Waldenström’s macroglobulinemia (WM) is a B-cell lymphoproliferative disorder defined by a lymphoplasmacytic infiltration in the bone marrow or lymphatic tissue and a monoclonal immunoglobulin M (IgM) protein in the serum [1, 2]. The infiltration of the bone marrow and extramedullary sites by malignant B-cells, as well as elevated IgM levels, accounts for the symptoms associated with this disease. This may result in patients developing constitutional symptoms, pancytopenia, organomegaly, neuropathy, symptoms associated with immunoglobulin deposition, or hyperviscosity [3, 4]. There is significant heterogeneity, however, in the symptoms with which patients present. While some patients present with the symptoms listed above, many are asymptomatic at the time of diagnosis. Waldenström’s macroglobulinemia remains incurable with current therapy with a median survival for symptomatic patients of approximately 8 years . However, because many patients are diagnosed with this disease at an advanced age, approximately half of the patients die from causes unrelated to Waldenström’s macroglobulinemia. Due to the incurable nature of the disease, the heterogeneity of clinical presentation, as well as the comorbid conditions and competing causes of death, the decision to treat patients and the choice of treatment can be rather complex. A number of consensus meetings have listed reasonable treatment options [6–8], but the treating physician is still faced with a difficult treatment decision in a patient with an uncommon disease.
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