Proteasome Inhibitors in Waldenström Macroglobulinemia.

Waldenstrom macroglobulinemia (WM) remains an incurable B-cell lymphoproliferative disorder, yet therapy is only considered for patients with symptomatic disease. Primary therapy options for WM include combinations based on anti-CD20 monoclonal antibodies, mainly rituximab. However, proteasome inhibitors have become an important part of WM therapy both as primary therapy and as salvage option. Bortezomib is the proteasome inhibitor most studied and with extensive clinical experience, but new proteasome inhibitors (carfilzomib, ixazomib, oprozomib), with different toxicity profiles, routes of administration, and probably with preserved or improved activity, have become available and may also find their way into WM therapy.