Waldenstrom macroglobulinemia (WM) is a rare lymphoplasmacytic lymphoma characterized by the presence of an immunoglobulin?M (IgM) monoclonal protein. WM is a chronic, indolent disease, which can remain undiagnosed for years. A?better understanding of the role of signaling pathways in the development of the disease and the identification of clinical and genetic markers have driven the development of targeted therapeutic strategies, improving overall survival.