Waldenstrom’s macroglobulinemia

Waldenstrom’s macroglobulinemia is an indolent B-cell malignancy defined by a lymphoplasmacytic infiltration in the bone marrow or in other organs including lymph nodes, liver, and spleen, as well as a monoclonal immunoglobulin M protein (IgM) in the serum. The infiltration of the bone marrow and extramedullary sites by malignant B lymphocytes, as well as elevated IgM levels, typically leads to symptoms associated with this disease. While Waldenstrom’s macroglobulinemia typically follows an indolent course, the disease remains incurable with current therapy. Due to the heterogeneous clinical presentation, the presence of multiple comorbidities and competing causes of death, the decision to treat patients as well as the choice of treatment can be complex as many patients do not require treatment initially. When patients do require therapy, it is important to select therapies that do not limit future treatment options. To provide a simple risk-adapted approach to managing patients with Waldenstrom’s macroglobulinemia, a rational approach to this disease is presented.