaxis (conditional recommendation based on low certainty in the evidence of effects).
- Prophylaxis was defined as a period of at least 6 months of treatment consisting of VWF replacement administered at least once weekly.
DOI: 10.1182/bloodadvances.2020003264.
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