Treatment of Acquired von Willebrand Syndrome in IgM Monoclonal Gammopathy of Undetermined Significance

Acquired von Willebrand syndrome (AvWS) occurring in the setting of IgM monoclonal gammopathy of undetermined significance (IgM MGUS) represents a clinically distinct subgroup that does not respond to therapies effective in other gammopathy subtypes.

Clinical Scenario

In patients with AvWS and IgM MGUS, intravenous immunoglobulins (IVIG) — effective in IgG MGUS — show markedly reduced effectiveness. This subgroup requires a different first-line therapeutic strategy.

Treatment Approach

The recommended first-line intervention for AvWS in IgM MGUS involves an extracorporeal blood-purification procedure. Full eligibility criteria, procedural details, monitoring parameters, and follow-up are available in the complete structured protocol.

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References

DOI: 10.5603/ahp.107038

An exception is IgM MGUS, where the effectiveness of intravenous immunoglobulins is much lower than in IgG MGUS.
IVIG was not effective in patients with AvWS with IgM MGUS (IgM-MGUS).
In this case, the treatment of choice is a total plasma exchange (TPE).

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