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

Abstract

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 MYD88 mutations were lower (odds ratio (OR) 0.22, p = .05), and median time to treatment was shorter in cases than in controls (4 vs. 32 months; p < .001). Odds of extramedullary disease were higher (OR 4.20, p = .01), while odds of neuropathy (OR 0.22, p = .25), and hyperviscosity (OR 0.26, p = .26) were lower in cases than in controls. Odds of using chemoimmunotherapy were higher (OR 2.62, p = .11) while odds of using proteasome inhibitors (OR 0.35, p = .15) and BTK inhibitors (OR 0.17, p = .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.

Keywords:

• Waldenstrom’s macroglobulinaemia
• lymphoplasmacytic lymphoma
• response
• survival
• case–control study

Acknowledgments

Portions of this research have been presented at the 60th American Society of Hematology Meeting in San Diego, CA, in December 2018.

Disclosure statement

JJC has received honoraria and/or research funds from Abbvie, Beigene, Janssen, Millennium, Pharmacyclics and TG Therapeutics. SPT has received research funding and/or consulting fees from Pharmacyclics and Janssen. All other authors have no conflicts of interest to disclose.

Author contributions

JJC designed the study and performed the analysis. JJC, GI and JNG gathered patients’ data. MGD, MLG, AKo, CJ, XL, MM, NT, CJP, LX, GY and ZRH performed molecular testing in patients’ samples. JJC, CAF and SPT took care of patients. JJC and JNG drafted the manuscript. All authors critically reviewed and approved the final manuscript.

Additional information

Funding

Dr. Castillo would like to acknowledge the support of the WMR fund.