Treatment of Acquired von Willebrand Syndrome in Lymphoproliferative Neoplasm — MGUS, Multiple Myeloma, and Waldenström Macroglobulinemia
Clinical Scenario
Acquired von Willebrand syndrome (AvWS) can arise in patients with a lymphoproliferative neoplasm, including monoclonal gammopathy of undetermined significance (MGUS), multiple myeloma, and Waldenström macroglobulinemia.
Both plasma cell tumour subtypes and lymphocyte tumour subtypes fall within this clinical context, and managing the haemostatic deficit requires an approach informed by the specific underlying diagnosis.
Important exception: IgM MGUS shows a markedly different response profile compared with IgG MGUS and the other subtypes in this group; the protocol accounts for this distinction.
First-Line Treatment Approach (Overview Only)
The recommended approach for this setting centres on high-dose intravenous immunoglobulin therapy as the primary intervention. The complete protocol defines precise dosing, administration schedule, repeat criteria, and alternative pathways — none of which are reproduced here.
Clinical Goals
The therapeutic aim is an increase in von Willebrand factor measurements and a measurable reduction in bleeding time. A response is expected to become visible within 2–4 days of initiating treatment.
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.
Doses of 1 g/kg per day were administered for 2 days, with an increase in vWF measurements the day after the second infusion and a reduction in bleeding time observed the day after the second infusion (subsequent IVIG administrations can be repeated every 21 days).
The effect is visible after 2–4 days and may last for up to 3–6 weeks.
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