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Waldenstrom macroglobulinemia (WM) is a B-cell lymphoplasmacytic lymphoma characterized by the production of a monoclonal IgM paraprotein and frequent mutation of the MYD88 gene. Treatment options have evolved over the past 10 to 15 years to include immunochemotherapy, proteasome inhibitors, and Bruton tyrosine kinase inhibitors (BTKi). To develop an updated prognostic scoring metric, investigators conducted a retrospective analysis using a derivation cohort (n=341) from a single institution and an external validation cohort (n=335) from multiple sites.
Median follow-up was 8.2 years in the derivation cohort and 6.3 years in the validation cohort. Univariate and multivariate analyses in the derivation cohort identified three factors that wer…