Acquired von Willebrand Syndrome in Multiple Myeloma, MGUS, and Waldenström Macroglobulinemia — What to Do After IVIG or vWF/FVIII Concentrate Failure

Clinical Scenario

This protocol applies to patients with acquired von Willebrand syndrome in the setting of a lymphoproliferative neoplasm — specifically multiple myeloma, MGUS (excluding the IgM subtype), or Waldenström macroglobulinemia. In plasma cell and lymphocyte tumors, intravenous immunoglobulins are highly effective in treating and preventing bleeding; an exception is IgM MGUS, where their effectiveness is substantially lower than in IgG MGUS.

Previous Treatment — Goals Not Achieved

This next-line protocol is indicated when high-dose intravenous immunoglobulin (IVIG) or vWF/FVIII concentrate has not achieved the treatment targets: a measurable increase in von Willebrand factor levels and reduction in bleeding time (an effect expected to be visible within 2–4 days).

Next-Line Approach

The escalation strategy centres on plasmapheresis to remove autoantibodies and paraproteins, with specific attention to replacing what is removed during the procedure in order to protect clotting factor levels. The complete indication criteria, procedural specifics, and full treatment algorithm are available in the structured protocol.

Instant Access to Structured Evidence-Based Regimens

References

DOI: 10.5603/ahp.107038

In both patients with plasma cell tumors and those with lymphocyte tumors, intravenous immunoglobulins are highly effective in treating and preventing bleeding.

An exception is IgM MGUS, where the effectiveness of intravenous immunoglobulins is much lower than in IgG MGUS.

Plasmapheresis can be used to remove autoantibodies and paraproteins of any immunoglobulin class, with the efficacy of the procedures in patients with monoclonal gammopathy estimated at 20%.

To prevent deficiencies of fibrinogen and other clotting factors, fresh frozen plasma should be transfused instead of albumin during plasmapheresis.

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