Waldenstrom macroglobulinemia is a distinct low-grade lymphoproliferative disease. There have been recent significant advances in understanding the underlying pathogenesis of this disease, including genetic and epigenetic regulators of tumor progression.
Current studies have shown that the tumor microenvironment plays a critical role in cell proliferation, dissemination, and drug resistance.
This review provides an update of the advances in the pathogenesis of factors both intrinsic (in the tumor clone) and extrinsic (in the bone marrow microenvironment) that regulate tumor progression in Waldenstrom macroglobulinemia. We next discuss novel agents that have been recently tested in clinical trials based on the advances observed in the pathogenesis of Waldenstrom macroglobulinemia.