Waldenström's macroglobulinemia (WM) is a rare subclass of the group of indolent B-cell lymphomas representing only 1-2 % of all hematological neoplasms. Genetic characterization of the disease allows a subdivision of WM into genotypes. The combination of rituximab and chemotherapy is still the backbone of treatment. The proteasome inhibitor bortezomib is another effective treatment option. The newly introduced oral Bruton's tyrosine kinase inhibitor ibrutinib provides an effective chemotherapy-free therapy for WM.
Keywords: Bortezomib; Ibrutinib; MYD88 protein; Rituximab; Waldenstrom macroglobulinemia; chemotherapy.