A matched case-control study comparing features, treatment and outcomes between patients with non-IgM lymphoplasmacytic lymphoma and Waldenström macroglobulinemia.

Cases of non-IgM lymphoplasmacytic lymphoma (LPL) are rare. We performed a case-control study comparing features and outcomes of 31 non-IgM LPL cases and 93 Waldenström macroglobulinemia (WM) controls matched by age, sex, and year of diagnosis. Odds of mutations were lower (odds ratio (OR) 0.22,  = .05), and median time to treatment was shorter in cases than in controls (4 32 months;  < .001). Odds of extramedullary disease were higher (OR 4.20,  = .01), while odds of neuropathy (OR 0.22,  = .25), and hyperviscosity (OR 0.26,  = .26) were lower in cases than in controls. Odds of using chemoimmunotherapy were higher (OR 2.62,  = .11) while odds of using proteasome inhibitors (OR 0.35,  = .15) and BTK inhibitors (OR 0.17,  = .21) were lower in cases than in controls. There were no differences in response and overall survival (OS) between cases and controls. Despite clinicopathological differences, response, and survival outcomes are similar between non-IgM LPL cases and WM controls.