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This algorithm outlines treatment of symptomatic Waldenström macroglobulinemia. Hyperviscosity is addressed first as an emergency: plasmapheresis is performed before or together with systemic therapy.
In the frontline setting, the chart offers two approaches. Continuous BTK inhibitor therapy with zanubrutinib or ibrutinib is supported by ASPEN, and fixed-duration chemoimmunotherapy with bendamustine plus rituximab is the alternative. For relapsed or refractory disease, treatment is chosen according to prior therapy, switching to an alternate BTK inhibitor, bendamustine plus rituximab, or a proteasome inhibitor-based regimen. The chart provides no trial summary for ASPEN or the other branches, so the overview is limited to the sequence shown: urgent plasmapheresis when hyperviscosity is present, then a BTK inhibitor or bendamustine plus rituximab frontline, and a class switch at relapse.
Starting point: Waldenström macroglobulinemia (symptomatic).