ABSTRACT
Waldenström's macroglobulinemia is a rare, low-grade non-Hodgkin lymphoma of B cell origin, most common in elderly male patients with a median age of 64 years at diagnosis. It accounts for approximately 2% of hematologic malignancies. The disease is incurable now with a median overall survival of 6.2 years. In the past decade growing evidence suggests the role of the complex signaling pathways and microenvironment as a potential target of the therapy in the lymphoproliferative disorders as well as Waldenström's macroglobulinemia. In this review we try to highlight the importance of these novel targets and their possible role in the therapeutic strategies. We further summarize our knowledge about the pathophysiology, diagnostics and therapeutics standards of the disease.
