Second Primary Malignancies And Disease Transformation In Newly Diagnosed Symptomatic Patients With Waldenstrom'S Macroglobulinemia: An Analysis From The Greek Myeloma Study Group

Waldenström's Macroglobulinemia (WM) is an indolent lymphoma with heterogeneous clinical presentation and prolonged course. In this context, determining the overall disease and therapy burden in terms of complications and associated comorbidities is necessary. Of special interest is the occurrence of a second malignancy and transformation to high-grade lymphoma, due to their impact on survival and quality of life but also on health resource use and follow up strategies. Several factors including immune dysfunction, genetic predisposition, environmental factors and treatment effects have been suggested, as possible underlying pathophysiologic mechanisms for development of second primary malignancies or transformation in patients with WM. While there have been data published from various series, it is crucial to have data after prolonged follow up, as the time for development of such complications may be long. The current study included all patients with symptomatic WM that have been registered in the prospectively maintained database of the Greek Myeloma Study Group, in which the development of a second malignancy and disease transformation is being recorded, including type of second cancer and date of diagnosis. All patients fulfilled criteria for symptomatic WM diagnosis and for treatment initiation according to the Consensus Recommendations. The analysis included 598 symptomatic newly diagnosed patients with WM with a median follow up of 114 months. The median age was 69 years and 284 (47%) have died. Primary therapy was rituximab-based in 65%, contained alkylating agents in 78%, a nucleoside analogue in …