Waldenstrom's macroglobulinemia in the era of immunotherapy

Waldenstrom macroglobulinemia (WM) is a lymphoplasmacytic lymphoma that presents with symptomatic anemia, thrombocytopenia, constitutional symptoms, extramedullary disease and rarely hyperviscosity syndrome. The presence of both IgM monoclonal protein and ≥10% monoclonal lymphoplasmacytic cells is required for the diagnosis. MyD88 is present in 67-90% of patients but is not pathognomonic for WM. Many patients who fulfill the criteria of WM are asymptomatic and do not require treatment. Recent advances in the understanding of the biology of WM have paved the way for new treatment options. The use of novel agents with or without rituximab enables the use of effective chemotherapy-free regiments upfront and in the relapsed setting. New targeted treatments such as venetoclax and CXCR4 antagonists are being investigated.