Waldenstrom macroglobulinemia (WM) is a rare lymphoplasmacytic lymphoma associated with immunoglobulin M monoclonal gammopathy. The majority of patients carry the L265P mutation in MYD88, whereas 40% of patients carry mutations in CXCR4. Mutation status has an impact on response to treatment with ibrutinib, ixazomib, and everolimus, with CXCR4-mutated WM cases demonstrating resistance to these agents.4-6 

Bortezomib, a proteasome inhibitor that is the mainstay of treatment of multiple myeloma, is also effective in WM; however, its relation to CXCR4 mutation status in WM has not been fully explored. In this study, we describe the impact of CXCR4 mutations on progression-free survival and overall survival of WM patients under treatment with a bortezomib-based combination and attempt to frame that in the context of pan-cancer data on CXCR4 expression and bortezomib sensitivity.

Sixty-three patients with...

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