Treatment of Acquired von Willebrand Syndrome in Systemic Mastocytosis

Clinical Scenario

This protocol addresses Acquired von Willebrand syndrome (AvWS) occurring in the context of systemic mastocytosis. In this setting, episodes of mast cell degranulation are linked to the onset or worsening of AvWS, making the underlying mast cell disease a central driver of bleeding risk.

Treatment Approach

Management of AvWS in this context is directed at the underlying mast cell disease activity. A corticosteroid-based intervention is part of the approach, timed to the exacerbation.

The full structured regimen — including agent selection, sequencing, and clinical decision points — is available in the complete protocol.

Instant Access to Structured Evidence-Based Regimens

References

DOI: 10.5603/ahp.107038

In this case, one solution is to administer corticosteroids during an exacerbation of systemic mastocytosis (associated with increased mast cell degranulation).

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