Abstract
AbstractWaldenström macroglobulinemia (WM) is a rare subtype of non-Hodgkin lymphoma characterized by the accumulation of malignant lymphoplasmacytic cells in the bone marrow and other organs that secrete a monoclonal IgM paraprotein. Whole genome sequencing studies have detected recurrent somatic mutations in MYD88 and CXCR4, with a prevalence of 90% and 40%, respectively. Classic therapeutic options include alkylating agents and proteasome inhibitors, both in combination with rituximab. These regimens are associated with overall response rates (ORR) of 90% and median progression-free survival (PFS) between 4 and 6 years.
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